Dr. Lui earned his medical degree from the Yale University School of Medicine in New Haven, Connecticut, and subsequently completed his combined medicine and pediatric residency training at Harvard University in Boston, Massachusetts. He completed his fellowship in adult cardiology and adult congenital heart disease at Columbia University Medical Center in New York, New York.

Dr. Lui is the Medical Director of The Adult Congenital Heart Program at Stanford, a Lucile Packard Children’s Hospital (LPCH) and Stanford Healthcare collaboration and Program Director for the ACGME accredited adult congenital heart disease fellowship at Stanford. Dr. Lui is also appointed Clinical Professor of Medicine and Pediatrics at Stanford University School of Medicine.

Dr. Lui is a board certified ACHD physician and has gained national recognition for both his clinical work and research. His research interests include management of adults with congenital heart disease, pregnancy and congenital heart disease, adolescent transition to adult care, and echocardiography.

Clinical Focus

  • Adult Congenital Heart Disease
  • Echocardiography

Academic Appointments

Administrative Appointments

  • Medical Director, The Adult Congenital Heart Program at Stanford (2013 - Present)
  • Program Director, Adult Congenital Heart Disease Fellowship (2014 - Present)

Honors & Awards

  • M.D. cum laude, Yale University (2002)
  • Norma Bailey Berniker Prize, Yale University (2002)
  • Outstanding Research Award in Pediatric Cardiology, American Heart Association (2009)
  • E. William Hancock Teaching Award in Division of Cardiovascular Medicine, Stanford University (2015)

Boards, Advisory Committees, Professional Organizations

  • Associate Editor, International Journal of Cardiology Congenital Heart Disease (2021 - Present)
  • Fellow, American Society of Echocardiography (2018 - Present)
  • Fellow, American College of Cardiology (2012 - Present)
  • Member, American Heart Association (2010 - Present)
  • Member, International Society of Adult Congenital Heart Disease (2010 - Present)
  • Medical Advisory Board, Adult Congenital Heart Association (2013 - Present)

Professional Education

  • Board Certification: National Board of Echocardiography, Adult Echocardiography (2009)
  • Medical Education: Yale School Of Medicine (2002) CT
  • Board Certification: American Board of Internal Medicine, Adult Congenital Heart Disease (2015)
  • Residency: Brigham and Women's Hospital Harvard Medical School (2006) MA
  • Internship: Brigham and Women's Hospital Harvard Medical School (2003) MA
  • Board Certification: American Board of Internal Medicine, Cardiovascular Disease (2009)
  • Fellowship: Columbia University Medical Center (2009) NY
  • Board Certification, Adult Congenital Heart Disease, American Board of Internal Medicine (2015)
  • Board Certification, Adult Comprehensive Echocardiography, National Board of Echocardiography (2009)
  • Board Certification, Cardiovascular Disease, American Board of Internal Medicine (2009)
  • Board Certification, Internal Medicine, American Board of Internal Medicine (2006)
  • Fellowship, Columbia University Medical Center (2009)
  • Residency, Harvard Combined Internal Medicine and Pediatrics Residency Program (2006)
  • M.D., Yale University School of Medicine (2002)

Current Research and Scholarly Interests

Adult Congenital Heart Disease

Graduate and Fellowship Programs

All Publications

  • Multimodality Imaging in Management of Adults with Dextro-Transposition of the Great Arteries Post Arterial Switch Operation. CASE (Philadelphia, Pa.) Conrad, D. R., Woo, J. P., Lui, G. K., Clark, D. E. 2024; 8 (3Part A): 162-166


    • Echo is an essential tool for monitoring long-term complications of D-TGA with ASO. • Multimodality imaging is complementary to echo in the assessment of complications. • 4D flow CMR can assess supravalvular PS post ASO. • Branch pulmonary arteries after LeCompte maneuver can be challenging to image.

    View details for DOI 10.1016/

    View details for PubMedID 38524978

    View details for PubMedCentralID PMC10954704

  • Percutaneous Closure of a Ruptured Sinus of Valsalva Aneurysm under Transesophageal Echocardiography Guidance. CASE (Philadelphia, Pa.) Li, X. E., McElhinney, D. B., Lui, G. K., Clark, D. E., Woo, J. P. 2024; 8 (3Part A): 186-192


    • SOVA is a rare cardiac anomaly. • Ruptured SOVA carries a high mortality rate. • SOVA often coexists with other congenital lesions, most commonly VSD and bicuspid AV. • Ruptured SOVA needs repair; percutaneous repair is a safe alternative to surgery. • Echo plays a vital role in both diagnosing SOVA and guiding percutaneous closure.

    View details for DOI 10.1016/

    View details for PubMedID 38524988

    View details for PubMedCentralID PMC10954579

  • Summary of a Consensus Conference on Heart-Liver Transplantation. American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons Kobashigawa, J., VanWagner, L. B., Hall, S., Emamaullee, J., Entwistle, J. W., Ganger, D., Gebel, H., Jeevanandam, V., Kaldas, F., Kilic, A., Kittleson, M., Kushwaha, S., Kwong, A., Lui, G. K., Motayagheni, N., Patel, J., Patel, N., Pereira, N., Potter, L., Sani, M., Schiano, T. D., Shingina, A. 2023


    Patients with severe heart disease may have co-existing liver disease from various causes. The incidence of combined heart-liver transplant (CHLT) is increasing as more patients with congenital heart disease survive to adulthood and develop advanced heart failure with associated liver disease from chronic right-sided heart or Fontan failure. However, the criteria for CHLT have not been established. To address this unmet need, a virtual consensus conference was organized on June 10, 2022, endorsed by the American Society of Transplantation. The conference represented a collaborative effort by experts in cardiothoracic and liver transplantation from across the United States to assess interdisciplinary criteria for liver transplantation in the CHLT candidate, surgical considerations of CHLT, current allocation system that generally results in the liver following the heart for CHLT, and the optimal post-CHLT management. The conference served as a forum to unify criteria between the different specialties and to forge a pathway for patients who may need dual organ transplantation. Due to the continuing shortage of available donor organs, ethical issues related to multi-organ transplantation were also debated. The findings and consensus statements are presented.

    View details for DOI 10.1016/j.ajt.2023.12.002

    View details for PubMedID 38072122

  • Combined Heart Liver Transplantation in the Patients with Advanced Liver Disease and/or Hepatocellular Carcinoma: Why Can't Liver Pull the Heart? American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons Shingina, A., Bansal, N., Cedars, A., Chen, S., Daugherty, T., Emamaullee, J., Ganger, D., Ge, J., Heller, T., Hughes, D., Kay, J., Ortega Legaspi, J., Menachem, J., Montenovo, M., Sack, J., Reardon, L., Schiano, T., Simpson, K., Teuteberg, J., Tompkins, R., Vodkin, I., Wu, F., Lui, G. 2023

    View details for DOI 10.1016/j.ajt.2023.10.008

    View details for PubMedID 37839708

  • Transplant in Single Ventricle Physiology: The Fourth Stage? Journal of the American College of Cardiology Hsu, D. T., Lui, G. K. 2023; 82 (12): 1242-1244

    View details for DOI 10.1016/j.jacc.2023.06.038

    View details for PubMedID 37704314

  • Short- and Mid-Term Results of Pulmonary Valve Replacement with the Inspiris Valve. The Annals of thoracic surgery Ragheb, D. K., Martin, E., Jaggi, A., Lui, G. K., Maskatia, S. A., Ma, M., Hanley, F. L., McElhinney, D. B. 2023


    BACKGROUND: Various bioprosthetic valves are used off-label for pulmonary valve replacement (PVR), but there is no consensus about whether a particular valve is best for this application. Recently, the Inspiris Valve was approved for aortic valve replacement, and surgeons have begun utilizing it for PVR. There is limited evidence about the performance of the Inspiris valve compared to other valves in the pulmonary position.METHODS: We reviewed all patients who underwent PVR with a size 19-27mm Inspiris or Mosaic valve from 2007-2022. Mid-term outcomes included freedom from moderate or severe pulmonary regurgitation (PR), a maximum Doppler gradient >36mmHg, and freedom from reintervention.RESULTS: A total of 225 consecutive patients who underwent PVR with a size 19-27mm Mosaic (n=163) or Inspiris (n=62) valve were included. There was no difference in baseline characteristics. Early postoperative gradients were low in both groups, but higher in the Mosaic cohort, and neither group had more than mild PR on discharge. On uni- and multivariable analysis, Inspiris valves were significantly more likely to develop moderate or greater PR over time. There was no significant difference between the valves in freedom from reintervention or from a maximum gradient >36mmHg.CONCLUSIONS: Early and short-term gradients were similar in patients undergoing PVR with Inspiris and Mosaic valves, but significant PR was more common in patients who received an Inspiris valve. These preliminary findings suggest that the durability of the Inspiris valve in the pulmonary position may not be superior to other bioprosthetic valves used for PVR.

    View details for DOI 10.1016/j.athoracsur.2023.07.049

    View details for PubMedID 37625611

  • Improved Right Ventricular Energy Efficiency by 4-Dimensional Flow Magnetic Resonance Imaging After Harmony Valve Implantation. JACC. Advances Woo, J. P., Dong, M. L., Kong, F., McElhinney, D. B., Schiavone, N., Chan, F., Lui, G. K., Haddad, F., Bernstein, D., Marsden, A. 2023; 2 (3)

    View details for DOI 10.1016/j.jacadv.2023.100284

    View details for PubMedID 37691969

    View details for PubMedCentralID PMC10487049

  • PAPVR - An incidental finding that may not be so benign INTERNATIONAL JOURNAL OF CARDIOLOGY CONGENITAL HEART DISEASE Romfh, A., Lui, G. K. 2023; 11
  • First described mitral clip in an adult extracardiac Fontan patient: a case report. European heart journal. Case reports Haeffele, C. L., Lui, G. K., Peng, L., Chan, F., Sharma, R. P. 2023; 7 (1): ytac479


    The use of transcatheter edge-to-edge repair (TEER) in patients with advanced heart failure has been shown to reduce hospitalizations and increase survival. As patients with Fontan circulations grow older, a significant proportion of them will develop severe atrioventricular (AV) valve regurgitation in the systemic ventricle. Conventional surgical repair and transplant carry high mortality risk for the adult Fontan patient with progressive heart failure.A 51-year-old female extracardiac Fontan patient developed severe AV valve regurgitation and progressive functional decline. Based on her operative risk for conventional surgical intervention or transplant, TEER using the Abbott MitraClip device was performed. The degree of mitral regurgitation was decreased from severe to moderate regurgitation.This is the first known case describing the use of a successful TEER in an adult patient with an extracardiac Fontan. Given the increasing numbers of patients surviving into adulthood with a Fontan circulation, transcatheter interventions may provide an alternative treatment option to conventional surgeries and medical therapies.

    View details for DOI 10.1093/ehjcr/ytac479

    View details for PubMedID 36733686

    View details for PubMedCentralID PMC9887705

  • Improved Right Ventricular Energy Efficiency by 4-Dimensional Flow Magnetic Resonance Imaging After Harmony Valve Implantation JACC:Advances Woo, J. P., Dong, M. L., Kong, F., McElhinney, D. B., Schiavone, N., Chan, F., Lui, G. K., Haddad, F., Bernstein, D., Marsden, A. 2023; 2 (3)
  • Health Care Usage Among Adolescents With Congenital Heart Defects at 5 Sites in the United States, 2011 to 2013. Journal of the American Heart Association Lui, G. K., Sommerhalter, K., Xi, Y., Botto, L. D., Crume, T., Farr, S., Feldkamp, M. L., Glidewell, J., Hsu, D., Khanna, A., Krikov, S., Li, J., Raskind-Hood, C., Sarno, L., Van Zutphen, A. R., Zaidi, A., Soim, A., Book, W. M. 2022: e026172


    Background We sought to characterize health care usage for adolescents with congenital heart defects (CHDs) using population-based multisite surveillance data. Methods and Results Adolescents aged 11 to 18years with ≥1 CHD-related diagnosis code and residing in 5 US sites were identified in clinical and administrative data sources for the years 2011 to 2013. Sites linked data on all inpatient, emergency department (ED), and outpatient visits. Multivariable log-binomial regression models including age, sex, unweighted Charlson comorbidity index, CHD severity, cardiology visits, and insurance status, were used to identify associations with inpatient, ED, and outpatient visits. Of 9626 eligible adolescents, 26.4% (n=2543) had severe CHDs and 21.4% had Charlson comorbidity index >0. At least 1 inpatient, ED, or outpatient visit was reported for 21%, 25%, and 96% of cases, respectively. Cardiology visits, cardiac imaging, cardiac procedures, and vascular procedures were reported for 38%, 73%, 10%, and 5% of cases, respectively. Inpatient, ED, and outpatient visits were consistently higher for adolescents with severe CHDs compared with nonsevere CHDs. Adolescents with severe and nonsevere CHDs had higher health care usage compared with the 2011 to 2013 general adolescent US population. Adolescents with severe CHDs versus nonsevere CHDs were twice as likely to have at least 1 inpatient visit when Charlson comorbidity index was low (Charlson comorbidity index =0). Adolescents with CHDs and public insurance, compared with private insurance, were more likely to have inpatient (adjusted prevalence ratio, 1.5 [95% CI, 1.3-1.7]) and ED (adjusted prevalence ratio, 1.6 [95% CI, 1.4-1.7]) visits. Conclusions High resource usage by adolescents with CHDs indicates a substantial burden of disease, especially with public insurance, severe CHDs, and more comorbidities.

    View details for DOI 10.1161/JAHA.122.026172

    View details for PubMedID 36102252

  • How Well Do ICD-9-CM Codes Predict True Congenital Heart Defects? A Centers for Disease Control and Prevention-Based Multisite Validation Project. Journal of the American Heart Association Rodriguez, F. H., Raskind-Hood, C. L., Hoffman, T., Farr, S. L., Glidewell, J., Li, J. S., D'Ottavio, A., Botto, L., Reeder, M. R., Hsu, D., Lui, G. K., Sullivan, A. M., Book, W. M. 2022: e024911


    Background The Centers for Disease Control and Prevention's Surveillance of Congenital Heart Defects Across the Lifespan project uses large clinical and administrative databases at sites throughout the United States to understand population-based congenital heart defect (CHD) epidemiology and outcomes. These individual databases are also relied upon for accurate coding of CHD to estimate population prevalence. Methods and Results This validation project assessed a sample of 774 cases from 4 surveillance sites to determine the positive predictive value (PPV) for identifying a true CHD case and classifying CHD anatomic group accurately based on 57 International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes. Chi-square tests assessed differences in PPV by CHD severity and age. Overall, PPV was 76.36% (591/774 [95% CI, 73.20-79.31]) for all sites and all CHD-related ICD-9-CM codes. Of patients with a code for complex CHD, 89.85% (177/197 [95% CI, 84.76-93.69]) had CHD; corresponding PPV estimates were 86.73% (170/196 [95% CI, 81.17-91.15]) for shunt, 82.99% (161/194 [95% CI, 76.95-87.99]) for valve, and 44.39% (83/187 [95% CI, 84.76-93.69]) for "Other" CHD anatomic group (X2=142.16, P<0.0001). ICD-9-CM codes had higher PPVs for having CHD in the 3 younger age groups compared with those >64years of age, (X2=4.23, P<0.0001). Conclusions While CHD ICD-9-CM codes had acceptable PPV (86.54%) (508/587 [95% CI, 83.51-89.20]) for identifying whether a patient has CHD when excluding patients with ICD-9-CM codes for "Other" CHD and code 745.5, further evaluation and algorithm development may help inform and improve accurate identification of CHD in data sets across the CHD ICD-9-CM code groups.

    View details for DOI 10.1161/JAHA.121.024911

    View details for PubMedID 35862148

  • Tetralogy of Fallot and Aortic Dissection: Implications in Management. JACC. Case reports Vaikunth, S. S., Chan, J. L., Woo, J. P., Bykhovsky, M. R., Lui, G. K., Ma, M., Romfh, A. W., Lamberti, J., Mastrodicasa, D., Fleischmann, D., Fischbein, M. P. 2022; 4 (10): 581-586


    We present the case of a 61-year-old man with tetralogy of Fallot postrepair and mechanical aortic valve replacement with an aortic root/ascending/arch aneurysm with chronic type A aortic dissection. He underwent uncomplicated aortic root and total arch replacement. Continued surveillance for aortic aneurysm is necessary in the tetralogy of Fallot population. (Level of Difficulty: Intermediate.).

    View details for DOI 10.1016/j.jaccas.2022.02.021

    View details for PubMedID 35615213

  • Classic pattern dyssynchrony is associated with outcome in patients with Fontan circulation. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Rosner, A., McElhinney, D. B., Diab, S. G., Friedberg, M. K., Lui, G. K. 2022


    BACKGROUND: Morbidity and mortality increase as Fontan patients age into adulthood. Limited studies have examined cardiac magnetic resonance and echocardiographic parameters to predict death and transplantation in children after Fontan operation. The aim of the study was to investigate echocardiographic parameters in adolescents and adults after Fontan operation including myocardial mechanics including classic pattern dyssynchrony (CPD) as predictors of transplantation or death.METHODS: In a cross-sectional retrospective study, strain analysis was performed on echocardiography studies performed between 2001 and 2015 of 110 patients with single ventricle physiology after the Fontan procedure. Strain curves were measured and visually assessed for the presence of CPD. The primary end point was death or transplantation after a follow-up period of 85±35 months after echocardiography.RESULTS: Median age at date of echocardiography was 20, range 3 to 45 years. Of 110 patients 28 patients were transplanted. During the study-period 3 patients died after transplantation and 7 patients died without being transplanted. CPD was seen in 16 and protein losing enteropathy (PLE) in 21 of 110 patients. By multivariate-analysis, CPD (HR 9.4 CI 2.6-34.6), PLE (HR 10.6 CI 3.4-33.2); systolic blood pressure (HR 0.954 CI 0.913-0.996), systolic/diastolic duration ratio (HR 6.83 CI 1.33-35.0) and E wave deceleration time (HR 0.98 CI 0.97 - 0.99) were independently associated with the primary end point.CONCLUSION: CPD, PLE, systolic and diastolic ventricular dysfunction are significantly associated with transplantation or death in Fontan operated patients. In selected patients, the presence of CPD may be a basis to investigate cardiac resynchronization therapy as a treatment strategy.

    View details for DOI 10.1016/j.echo.2022.01.012

    View details for PubMedID 35121052

  • Fingers in a dike: Systematic approach to coiling of aortopulmonary and venovenous collaterals in the Fontan referred for transplantation INTERNATIONAL JOURNAL OF CARDIOLOGY CONGENITAL HEART DISEASE Vaikunth, S. S., Lui, G. K., Kim, Y. Y. 2021; 6
  • Exercise prescription as medicine INTERNATIONAL JOURNAL OF CARDIOLOGY CONGENITAL HEART DISEASE Lui, G. K., Moons, P. 2021; 5
  • The atrial switch patient with a dilated subpulmonic left ventricle (vol 4,100153, 2021) INTERNATIONAL JOURNAL OF CARDIOLOGY CONGENITAL HEART DISEASE Vaikunth, S. S., Haeffele, C., Mcelhinney, D. B., Lui, G. K., Romfh, A. W. 2021; 5
  • The challenges of an aging Tetralogy of Fallot population. Expert review of cardiovascular therapy Woo, J. P., McElhinney, D. B., Lui, G. K. 2021


    INTRODUCTION: Advancements in surgery and management have resulted in a growing population of aging adults with tetralogy of Fallot (TOF). As a result, there has been a parallel growth in late complications associated with the sequelae from the underlying cardiac anomalies as well as the surgical and other interventional treatments.AREAS COVERED: Here, we review challenges related to an aging population of patients with TOF, particularly late complications, and highlight advances in management and key areas for future research. Pulmonary regurgitation, heart failure, arrhythmias, and aortic complications are some of these late complications. There is also a growing incidence of acquired cardiovascular disease, obesity, and diabetes associated with aging. Management of these late complications and acquired comorbidities continues to evolve as research provides insights into long-term outcomes from medical therapies and surgical interventions.EXPERT OPINION: The management of an aging TOF population will continue to transform with advances in imaging technologies to identify subclinical disease and valve replacement technologies that will prevent and mitigate disease progression. In coming years, we speculate there will be more data to support the use of novel heart failure therapies in TOF and consensus guidelines on management of refractory arrhythmias and aortic complications.

    View details for DOI 10.1080/14779072.2021.1940960

    View details for PubMedID 34102942

  • Durability of Pulmonary Valve Replacement with Large Diameter Stented Porcine Bioprostheses. Seminars in thoracic and cardiovascular surgery Maeda, K., Lui, G. K., Zhang, Y., Maskatia, S. A., Romfh, A., Yarlagadda, V. V., Hanley, F. L., McElhinney, D. B. 2021


    There is limited information about durability of large diameter porcine bioprostheses implanted for pulmonary valve replacement (PVR). We studied patients who underwent surgical PVR from 2002-2019 with a stented porcine bioprosthetic valve (BPV) with a labeled size ≥27 mm. The primary outcome was freedom from reintervention. During the study period, 203 patients underwent PVR using a porcine BPV ≥27 mm, 94% of whom received a Mosaic valve (Medtronic Inc., Minneapolis, MN). Twenty patients underwent reintervention from 3.4-12.0 years after PVR: 5 surgical and 15 transcatheter PVR procedures. The indication for reintervention was regurgitation in 13 patients, stenosis in 2, mixed disease in 4, and endocarditis in 1. Estimate freedom from reintervention was 97±1% at 5 years and 82±4% at 10 years, and freedom from prosthesis dysfunction (moderate or severe regurgitation and/or a maximum Doppler gradient ≥50 mmHg) over time was 91±2% at 5 years and 74±4% at 10 years. Younger age and smaller true valve diameter were associated with shorter freedom from reintervention, but valve oversizing was not. The durability of large stented porcine bioprostheses in the pulmonary position is generally excellent, particularly in adolescents and adults, similar to various other types of BPV. In the current study, relative valve size was not associated with valve longevity, although the low event-rate in this population was a limiting factor.

    View details for DOI 10.1053/j.semtcvs.2021.03.044

    View details for PubMedID 33971298

  • COVID-19 in Adults With CongenitalHeart Disease. Journal of the American College of Cardiology Broberg, C. S., Kovacs, A. H., Sadeghi, S., Rosenbaum, M. S., Lewis, M. J., Carazo, M. R., Rodriguez, F. H., Halpern, D. G., Feinberg, J., Galilea, F. A., Baraona, F., Cedars, A. M., Ko, J. M., Porayette, P., Maldonado, J., Sarubbi, B., Fusco, F., Frogoudaki, A. A., Nir, A., Chaudhry, A., John, A. S., Karbassi, A., Hoskoppal, A. K., Frischhertz, B. P., Hendrickson, B., Bouma, B. J., Rodriguez-Monserrate, C. P., Broda, C. R., Tobler, D., Gregg, D., Martinez-Quintana, E., Yeung, E., Krieger, E. V., Ruperti-Repilado, F. J., Giannakoulas, G., Lui, G. K., Ephrem, G., Singh, H. S., Almeneisi, H. M., Bartlett, H. L., Lindsay, I., Grewal, J., Nicolarsen, J., Araujo, J. J., Cramer, J. W., Bouchardy, J., Al Najashi, K., Ryan, K., Alshawabkeh, L., Andrade, L., Ladouceur, M., Schwerzmann, M., Greutmann, M., Meras, P., Ferrero, P., Dehghani, P., Tung, P. P., Garcia-Orta, R., Tompkins, R. O., Gendi, S. M., Cohen, S., Klewer, S., Hascoet, S., Mohammadzadeh, S., Upadhyay, S., Fisher, S. D., Cook, S., Cotts, T. B., Aboulhosn, J. A. 2021; 77 (13): 1644–55


    BACKGROUND: Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications.OBJECTIVES: This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes.METHODS: Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined.RESULTS: From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p=0.001), whereas anatomic complexity or defect group were not.CONCLUSIONS: COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity.

    View details for DOI 10.1016/j.jacc.2021.02.023

    View details for PubMedID 33795039

  • Access to cardiac surgery centers for cardiac and non-cardiac hospitalizations in adolescents and adults with congenital heart defects- a descriptive case series study. American heart journal Insaf, T. Z., Sommerhalter, K. M., Jaff, T. A., Farr, S. L., Downing, K. F., Zaidi, A. N., Lui, G. K., Van Zutphen, A. R. 2021


    BACKGROUND: Individuals with congenital heart defects (CHDs) are recommended to receive all inpatient cardiac and non-cardiac care at facilities that can offer specialized care. We describe geographic accessibility to such centers in New York State and determine several factors associated with receiving care there.METHODS: We used inpatient hospitalization data from the Statewide Planning and Research Cooperative System (SPARCS) in New York State 2008-2013. In the absence of specific adult CHD care center designations during our study period, we identified pediatric/adult and adult-only cardiac surgery centers through the Cardiac Surgery Reporting System to estimate age-based specialized care. We calculated one-way drive and public transit time (in minutes) from residential address to centers using R gmapsdistance package and the Google Maps Distance Application Programming Interface (API). We calculated prevalence ratios using modified Poisson regression with model-based standard errors, fit with generalized estimating equations clustered at the hospital level and sub-clustered at the individual level.RESULTS: Individuals with CHDs were more likely to seek care at pediatric/adult or adult-only cardiac surgery centers if they had severe CHDs, private health insurance, higher severity of illness at encounter, a surgical procedure, cardiac encounter, and shorter drive time. These findings can be used to increase care receipt (especially for non-cardiac care) at pediatric/adult or adult-only cardiac surgery centers, identify areas with limited access, and reduce disparities in access to specialized care among this high-risk population.

    View details for DOI 10.1016/j.ahj.2021.02.018

    View details for PubMedID 33636136

  • A 40-Year-Old Man With Tricuspid Atresia, Status Post-Fontan, With Severe COVID-19 Pneumonia and Pneumothorax. JACC. Case reports Vaikunth, S. S., Bykhovsky, M. R., Romfh, A. W., Haeffele, C. L., Rogers, I. S., Dong, E., Scribner, C., Lui, G. K. 2021


    We report a case of COVID-19 in an adult single-ventricle patient post-Fontan-to our knowledge, the first report in this population documenting the use of the latest management recommendations for this novel disease. Additionally, this patient had significant pre-existing ventricular dysfunction, valvular disease, and comorbidities including HIV. (Level of Difficulty: Advanced.).

    View details for DOI 10.1016/j.jaccas.2020.10.032

    View details for PubMedID 33558861

  • The atrial switch patient with a dilated subpulmonic left ventricle International Journal of Cardiology Congenital Heart Disease Vaikunth, S. S., Haeffele, C., McElhinney, D. B., Lui, G. K., Romfh, A. 2021; Volume 4 (100153)
  • Race and Genetics in Congenital Heart Disease: Application of iPSCs, Omics, and Machine Learning Technologies. Frontiers in cardiovascular medicine Mullen, M. n., Zhang, A. n., Lui, G. K., Romfh, A. W., Rhee, J. W., Wu, J. C. 2021; 8: 635280


    Congenital heart disease (CHD) is a multifaceted cardiovascular anomaly that occurs when there are structural abnormalities in the heart before birth. Although various risk factors are known to influence the development of this disease, a full comprehension of the etiology and treatment for different patient populations remains elusive. For instance, racial minorities are disproportionally affected by this disease and typically have worse prognosis, possibly due to environmental and genetic disparities. Although research into CHD has highlighted a wide range of causal factors, the reasons for these differences seen in different patient populations are not fully known. Cardiovascular disease modeling using induced pluripotent stem cells (iPSCs) is a novel approach for investigating possible genetic variants in CHD that may be race specific, making it a valuable tool to help solve the mystery of higher incidence and mortality rates among minorities. Herein, we first review the prevalence, risk factors, and genetics of CHD and then discuss the use of iPSCs, omics, and machine learning technologies to investigate the etiology of CHD and its connection to racial disparities. We also explore the translational potential of iPSC-based disease modeling combined with genome editing and high throughput drug screening platforms.

    View details for DOI 10.3389/fcvm.2021.635280

    View details for PubMedID 33681306

    View details for PubMedCentralID PMC7925393

  • Does liver biopsy accurately measure fibrosis in Fontan associated liver disease? A comparison of liver biopsy pre-combined heart and liver transplant and liver explant post-transplant. Clinical transplantation Vaikunth, S. S., Higgins, J. P., Concepcion, W., Haeffele, C., Wright, G. E., Chen, S., Lui, G. K., Daugherty, T. 2020: e14120


    The accuracy of liver biopsy to stage fibrosis due to Fontan associated liver disease (FALD) remains unclear. We compared results of biopsy pre-combined heart and liver transplantation (CHLT) to results of whole liver explant. Liver biopsy and explants from 15 Fontan patients (ages 16 - 49, median 28 years) were retrospectively reviewed. Staging was as follows: stage 0: no fibrosis, stage 1: pericellular fibrosis, stage 2: bridging fibrosis, stage 3: regenerative nodules. There is no stage 4. Clinical characteristics including Model of End-stage Liver Disease eXcluding INR and Varices, Ascites, Splenomegaly, and Thrombocytopenia (VAST) scores were collected, and descriptive statistics and Mann-Whitney U tests used to analyze data. All patients had biopsies with at least bridging fibrosis, and all had nodularity on explant; transjugular biopsy never overestimated fibrosis. Explant showed higher grade fibrosis (Stage 3) than pre-CHLT biopsy (Stage 2) in 6 of 15 patients and equal grade of fibrosis (Stage 3) in 9 of 15 patients. Though clinical characteristics varied significantly, VAST score was ≥ 2 in all but two patients. Transjugular liver biopsy does not overestimate and can underestimate fibrosis in Fontan patients undergoing CHLT, likely due to the patchy nature of fibrosis in FALD.

    View details for DOI 10.1111/ctr.14120

    View details for PubMedID 33053213

  • Inpatient admissions and costs for adolescents and young adults with congenital heart defects in New York, 2009-2013. Birth defects research Hsu, W., Sommerhalter, K. M., McGarry, C. E., Farr, S. L., Downing, K. F., Lui, G. K., Zaidi, A. N., Hsu, D. T., Van Zutphen, A. R. 2020


    OBJECTIVES: Most individuals born with congenital heart defects (CHDs) survive to adulthood, but healthcare utilization patterns for adolescents and adults with CHDs have not been well described. We sought to characterize the healthcare utilization patterns and associated costs for adolescents and young adults with CHDs.METHODS: We examined 2009-2013 New York State inpatient admissions of individuals ages 11-30 years with ≥1 CHD diagnosis codes recorded during any admission. We conducted multivariate linear regression using generalized estimating equations to examine associations between inpatient costs and sociodemographic and clinical variables.RESULTS: We identified 5,100 unique individuals with 9,593 corresponding hospitalizations over the study period. Median inpatient cost and length of stay (LOS) were $10,720 and 3.0 days per admission, respectively; 55.1% were emergency admissions. Admission volume increased 48.7% from 2009 (1,538 admissions) to 2013 (2,287 admissions), while total inpatient costs increased 91.8% from 2009 ($27.2 million) to 2013 ($52.2 million). Inpatient admissions and costs rose more sharply over the study period for those with nonsevere CHDs compared to severe CHDs. Characteristics associated with higher costs were longer LOS, severe CHD, cardiac/vascular hospitalization classification, surgical procedures, greater severity of illness, and admission in New York City.CONCLUSION: This study provides an informative baseline of health care utilization patterns and associated costs among adolescents and young adults with CHDs in New York State. Structured transition programs may aid in keeping this population in appropriate cardiac care as they move to adulthood.

    View details for DOI 10.1002/bdr2.1809

    View details for PubMedID 32990389

  • Adult Congenital Heart Disease-Preparing for the Changing Work Force Demand. Cardiology clinics Gurvitz, M., Lui, G. K., Marelli, A. 2020; 38 (3): 283–94


    This volume is dedicated to advances in the care of adults with congenital heart disease (CHD). In this chapter the authors review the data cornerstone to the growing workforce needs.This first chapter serves as a backdrop to the second chapter that applies these observations to the planning of health care services delivery in the United States accounting for the definition and organization of multisystem expertise and centers for adults with CHD at a health systems level.

    View details for DOI 10.1016/j.ccl.2020.04.011

    View details for PubMedID 32622484

  • Health Care Transition Perceptions Among Parents of Adolescents with Congenital Heart Defects in Georgia and New York. Pediatric cardiology Gaydos, L. M., Sommerhalter, K., Raskind-Hood, C., Fapo, O., Lui, G., Hsu, D., Van Zutphen, A., Glidewell, J., Farr, S., Rodriguez, F. H., Hoffman, T., Book, W. 2020


    With increasing survival trends for children and adolescents with congenital heart defects (CHD), there is a growing need to focus on transition from pediatric to adult specialty cardiac care. To better understand parental perspectives on the transition process, a survey was distributed to 451 parents of adolescents with CHD who had recent contact with the healthcare system in Georgia (GA) and New York (NY). Among respondents, 90.7% reported excellent, very good or good health-related quality of life (HRQoL) for their adolescent. While the majority of parents (77.8%) had been told by a provider about their adolescent's need to transition to adult specialty cardiac care, most reported concerns about transitioning to adult care. Parents were most commonly concerned with replacing the strong relationship with pediatric providers (60.7%), locating an appropriate adult provider (48.7%), and accessing adult health insurance coverage (43.6%). These findings may offer insights into transition planning for adolescents with CHD.

    View details for DOI 10.1007/s00246-020-02378-z

    View details for PubMedID 32500288

  • Proximity to risk-appropriate perinatal hospitals for pregnant women with congenital heart defects in New York state. BMC pregnancy and childbirth Schlichting, L. E., Insaf, T., Lui, G., Zaidi, A., Van Zutphen, A. 2020; 20 (1): 338


    BACKGROUND: Women with congenital heart defects (CHDs) experiencing pregnancies require specialized delivery care and extensive monitoring that may not be available at all birthing hospitals. In this study, we examined proximity to, and delivery at, a hospital with an appropriate level of perinatal care for pregnant women with CHDs and evaluated predictors of high travel distance to appropriate care. Appropriate care was defined as Level 3 perinatal hospitals and Regional Perinatal Centers (RPCs).METHODS: Inpatient delivery records for women with CHD in New York State (NYS) between 2008 and 2013 were obtained. Driving time and transit time were calculated between the pregnant woman's residence and the actual delivery hospital as well as the closest Level 3 or RPC hospital using Geographic Information Systems (GIS). Linear and logistic regression models evaluated predictors of high distance to, and utilization of, appropriate delivery care respectively.RESULTS: From 2008 to 2013, there were 909 deliveries in a NYS hospital by women with CHDs. Approximately 75% of women delivered at a Level 3 or RPC hospital. Younger women, those who reside in rural and smaller urban areas, and those who are non-Hispanic White had a greater drive time to an appropriate care facility. After adjustment for geographic differences, racial/ethnic minorities and poor women were less likely to deliver at an appropriate delivery care center.CONCLUSIONS: Although most women with CHDs in NYS receive appropriate delivery care, there are some geographic and socio-demographic differences that require attention to ensure equitable access.

    View details for DOI 10.1186/s12884-020-03025-4

    View details for PubMedID 32487099

  • ACC/AHA/ASE/HRS/ISACHD/SCAI/SCCT/SCMR/SOPE 2020 Appropriate Use Criteria for Multimodality Imaging During the Follow-Up Care of Patients With Congenital Heart Disease JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Sachdeva, R., Valente, A., Armstrong, A. K., Cook, S. C., Han, B., Lopez, L., Lui, G. K., Pickard, S. S., Powell, A. J., Bhave, N. M., Pickard, S. S., Baffa, J. M., Banka, P., Cohen, S. B., Glickstein, J. S., Kanter, J. P., Kanter, R. J., Kim, Y. Y., Kipps, A. K., Latson, L. A., Lin, J. P., Parra, D. A., Rodriguez, F. H., Saarel, E. V., Srivastava, S., Stephenson, E. A., Stout, K. K., Zaidi, A. N., Gluckman, T. J., Aggarwal, N. R., Bhave, N. M., Dehmer, G. J., Gilbert, O. N., Kumbhani, D. J., Price, A. L., Winchester, D. E., Gulati, M., Dehmer, G. J., Doherty, J. U., Bhave, N. M., Daugherty, S. L., Dean, L. S., Desai, M. Y., Gillam, L. D., Mehrotra, P. 2020; 75 (6): 657–703

    View details for DOI 10.1016/j.jacc.2019.10.002

    View details for Web of Science ID 000512903000013

    View details for PubMedID 31918898

  • Sex differences in patients with repaired tetralogy of Fallot support a tailored approach for males and females: a cardiac magnetic resonance study. The international journal of cardiovascular imaging Hagdorn, Q. A., Beurskens, N. E., Gorter, T. M., Eshuis, G. n., Hillege, H. L., Lui, G. K., Ceresnak, S. R., Chan, F. P., van Melle, J. P., Berger, R. M., Willems, T. P. 2020


    Purpose Substantial differences between sexes exist with respect to cardiovascular diseases, including congenital heart disease. Nevertheless, clinical decisions in the long-term follow-up of patients with repaired tetralogy of Fallot (rTOF) are currently based on unisex thresholds for cardiac magnetic resonance (CMR) measurements. This study aimed to assess whether sex differences exist in cardiac adaptation to hemodynamic loading conditions in patients with rTOF. Methods and Results This cross-sectional, two-center, combined pediatric and adult cohort included 320 rTOF patients (163 males, 51%) who underwent routine CMR. Despite similar age (median and interquartile range [m + IQR] 23.4 [15.2-34.4] years), surgical history, and hemodynamic loading, males with rTOF demonstrated higher biventricular CMR-derived volumes and masses, indexed for body surface area, compared to females (e.g. m + IQR right ventricular (RV) end-diastolic volume: males 123 [100-151] mL/m2, females 114 [94-131] mL/m2, P = 0.007). Sex-specific Z-scores of biventricular volumes and masses were similar for males and females. RV volumes and masses correlated with hemodynamic loading, but these relations did not differ between sexes. Biventricular ejection fraction (EF) appeared to be lower in male patients, compared to female patients (e.g. m + IQR RVEF: males 48 [43-54]%, females 52 [46-57]%, P < 0.001). Conclusion Indexed ventricular volumes and masses are higher in males with rTOF, compared to females, similar to the healthy population. RV hypertrophy and dilatation correlated to loading conditions similarly for both sexes. However, under comparable loading conditions, males demonstrated more severe functional impairment. These results indicate that sex-differences should no longer be ignored in treatment strategies, including timing of pulmonary valve replacement.

    View details for DOI 10.1007/s10554-020-01900-x

    View details for PubMedID 32472300

  • Predicting 10-year mortality in adults with congenital heart disease International Journal of Cardiology Congenital Heart Disease Fernandes, S. M., Lui, G. K., Long, J., Lin, A., Rogers, I. S., Sillman, C., Romfh, A., Dade, T., Dong, E., Haeffele, C., Scribner, C., Major, M., McElhinney, D. 2020
  • RNA Sequencing Analysis of Induced Pluripotent Stem Cell-Derived Cardiomyocytes from Congenital Heart Disease Patients. Circulation research Kitani, T. n., Tian, L. n., Zhang, T. n., Itzhaki, I. n., Zhang, J. Z., Ma, N. n., Liu, C. n., Rhee, J. W., Romfh, A. n., Lui, G. K., Wu, J. C. 2020

    View details for DOI 10.1161/CIRCRESAHA.119.315653

    View details for PubMedID 32070195

  • The Hidden Victims of the COVID-19 Pandemic: Congenital Heart Disease Patients. JACC. Case reports El-Saiedi, S. A., Haeffele, C. n., Hanna, B. M., Lui, G. K. 2020; 2 (9): 1411–13

    View details for DOI 10.1016/j.jaccas.2020.05.081

    View details for PubMedID 32835286

    View details for PubMedCentralID PMC7294284

  • Adults With Mild-to-Moderate Congenital Heart Disease Demonstrate Measurable Neurocognitive Deficits. Journal of the American Heart Association Perrotta, M. L., Saha, P. n., Zawadzki, R. n., Beidelman, M. n., Ingelsson, E. n., Lui, G. K., Priest, J. R. 2020: e015379


    Background Neurocognitive impairment is a common complication of congenital heart disease (CHD) as well as acquired cardiovascular disease. Data are limited on neurocognitive function in adults with CHD (ACHD). Methods and Results A total of 1020 individuals with mild-to-moderate ACHD and 497 987 individuals without ACHD from the volunteer-based UK Biobank study underwent neurocognitive tests for fluid intelligence, reaction time, numeric memory, symbol-digit substitution, and trail making at enrollment and follow-up. Performance scores were compared before and after exclusion of preexisting stroke or coronary artery disease as measures of cerebro- and cardiovascular disease. Individuals with ACHD had significantly poorer performance on alpha-numeric trail making, a measure of visual attention and cognitive flexibility, spending 6.4 seconds longer on alpha-numeric trail making (95% CI, 3.0-9.9 seconds, P=0.002) and 2.5 seconds longer on numeric trail making (95% CI, 0.5-4.6 seconds, P=0.034), a measure of visual attention and processing speed. The ACHD cohort had modestly lower performance on symbol-digit substitution, a measure of processing speed, with 0.9 fewer correct substitutions (95% CI, - 1.5 to - 0.2 substitutions, P=0.021). After excluding preexisting stroke or coronary artery disease, individuals with ACHD continued to show poorer performance in all 6 domains (P=NS). Conclusions Individuals with mild-to-moderate ACHD had poorer neurocognitive performance, most significantly in tests of cognitive flexibility, analogous to deficits in children with CHD. These differences appear to be driven by increased burden of cerebro- and cardiovascular disease among individuals with ACHD.

    View details for DOI 10.1161/JAHA.119.015379

    View details for PubMedID 32981450

  • Characteristics of Adults With Congenital Heart Defects in the United States. Journal of the American College of Cardiology Gurvitz, M. n., Dunn, J. E., Bhatt, A. n., Book, W. M., Glidewell, J. n., Hogue, C. n., Lin, A. E., Lui, G. n., McGarry, C. n., Raskind-Hood, C. n., Van Zutphen, A. n., Zaidi, A. n., Jenkins, K. n., Riehle-Colarusso, T. n. 2020; 76 (2): 175–82


    In the United States, >1 million adults are living with congenital heart defects (CHDs), but gaps exist in understanding the health care needs of this growing population.This study assessed the demographics, comorbidities, and health care use of adults ages 20 to 64 years with CHDs.Adults with International Classification of Disease-9th Revision-Clinical Modification CHD-coded health care encounters between January 1, 2008 (January 1, 2009 for Massachusetts) and December 31, 2010 were identified from multiple data sources at 3 U.S. sites: Emory University (EU) in Atlanta, Georgia (5 counties), Massachusetts Department of Public Health (statewide), and New York State Department of Health (11 counties). Demographics, insurance type, comorbidities, and encounter data were collected. CHDs were categorized as severe or not severe, excluding cases with isolated atrial septal defect and/or patent foramen ovale.CHD severity and comorbidities varied across sites, with up to 20% of adults having severe CHD and >50% having ≥1 additional cardiovascular comorbidity. Most adults had ≥1 outpatient encounters (80% EU, 90% Massachusetts, and 53% New York). Insurance type differed across sites, with Massachusetts having a large proportion of Medicaid (75%) and EU and New York having large proportions of private insurance (44% EU, 67% New York). Estimated proportions of adults with CHD-coded health care encounters varied greatly by location, with 1.2 (EU), 10 (Massachusetts), and 0.6 (New York) per 1,000 adults based on 2010 census data.This was the first surveillance effort of adults with CHD-coded inpatient and outpatient health care encounters in 3 U.S. geographic locations using both administrative and clinical data sources. This information will provide a clearer understanding of health care use in this growing population.

    View details for DOI 10.1016/j.jacc.2020.05.025

    View details for PubMedID 32646567

  • Assessing Pregnancy, Gestational Complications, and Co-morbidities in Women With Congenital Heart Defects (Data from ICD-9-CM Codes in 3 US Surveillance Sites). The American journal of cardiology Raskind-Hood, C., Saraf, A., Riehle-Colarusso, T., Glidewell, J., Gurvitz, M., Dunn, J. E., Lui, G. K., Van Zutphen, A., McGarry, C., Hogue, C. J., Hoffman, T., Rodriguez Iii, F. H., Book, W. M. 2019


    Improved treatment of congenital heart defects (CHDs) has resulted in women with CHDs living to childbearing age. However, no US population-based systems exist to estimate pregnancy frequency or complications among women with CHDs. Cases were identified in multiple data sources from 3 surveillance sites: Emory University (EU) whose catchment area included 5 metropolitan Atlanta counties; Massachusetts Department of Public Health (MA) whose catchment area was statewide; and New York State Department of Health (NY) whose catchment area included 11 counties. Cases were categorized into one of 5 mutually exclusive CHD severity groups collapsed to severe versus not severe; specific ICD-9-CM codes were used to capture pregnancy, gestational complications, and nongestational co-morbidities in women, age 11 to 50 years, with a CHD-related ICD-9-CM code. Pregnancy, CHD severity, demographics, gestational complications, co-morbidities, and insurance status were evaluated. ICD-9-CM codes identified 26,655 women with CHDs, of whom 5,672 (21.3%, range: 12.8% in NY to 22.5% in MA) had codes indicating a pregnancy. Over 3 years, age-adjusted proportion pregnancy rates among women with severe CHDs ranged from 10.0% to 24.6%, and 14.2% to 21.7% for women with nonsevere CHDs. Pregnant women with CHDs of any severity, compared with nonpregnant women with CHDs, reported more noncardiovascular co-morbidities. Insurance type varied by site and pregnancy status. These US population-based, multisite estimates of pregnancy among women with CHD indicate a substantial number of women with CHDs may be experiencing pregnancy and complications. In conclusion, given the growing adult population with CHDs, reproductive health of women with CHD is an important public health issue.

    View details for DOI 10.1016/j.amjcard.2019.12.001

    View details for PubMedID 31902476

  • Secondary repair of incompetent pulmonary valves after previous surgery or intervention: Patient selection and outcomes. The Journal of thoracic and cardiovascular surgery Adamson, G. T., McElhinney, D. B., Lui, G., Meadows, A. K., Rigdon, J., Hanley, F. L., Maskatia, S. A. 2019


    OBJECTIVES: Pulmonary valve (PV) regurgitation (PR) is common after intervention for a hypoplastic right ventricular outflow tract. Secondary PV repair is an alternative to replacement (PVR), but selection criteria are not established. We sought to elucidate preoperative variables associated with successful PV repair and to compare outcomes between repair and PVR.METHODS: Patients who underwent surgery for secondary PR from 2010 to 2017 by a single surgeon were studied. The PV annulus and leaflets were measured on the preoperative echocardiogram and magnetic resonance images, and the primary predictor variable was leaflet area indexed to ideal PV annulus area (iPLA) by magnetic resonance imaging. PV repair and PVR groups were compared using multivariable logistic regression, and with a conditional inference tree. Freedom from PV dysfunction and from reintervention were assessed with Kaplan-Meier survival analyses.RESULTS: Of 85 patients, 31 (36%) underwent PV repair. By multivariable analysis, longer PV total leaflet length (cm/m2) (beta=3.00, standard error [SE]=0.82, P<.001), larger PV z score (beta=1.34, SE=0.39, P=.001), and larger iPLA (beta=8.13, SE=2.62, P=.002) were associated with repair. iPLA of 0.90 or greater was 91% sensitive and 83% specific for achieving PV repair. At a median of 4.1years follow-up, there was greater freedom from significant PR in the PV repair group (log rank P=.008).CONCLUSIONS: Patients with an iPLA >0.9, and those with an iPLA between 0.7 and 0.9 with a PV annulus z score >0 should be considered for a native PV repair. At midterm follow-up, patients with a PV repair were not more likely to developPR or to require reintervention when compared with patients undergoing PVR.

    View details for DOI 10.1016/j.jtcvs.2019.06.110

    View details for PubMedID 31585750

  • 2019 ACC/AHA/ASE Advanced Training Statement on Echocardiography (Revision of the 2003 ACC/AHA Clinical Competence Statement on Echocardiography): A Report of the ACC Competency Management Committee JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Wiegers, S. E., Ryan, T., Arrighi, J. A., Brown, S. M., Canaday, B., Damp, J. B., Diaz-Gomez, J. L., Figueredo, V. M., Garcia, M. J., Gillam, L. D., Griffin, B. P., Kirkpatrick, J. N., Klarich, K. W., Lui, G. K., Maffett, S., Naqvi, T. Z., Patel, A. R., Poulin, M., Rose, G. A., Swaminathan, M., Mendes, L. A., Adams, J. E., Brush, J. E., Dec, G., Denktas, A., Fernandes, S., Freeman, R., Hahn, R. T., Halperin, J. L., Housholder-Hughes, S. D., Khan, S. S., Lin, H., Marine, J. E., McPherson, J. A., Niazi, K., Solomon, M. A., Spicer, R. L., Tam, M., Wang, A., Weissman, G., Weitz, H. H., Williams, E. S., Writing Comm Members, ACC Competency Management Comm 2019; 32 (8): 919-+

    View details for DOI 10.1016/j.echo.2019.04.002

    View details for Web of Science ID 000478014600002

    View details for PubMedID 31378259

  • 2019 ACC/AHA/ASE advanced training statement on echocardiography (revision of the 2003 ACC/AHA clinical competence statement on echocardiography) A Report of the ACC Competency Management Committee CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Wiegers, S. E., Ryan, T., Arrighi, J. A., Brown, S. M., Canaday, B., Damp, J. B., Diaz-Gomez, J. L., Figueredo, V. M., Garcia, M. J., Gillam, L. D., Griffin, B. P., Kirkpatrick, J. N., Klarich, K. W., Lui, G. K., Maffett, S., Naqvi, T. Z., Patel, A. R., Poulin, M., Rose, G. A., Swaminathan, M., Mendes, L. A., Adams, J. E., Brush, J. E., Dec, G., Denktas, A., Fernandes, S., Freeman, R., Hahn, R. T., Halperin, J. L., Housholder-Hughes, S. D., Khan, S. S., Lin, C., Marine, J. E., McPherson, J. A., Niazi, K., Solomon, M. A., Spicer, R. L., Tam, M., Wang, A., Weissman, G., Weitz, H. H., Williams, E. S., ACC Competency Management Comm, Amer Thoracic Soc, Soc Cardiovasc Angiography Inter, Soc Cardiovasc Anesthesiologists, Soc Critical Care Med 2019

    View details for DOI 10.1002/ccd.28313

    View details for Web of Science ID 000476369600001

    View details for PubMedID 31313449

  • Surveillance of Congenital Heart Defects among Adolescents at Three US Sites AMERICAN JOURNAL OF CARDIOLOGY Lui, G. K., McGarry, C., Bhatt, A., Book, W., Riehle-Colarusso, T. J., Dunn, J. E., Glidewell, J., Gurvitz, M., Hoffman, T., Hogue, C. J., Hsu, D., Obenhaus, S., Raskind-Hood, C., Rodriguez, F. H., Zaidi, A., Van Zutphen, A. R. 2019; 124 (1): 137–43
  • 2019 ACC/AHA/ASE Advanced Training Statement on Echocardiography (Revision of the 2003 ACC/AHA Clinical Competence Statement on Echocardiography): A Report of the ACC Competency Management Committee CIRCULATION-CARDIOVASCULAR IMAGING Wiegers, S. E., Ryan, T., Arrighi, J. A., Brown, S. M., Canaday, B., Damp, J. B., Diaz-Gomez, J. L., Figueredo, V. M., Garcia, M. J., Gillam, L. D., Griffin, B. P., Kirkpatrick, J. N., Klarich, K. W., Lui, G. K., Maffett, S., Naqvi, T. Z., Patel, A. R., Poulin, M., Rose, G. A., Swaminathan, M. 2019; 12 (7)
  • Short-term outcomes of en bloc combined heart and liver transplantation in the failing Fontan CLINICAL TRANSPLANTATION Vaikunth, S. S., Concepcion, W., Daugherty, T., Fowler, M., Lutchman, G., Maeda, K., Rosenthal, D. N., Teuteberg, J., Woo, Y., Lui, G. K. 2019; 33 (6)

    View details for DOI 10.1111/ctr.13540

    View details for Web of Science ID 000473087200014

  • Maternal Comorbidities and Complications of Delivery in Pregnant Women With Congenital Heart Disease JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Schlichting, L. E., Insaf, T. Z., Zaidi, A. N., Lui, G. K., Van Zutphen, A. R. 2019; 73 (17): 2181–91
  • Extended cardiac ambulatory rhythm monitoring in adults with congenital heart disease: Arrhythmia detection and impact of extended monitoring CONGENITAL HEART DISEASE Schultz, K. E., Lui, G. K., McElhinney, D. B., Long, J., Balasubramanian, V., Sakarovitch, C., Fernandes, S. M., Dubin, A. M., Rogers, I. S., Romfh, A. W., Motonaga, K. S., Viswanathan, M. N., Ceresnak, S. R. 2019; 14 (3): 410–18

    View details for DOI 10.1111/chd.12736

    View details for Web of Science ID 000471070900013

  • Echocardiographic Evaluation of Patients Undergoing Transcatheter Tricuspid Valve-In-Valve Replacement JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Tompkins, R., Kelle, A. M., Cabalka, A. K., Lui, G. K., Aboulhosn, J., Dvir, D., McElhinney, D. B., VIVID Registry 2019; 32 (5): 616–23
  • Substantial Cardiovascular Morbidity in Adults With Lower-Complexity Congenital Heart Disease CIRCULATION Saha, P., Potiny, P., Rigdon, J., Morello, M., Tcheandjieu, C., Romfh, A., Fernandes, S. M., McElhinney, D. B., Bernstein, D., Lui, G. K., Shaw, G. M., Ingelsson, E., Priest, J. R. 2019; 139 (16): 1889–99
  • Repair of Untreated Older Patients With Tetralogy of Fallot With Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Vaikunth, S. S., Bauser-Heaton, H., Lui, G. K., Wise-Faberowski, L., Chan, F. P., Asija, R., Hanley, F. L., McElhinney, D. B. 2019; 107 (4): 1218–25
  • Transcatheter redirection of hepatic venous blood to treat unilateral pulmonary arteriovenous malformations in a Fontan circulation by short-term total exclusion of the unaffected lung CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Adamson, G. T., Peng, L. F., Lui, G. K., Perry, S. B. 2019; 93 (4): 660–63

    View details for DOI 10.1002/ccd.28073

    View details for Web of Science ID 000461016400030

  • Predicting acquired cardiovascular disease in adults with congenital heart disease is risky business INTERNATIONAL JOURNAL OF CARDIOLOGY Lui, G. K., Bhatt, A. B. 2019; 277: 106–7
  • Guidelines for Performing a Comprehensive Transesophageal Echocardiographic Examination in Children and All Patients with Congenital Heart Disease: Recommendations from the American Society of Echocardiography JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Puchalski, M. D., Lui, G. K., Miller-Hance, W. C., Brook, M. M., Young, L. T., Bhat, A., Roberson, D. A., Mercer-Rosa, L., Miller, O. I., Parra, D. A., Burch, T., Carron, H. D., Wong, P. C. 2019; 32 (2): 173–215
  • Heart failure with reduced and preserved ejection fraction in adult congenital heart disease. Heart failure reviews Vaikunth, S. S., Lui, G. K. 2019


    Heart failure with reduced ejection fraction (HFrEF) is common in patients with adult congenital heart disease. Many of the most common congenital defects have a high prevalence of HFrEF, including left-sided obstructive lesions (aortic stenosis, coarctation of the aorta, Shone complex), tetralogy of Fallot, Ebstein anomaly, lesions in which there is a systemic right ventricle, and lesions palliated with a Fontan circulation. However, heart failure with preserved ejection fraction (HFpEF) is also prevalent in all these lesions. Comprehensive evaluation includes physical exam, biomarkers, echocardiography and advanced imaging, exercise stress testing, and, in some cases, invasive hemodynamics. Guideline-directed medical therapy for HFrEF can be applied to left-sided lesions and may be considered on an individual basis for systemic right ventricle and single-ventricle patients. Medical therapy is limited for HFpEF. However, in both HFrEF and HFpEF, ventricular dyssynchrony and arrhythmias play an important role, and medications for rhythm control, ablation, and cardiac resynchronization therapy should be considered. Finally, aggressive management of cardiovascular risk factors and comorbidities, including, but not limited to, hypertension, obesity, diabetes, dyslipidemia, and obstructive sleep apnea, cannot be overemphasized.

    View details for DOI 10.1007/s10741-019-09904-z

    View details for PubMedID 31873841

  • Fontan Liver Lesions: Not Always HCC. JACC. Case reports Haeffele, C., Aggarwal, A., Lutchman, G., Veldtman, G. R., Wu, F. M., Lui, G. K. 2019; 1 (2): 175-178


    A 24-year-old Fontan procedure patient underwent surveillance liver cardiac magnetic resonance imaging. Findings were suggestive of hepatocellular carcinoma (HCC). Currently, HCC is diagnosed based on imaging alone. Given her otherwise reassuring clinical profile, she underwent liver biopsy. Pathology demonstrated focal nodular hyperplasia. This raises concern for overdiagnosis of HCC in Fontan patients without tissue confirmation. (Level of Difficulty: Advanced.).

    View details for DOI 10.1016/j.jaccas.2019.05.031

    View details for PubMedID 34316779

    View details for PubMedCentralID PMC8301494

  • Commentary: Risk prediction model on adult congenital heart surgery. And more…. The Journal of thoracic and cardiovascular surgery Maeda, K. n., Lui, G. K. 2019

    View details for DOI 10.1016/j.jtcvs.2019.08.111

    View details for PubMedID 31669012

  • Surgical Repair of Ebstein's Anomaly Utilizing A Bicuspidization Approach. The Annals of thoracic surgery Mainwaring, R. D., Rosenblatt, T. R., Lui, G. K., Ma, M. n., Hanley, F. L. 2019


    Ebstein's anomaly of the tricuspid valve is a rare and heterogeneous form of congenital heart defect. The purpose of this study was to review the surgical experience with Ebstein's anomaly at a single institution.This was a retrospective review of 47 patients with Ebstein's anomaly who were previously unrepaired and underwent surgical repair of the tricuspid valve. Our surgical approach repairs the leaflets at the pre-existing level and does not attempt to reposition the hingepoints at the anatomic annulus. The median age at surgery was 17 years (range 2 to 53 years). Pre-operatively, the median degree of tricuspid regurgitation was graded as moderate-to-severe and the median right ventricular function was slightly below normal.Forty-seven patients underwent surgical repair without any mortality. Forty-three of the 47 patients (91%) had a successful repair as evidenced by a decrease in the amount of tricuspid regurgitation to trace/mild. Four patients (9%) had an unsuccessful initial repair. Thirteen of the 47 (29%) underwent a concomitant bi-directional Glenn procedure. There were 8 patients (17%) who have required late re-operation. Seven of these 8 patients initially had undergone a successful repair but returned with recurrent tricuspid regurgitation. Six of the 8 late re-operations entailed a re-repair, while two (4%) required ; tricuspid valve replacement.The bicuspidization surgical technique resulted in 91% percent of patients undergoing a successful initial repair. These results suggest this technique may be a viable alternative to the "cone" procedure.

    View details for DOI 10.1016/j.athoracsur.2019.06.026

    View details for PubMedID 31394091

  • Risk of cardiac tachyarrhythmia in patients with repaired tetralogy of Fallot: a multicenter cardiac MRI based study INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING Beurskens, N. G., Hagdorn, Q. J., Gorter, T. M., Berger, R. F., Vermeulen, K. M., van Melle, J. P., Ebels, T. E., Lui, G. K., Ceresnak, S. R., Chan, F. P., Willems, T. P. 2019; 35 (1): 143–51


    Cardiac tachyarrhythmias are the leading cause of morbidity and mortality in patients with repaired tetralogy of Fallot (TOF). We evaluated risk factors for sustained ventricular tachyarrhythmia (VT) and atrial tachyarrhythmia (ATA) in these patients. Patients (n = 319) who underwent cardiac magnetic resonance (CMR) imaging at two tertiary centers between 2007 and 2016 were assessed. Potential risk markers, based on history, cardiac magnetic resonance imaging (CMR), electrocardiography (ECG) and echocardiography, were analyzed for prediction of the primary endpoint of VT, and the secondary endpoint of ATA. During a follow-up of 3.5 (0.9-6.1) years, 20 (6.3%) patients reached the primary endpoint, and 30 (9.4%) the secondary endpoint. Multivariable cox hazards regression identified right ventricular (RV) end-diastolic volume (Hazard ratio [HR] 2.03, per 10 ml/m2 increase; p = 0.02), RV end-systolic volume (HR 3.04, per 10 ml/m2 increase; p = 0.04), RV mass (HR 1.88, per 10 g/m2 increase; p = 0.02), and RV ejection fraction (HR 6.06, per 10% decrease; p = 0.02) derived from CMR to be independent risk factors of VT. In addition, QRS-duration (HR 1.70, per 10 ms increase; p = 0.001) and body mass index (BMI: HR 1.8, per 5 kg/m2 increase; p = 0.02) were independent markers of VT. Older age at TOF repair (HR 1.33, per 2 months increase; p = 0.03) and BMI (HR 1.76, per 5 kg/m2 increase; p < 0.001) independently predicted ATA. RV systolic dysfunction, hypertrophy and dilatation on CMR, together with QRS prolongation, and obesity are predictive of VT in TOF patients. Older age at TOF repair and obesity were associated with the occurrence of ATA.

    View details for PubMedID 30094564

  • Transcatheter redirection of hepatic venous blood to treat unilateral pulmonary arteriovenous malformations in a Fontan circulation by short-term total exclusion of the unaffected lung. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Adamson, G. T., Peng, L. F., Lui, G. K., Perry, S. B. 2019


    Clinically significant unilateral pulmonary arteriovenous malformations (PAVM) can develop in patients with a Fontan circulation when there is unbalanced distribution of hepatic venous (HV) blood flow to the lungs. There are reported surgical and transcatheter techniques to treat PAVMs by rerouting HV return, with promising short-term results. We report a case of a novel, technically simple transcatheter approach to redirect HV blood flow in an adult Fontan patient with polysplenia syndrome and severe unilateral PAVMs. Our patient had a two-stage procedure, the first to redirect all HV blood flow to the affected lung with a single covered stent, and a second to confirm resolution of PAVMs and to reintroduce HV effluent to the unaffected lung. At 10-month follow-up, her oxygen saturations had increased from 75% to 93% with a marked improvement in her functional status.

    View details for PubMedID 30629315

  • Guidelines for Performing a Comprehensive Transesophageal Echocardiographic: Examination in Children and All Patients with Congenital Heart Disease: Recommendations from the American Society of Echocardiography. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Puchalski, M. D., Lui, G. K., Miller-Hance, W. C., Brook, M. M., Young, L. T., Bhat, A., Roberson, D. A., Mercer-Rosa, L., Miller, O. I., Parra, D. A., Burch, T., Carron, H. D., Wong, P. C. 2018

    View details for PubMedID 30579694

  • Repair of Untreated Older Patients with Tetralogy of Fallot with Major Aortopulmonary Collaterals. The Annals of thoracic surgery Vaikunth, S., Bauser-Heaton, H., Lui, G. K., Wise-Faberowski, L., Chan, F. P., Asija, R., Hanley, F. L., McElhinney, D. B. 2018


    BACKGROUND: Our programmatic approach to tetralogy of Fallot with major aortopulmonary collaterals emphasizes single-stage unifocalization with complete intracardiac repair during infancy. Little is known about suitability for complete repair in patients beyond infancy. We sought to analyze outcomes of our approach in older patients with previously untreated tetralogy of Fallot with major aortopulmonary collaterals.METHODS: Any patient with this lesion not treated prior to 2 years of age referred to our center from 2002-17 met inclusion criteria.RESULTS: Of 33 patients, 32 were out-of-state (64% international) referrals, and 33% (n=11) were >9 years old, had polycythemia, and/or at least 1 high pressure collateral (>25 mm Hg). Complete repair was achieved in 94% (n=31) of patients, 82% (n=27) in one stage and 12% (n=4) after unifocalization to a central shunt. The median right ventricular to aortic pressure ratio was 0.31 after surgery and 0.37 at follow-up. At a median of 4.8 years after repair, 9 patients (19%) underwent reintervention, including 5 conduit and 7 branch pulmonary artery interventions. Three patients also underwent aortic valve replacement.CONCLUSIONS: In this selected cohort of older patients with previously unoperated tetralogy of Fallot with major aortopulmonary collaterals, outcomes were comparable to infants undergoing treatment according to our approach. These findings support the notion that patients who are either born in low-resource settings or present to healthcare providers beyond infancy should be considered candidates and evaluated for complete repair.

    View details for PubMedID 30550802

  • Ventricular mechanics in adolescent and adult patients with a Fontan circulation: Relation to geometry and wall stress ECHOCARDIOGRAPHY-A JOURNAL OF CARDIOVASCULAR ULTRASOUND AND ALLIED TECHNIQUES Rosner, A., Khalapyan, T., Pedrosa, J., Dalen, H., McElhinney, D. B., Friedberg, M. K., Lui, G. K. 2018; 35 (12): 2035–46

    View details for DOI 10.1111/echo.14169

    View details for Web of Science ID 000451879200016

  • Population-based surveillance of congenital heart defects among adolescents and adults: surveillance methodology BIRTH DEFECTS RESEARCH Glidewell, J., Book, W., Raskind-Hood, C., Hogue, C., Dunn, J. E., Gurvitz, M., Ozonoff, A., McGarry, C., Van Zutphen, A., Lui, G., Downing, K., Riehle-Colarusso, T. 2018; 110 (19): 1395–1403

    View details for DOI 10.1002/bdr2.1400

    View details for Web of Science ID 000451387600004

  • Secondary Repair of Incompetent Pulmonary Valves After Prior Surgery or Intervention: Patient Selection and Outcomes Adamson, G. T., McElhinney, D. B., Lui, G., Meadows, A. K., Hanley, F. L., Maskatia, S. A. LIPPINCOTT WILLIAMS & WILKINS. 2018
  • Introducing Advance Care Planning Into the Transition Process: The ACHD Patient Perspective Edwards, L. A., Hansen, K., Sillman, C., Dong, E., Scribner, C., Romfh, A., Lui, G. K., Fernandes, S. M. LIPPINCOTT WILLIAMS & WILKINS. 2018
  • Ventricular mechanics in adolescent and adult patients with a Fontan circulation: Relation to geometry and wall stress. Echocardiography (Mount Kisco, N.Y.) Rosner, A., Khalapyan, T., Pedrosa, J., Dalen, H., McElhinney, D. B., Friedberg, M. K., Lui, G. K. 2018


    BACKGROUND: Patients with single ventricle physiology and Fontan circulation are at increased risk for late complications and reduced survival. The aim of the study was to investigate the correlation between ventricular geometry and systolic regional function in different underlying anatomic conditions in adolescent and adult Fontan-palliated patients.METHOD: In a retrospective cross-sectional study, we measured 2D strain, ventricular diameters, ventricular volumes, ejection fraction (EF), global and segmental wall stress, and sphericity index. The same analyses were performed in 99 age- and gender-matched healthy individuals.RESULTS: One hundred and one patients were included at a mean age of 21 (range 14-59) years. In comparison with healthy subjects, patients with Fontan circulation displayed larger ventricular volumes (153±78mL vs 116±38mL P<0.05), reduced EF (43%±15% vs 55%±8% P<0.05), reduced longitudinal (-13%±6% vs -21%±4% P<0.05) and circumferential strain values (-15%±7% vs -22%±4% P<0.05). Functionally single ventricles were more spherical (ratio of longitudinal to short-axis diameters 1.3±0.3 vs 1.7±0.2 P<0.05). Circumferential strain correlated well with global wall stress and the degree of sphericity (R2 =0.320), while segmental strain did not correlate with segmental wall stress. The percentage of segments with akinesia was relatively high (16±16% vs 0±0% P<0.05) indicating reduced segmental contractile function.CONCLUSION: Functionally single ventricles after Fontan palliation have significantly reduced systolic regional and global function with a high intersegmental inhomogeneity. The underlying pathological mechanisms might be multifactorial, including ventricular geometry, sphericity, and regional contractile properties. Future studies are needed to investigate the role of ventricular geometry for clinical performance and outcome.

    View details for PubMedID 30350438

  • Predicting acquired cardiovascular disease in adults with congenital heart disease is risky business. International journal of cardiology Lui, G. K., Bhatt, A. B. 2018

    View details for PubMedID 30482629

  • Applying current normative data to prognosis in heart failure: The Fitness Registry and the Importance of Exercise National Database (FRIEND) INTERNATIONAL JOURNAL OF CARDIOLOGY Moneghetti, K. J., Hock, J., Kaminsky, L., Arena, R., Lui, G. K., Haddad, F., Wheeler, M., Froelicher, V., Ashley, E., Myers, J., Christle, J. W. 2018; 263: 75–79


    Percent of predicted peak VO2 (ppVO2) is considered a standard measure for establishing disease severity, however, there are known limitations to traditional normative values. This study sought to compare ppVO2 from the newly derived "Fitness Registry and the Importance of Exercise: a National Database" (FRIEND) registry equation to conventional prediction equations in a clinical cohort of patients undergoing cardiopulmonary exercise testing (CPX).We selected 1094 patients referred for evaluation of heart failure (HF) symptoms who underwent CPX. ppVO2 was calculated using the FRIEND, Wasserman/Hansen and Jones equations. Participants were followed for a median of 4.5 years [Interquartile range 3.5-6.0] for the composite endpoint of death, advanced HF therapy, or acute decompensated HF requiring hospital admission. Mean age was 48 ± 15 years and 62% were female. The FRIEND registry equation predicted the lowest ppVO2 (measured/predicted; 71 ± 31%), compared to the Wasserman/Hansen (74 ± 29%) and Jones equations (83 ± 33%) (p < 0.001). All expressions of peak VO2 were significant as univariate predictors of outcome with no significant differences between equations on pairwise analysis of receiver operating characteristic curves. When compared at a similar threshold of ppVO2 the event rate was significantly lower in the FRIEND registry equation versus the currently used Wasserman and Jones equations.The use of the newly derived FRIEND registry equation predicts HF outcomes; however, it appears to predict a higher predicted VO2; the clinical implication being a lower threshold of percent predicted peak VO2 should be considered when risk stratifying patients with HF.

    View details for PubMedID 29525067

  • Classic-Pattern Dyssynchrony in Adolescents and Adults With a Fontan Circulation. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Rösner, A. n., Khalapyan, T. n., Dalen, H. n., McElhinney, D. B., Friedberg, M. K., Lui, G. K. 2018; 31 (2): 211–19


    Previous studies have suggested the presence of dyssynchrony in the functionally single ventricle. The aim of this study was to investigate the presence of classic-pattern dyssynchrony (CPD), characterized by typical early and late deformation of opposite walls, and its relation to QRS duration and myocardial function in patients with single-ventricle physiology after Fontan palliation.In a retrospective cross-sectional study, 101 adolescent and adult patients with single-ventricle physiology after the Fontan procedure were investigated. Strain curves were visually assessed for the presence of CPD. Systolic and diastolic function were assessed using echocardiography.One hundred one patients were included, with varying anatomic morphology: two sizable ventricular components (n = 21), right dominant (n = 21), left dominant (n = 49), and undefined anatomy (n = 10). Fifteen of 101 Fontan patients had CPD. Forty-three percent of patients with two sizable ventricular masses displayed CPD, mostly with prolonged QRS, while the number of patients with CPD with right-dominant (9%) and left-dominant (6%) morphology was significantly lower (P = .016). Those with CPD displayed significantly (P < .05) larger QRS widths (142 ± 22 vs 112 ± 24 msec), lower ejection fractions (31 ± 14% vs 45 ± 14%), lower global early diastolic strain rates (0.7 ± 0.5 vs 1.2 ± 0.8 sec-1), and global systolic circumferential (-10 ± 5% vs -16 ± 7%) and longitudinal (-9 ± 5% vs -14 ± 5%) strain, respectively.CPD is present in a proportion of adolescent and adult patients after Fontan palliation. The presence of CPD is associated with reduced systolic and diastolic function compared with Fontan patients without CPD. Because the presence of CPD appears to be a promising predictor for response to cardiac resynchronization therapy in patients with biventricular circulation, these findings may have important potential for prospective evaluation of cardiac resynchronization therapy in patients with univentricular circulation.

    View details for PubMedID 29229494

  • Population-based surveillance of congenital heart defects among adolescents and adults: surveillance methodology. Birth defects research Glidewell, J. n., Book, W. n., Raskind-Hood, C. n., Hogue, C. n., Dunn, J. E., Gurvitz, M. n., Ozonoff, A. n., McGarry, C. n., Van Zutphen, A. n., Lui, G. n., Downing, K. n., Riehle-Colarusso, T. n. 2018


    Improved treatment of congenital heart defects (CHDs) has increased survival of persons with CHDs; however, no U.S. population-based systems exist to assess prevalence, healthcare utilization, or longer-term outcomes among adolescents and adults with CHDs.Novel approaches identified individuals aged 11-64 years who received healthcare with ICD-9-CM codes for CHDs at three sites: Emory University in Atlanta, Georgia (EU), Massachusetts Department of Public Health (MA), New York State Department of Health (NY) between January 1, 2008 (2009 for MA) and December 31, 2010. Case-finding sources included outpatient clinics; Medicaid and other claims data; and hospital inpatient, outpatient, and emergency visit data. Supplemental information came from state vital records (EU, MA), and birth defects registries (EU, NY). Demographics and diagnostic and procedural codes were linked, de-duplicated, and shared in a de-identified dataset. Cases were categorized into one of five mutually exclusive CHD severity groups; non-cardiac comorbidity codes were grouped into broad categories.73,112 individuals with CHD codes in healthcare encounters were identified. Primary data source type varied: clinics (EU, NY for adolescents), claims (MA), hospital (NY for adults). There was a high rate of missing data for some variables and data varied in format and quality. Some diagnostic codes had poor specificity for CHD ascertainment.To our knowledge, this is the first population-based, multi-site CHD surveillance among adolescents and adults in the U.S. Identification of people living with CHDs through healthcare encounters using multiple data sources was feasible, though data quality varied and linkage/de-duplication was labor-intensive.

    View details for PubMedID 30394691

  • Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association. Circulation Lui, G. K., Saidi, A., Bhatt, A. B., Burchill, L. J., Deen, J. F., Earing, M. G., Gewitz, M., Ginns, J., Kay, J. D., Kim, Y. Y., Kovacs, A. H., Krieger, E. V., Wu, F. M., Yoo, S. J. 2017


    Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.

    View details for DOI 10.1161/CIR.0000000000000535

    View details for PubMedID 28993401

  • Risk Estimates for Atherosclerotic Cardiovascular Disease in Adults With Congenital Heart Disease AMERICAN JOURNAL OF CARDIOLOGY Lui, G. K., Rogers, I. S., Ding, V. Y., Hedlin, H. K., MacMillen, K., Maron, D. J., Sillman, C., Romfh, A., Dade, T. C., Haeffele, C., Grady, S. R., McElhinney, D. B., Murphy, D. J., Fernandes, S. M. 2017; 119 (1): 112-118


    The adult with congenital heart disease (CHD) is at risk of developing atherosclerotic cardiovascular disease (ASCVD). We performed a cross-sectional study to describe established ASCVD risk factors and estimate 10-year and lifetime risk of ASCVD in adults over age 18 with CHD of moderate or great complexity using 3 validated risk assessment tools-the Framingham Study Cardiovascular Disease Risk Assessment, the Reynolds Risk Score, and the ASCVD Risk Estimator. We obtained extensive clinical and survey data on 178 enrolled patients, with average age 37.1 ± 12.6 years, 51% men. At least 1 modifiable ASCVD risk factor was present in 70%; the 2 most common were overweight/obesity (53%) and systemic hypertension (24%). Laboratory data were available in 103 of the 178 patients. Abnormal levels of glycated hemoglobin, high-sensitivity C-reactive protein, and high-density lipoprotein were each found in around 30% of patients. The 10-year ASCVD predicted risk using all 3 tools was relatively low (i.e., at least 90% of patients <10% risk), yet the median estimated lifetime risk was 36%. In conclusion, ASCVD risk factors are prevalent in adults with CHD. The risk estimation tools suggest that this population is particularly vulnerable to ASCVD with aging and should undergo guideline-based screening and management of modifiable risk factors.

    View details for DOI 10.1016/j.amjcard.2016.09.023

    View details for PubMedID 28247847

  • An Analysis of Left Ventricular Retraining in Patients With Dextro- and Levo-Transposition of the Great Arteries. The Annals of thoracic surgery Mainwaring, R. D., Patrick, W. L., Ibrahimiye, A. N., Watanabe, N. n., Lui, G. K., Hanley, F. L. 2017


    Patients with dextro (D)-transposition of the great arteries (TGA) who have undergone a previous atrial switch and for some patients with levo (L)-TGA (ie, no ventricular septal defect or outflow tract obstruction), the left ventricle (LV) may require retraining before late arterial switch. The purpose of this study was to analyze the results of LV retraining for these two entities.This was a retrospective review of 51 patients enrolled in an LV retraining program. There were 25 patients with D-TGA and 26 with L-TGA. The median age of the D-TGA patients was 15 years, and 22 of 25 were in New York Heart Association class III or IV. The median age of the L-TGA patients was 12 months, and 3 of 26 patients were in New York Heart Association class III or IV.LV retraining was successful in 13 of the 25 patients (52%) with D-TGA, and 10 these 13 patients (77%) underwent successful late arterial switch. Of the 26 patients with L-TGA, LV retraining was successful in 24 (92%), and a double-switch operation was successful in 19 of 19 (100%) who subsequently underwent that procedure. A mathematical formula based on the incremental gain in left-to-right ventricular pressure ratio correctly predicted the success or failure in 94% of the patients.The data demonstrate differences in the success of LV retraining and late arterial switch for D-TGA and L-TGA. We would propose that the LV retraining ratio may be useful in objectively selecting patients eligible for late arterial switch.

    View details for PubMedID 29274314

  • Proximity to Pediatric Cardiac Surgical Care among Adolescents with Congenital Heart Defects in 11 New York Counties. Birth defects research Sommerhalter, K. M., Insaf, T. Z., Akkaya-Hocagil, T. n., McGarry, C. E., Farr, S. L., Downing, K. F., Lui, G. K., Zaidi, A. N., Van Zutphen, A. R. 2017; 109 (18): 1494–1503


    Many individuals with congenital heart defects (CHDs) discontinue cardiac care in adolescence, putting them at risk of adverse health outcomes. Because geographic barriers may contribute to cessation of care, we sought to characterize geographic access to comprehensive cardiac care among adolescents with CHDs.Using a population-based, 11-county surveillance system of CHDs in New York, we characterized proximity to the nearest pediatric cardiac surgical care center among adolescents aged 11 to 19 years with CHDs. Residential addresses were extracted from surveillance records documenting 2008 to 2010 healthcare encounters. Addresses were geocoded using ArcGIS and the New York State Street and Address Maintenance Program, a statewide address point database. One-way drive and public transit time from residence to nearest center were calculated using R packages gmapsdistance and rgeos with the Google Maps Distance Matrix application programming interface. A marginal model was constructed to identify predictors associated with one-way travel time.We identified 2522 adolescents with 3058 corresponding residential addresses and 12 pediatric cardiac surgical care centers. The median drive time from residence to nearest center was 18.3 min, and drive time was 30 min or less for 2475 (80.9%) addresses. Predicted drive time was longest for rural western addresses in high poverty census tracts (68.7 min). Public transit was available for most residences in urban areas but for few in rural areas.We identified areas with geographic barriers to surgical care. Future research is needed to determine how these barriers influence continuity of care among adolescents with CHDs. Birth Defects Research 109:1494-1503, 2017.© 2017 Wiley Periodicals, Inc.

    View details for DOI 10.1002/bdr2.1129

    View details for PubMedID 29152921

  • Dextro-Transposition of the Great Arteries Long-term Sequelae of Atrial and Arterial Switch CARDIOLOGY CLINICS Haeffele, C., Lui, G. K. 2015; 33 (4): 543-?


    Over the last 50 years, improved surgical techniques and progressive medical management have allowed patients with complete or dextro-transposition of the great arteries (D-TGA) to survive into adulthood. Older adult patients underwent an atrial switch procedure (Mustard or Senning operation), whereas the younger cohort of patients with TGA has undergone the arterial switch operation (ASO). The Mustard/Senning maintains the right ventricle as the systemic ventricle, whereas the more recently adopted ASO attempts to restore normal physiologic and anatomic relationships. Neither operation is without consequence. Neither is without consequence and require long term follow up.

    View details for DOI 10.1016/j.ccl.2015.07.012

    View details for Web of Science ID 000364729200006

    View details for PubMedID 26471819

  • A Rapid, High-Quality, Cost-Effective, Comprehensive and Expandable Targeted Next-Generation Sequencing Assay for Inherited Heart Diseases. Circulation research Wilson, K. D., Shen, P., Fung, E., Karakikes, I., Zhang, A., Inanloorahatloo, K., Odegaard, J., Sallam, K., Davis, R. W., Lui, G. K., Ashley, E. A., Scharfe, C., Wu, J. C. 2015; 117 (7): 603-611


    Thousands of mutations across >50 genes have been implicated in inherited cardiomyopathies. However, options for sequencing this rapidly evolving gene set are limited because many sequencing services and off-the-shelf kits suffer from slow turnaround, inefficient capture of genomic DNA, and high cost. Furthermore, customization of these assays to cover emerging targets that suit individual needs is often expensive and time consuming.We sought to develop a custom high throughput, clinical-grade next-generation sequencing assay for detecting cardiac disease gene mutations with improved accuracy, flexibility, turnaround, and cost.We used double-stranded probes (complementary long padlock probes), an inexpensive and customizable capture technology, to efficiently capture and amplify the entire coding region and flanking intronic and regulatory sequences of 88 genes and 40 microRNAs associated with inherited cardiomyopathies, congenital heart disease, and cardiac development. Multiplexing 11 samples per sequencing run resulted in a mean base pair coverage of 420, of which 97% had >20× coverage and >99% were concordant with known heterozygous single nucleotide polymorphisms. The assay correctly detected germline variants in 24 individuals and revealed several polymorphic regions in miR-499. Total run time was 3 days at an approximate cost of $100 per sample.Accurate, high-throughput detection of mutations across numerous cardiac genes is achievable with complementary long padlock probe technology. Moreover, this format allows facile insertion of additional probes as more cardiomyopathy and congenital heart disease genes are discovered, giving researchers a powerful new tool for DNA mutation detection and discovery.

    View details for DOI 10.1161/CIRCRESAHA.115.306723

    View details for PubMedID 26265630

  • Predicting Outcomes Using the Heart Failure Survival Score in Adults with Moderate or Complex Congenital Heart Disease. Congenital heart disease Lin, E. Y., Cohen, H. W., Bhatt, A. B., Stefanescu, A., Dudzinski, D., DeFaria Yeh, D., Johnson, J., Lui, G. K. 2015; 10 (5): 387-395


    Adults with congenital heart disease (CHD) face increased risk for morbidity and mortality with age, but few prognostic models exist.This study aims to assess whether the Heart Failure Survival Score (HFSS), which risk stratifies patients for heart transplantation, predicts outcomes in adults with moderate or complex CHD.This was a multicenter, retrospective study which identified 441 patients with moderate or complex CHD between 2005 and 2013, of whom 169 had all the HFSS parameters required to calculate the risk score. Because all study patients were deemed low risk by the HFSS, the score was dichotomized at the median (10.4). Outcomes included death, transplant or ventricular assist device (VAD), arrhythmia requiring treatment, nonelective cardiovascular (CV) hospitalizations, and the composite. Associations of mean HFSS and HFSS <10.4 with each outcome were assessed.The cohort had mean ± standard deviation age of 33.6 ± 12.6 years, peak VO2 21.8 ± 7.5 mL/kg/min, HFSS of 10.45 ± 0.88, and median years follow-up of 2.7 (1.1, 5.2). There were five deaths (2.8%), no transplants or VADs, 25 arrhythmias (14.8%), 22 CV hospitalizations (13%), and 39 composites (23.1%). Lower mean HFSS was observed for patients who died (9.6 ± 0.83 vs. 10.5 ± 0.87, P = .02), arrhythmia requiring treatment (10.0 ± 0.70 vs. 10.5 ± 0.89, P = .005), CV hospitalizations (9.9 ± 0.73 vs. 10.5 ± 0.88, P = .002), and the composite (10.0 ± 0.70 vs. 10.6 ± 0.89, P < .001). The positive and negative predictive values of HFSS <10.4 for the composite were 34% and 88% respectively, with sensitivity and specificity 74% and 56%.Although a low HFSS was significantly associated with outcomes, it did not adequately risk stratify adults with CHD, whose heterogeneous pathophysiology differs from that of the acquired heart failure population. Further studies are warranted to provide a more accurate prognosis.

    View details for DOI 10.1111/chd.12229

    View details for PubMedID 25358483

    View details for PubMedCentralID PMC4417083

  • Heart Murmur and Physical Examination in Athletes with a Focus on Congenital Heart Disease CURRENT SPORTS MEDICINE REPORTS Lui, G. K., Al Sindi, F., Bhatt, A. B. 2015; 14 (3): 263-265

    View details for Web of Science ID 000354551400031

    View details for PubMedID 25968870

  • Left Ventricular Retraining and Late Arterial Switch for D-Transposition of the Great Arteries ANNALS OF THORACIC SURGERY Watanabe, N., Mainwaring, R. D., Carrillo, S. A., Lui, G. K., Reddy, V. M., Hanley, F. L. 2015; 99 (5): 1655-1663


    For many decades, patients with d-transposition of the great arteries underwent an atrial switch procedure. Although many of these patients have continued to do well, a subset experience profound right ventricular failure. Some may be candidates for left ventricular (LV) retraining and late arterial switch. The purpose of this study was to review our experience with LV retraining and late arterial switch.This was a retrospective review of 32 patients with d-transposition. Thirty patients underwent a previous atrial switch and subsequently experienced right ventricular failure, whereas 2 presented late (8 months and 6 years) without previous intervention. The median age at the time of enrollment in this program was 15 years. Seven patients proceeded directly to late arterial switch owing to systemic LV pressures. The remaining 25 underwent a pulmonary artery band for LV retraining.Twenty of the 32 (63%) patients enrolled in this program were able to undergo a late arterial switch. There were 2 operative mortalities (10%). Two additional patients survived surgery but died in the early outpatient time period. There has been no late mortality after the arterial switch with a median follow-up of 5 years. Twelve patients underwent one or more pulmonary artery band procedures without evidence of effective LV retraining. There have been 2 early and 3 late (42%) deaths in this subgroup.The outcomes after arterial switch are encouraging and suggest that LV retraining and late arterial switch provide a viable option for this complex group of patients.

    View details for DOI 10.1016/j.athoracsur.2014.12.084

    View details for Web of Science ID 000353877900040

    View details for PubMedID 25817887

  • Management of Cardiovascular Risk Factors in Adults With Congenital Heart Disease JOURNAL OF THE AMERICAN HEART ASSOCIATION Lui, G. K., Fernandes, S., McElhinney, D. B. 2014; 3 (6)
  • Usefulness of the Seattle Heart Failure Model to Identify Adults With Congenital Heart Disease at High Risk of Poor Outcome AMERICAN JOURNAL OF CARDIOLOGY Stefanescu, A., Macklin, E. A., Lin, E., Dudzinski, D. M., Johnson, J., Kennedy, K. F., Jacoby, D., Yeh, D. D., Lewis, G. D., Yeh, R. W., Liberthson, R., Lui, G., Bhatt, A. B. 2014; 113 (5): 865-870


    Our objective was to determine whether the Seattle Heart Failure Model (SHFM) differentiates patients with adult congenital heart disease (ACHD) at high versus low risk for cardiovascular outcomes and poor exercise capacity. The ACHD population is growing and presents increasingly for care in the community and at tertiary centers. Few strategies exist to identify the patients with ACHD at high risk for heart failure and mortality.We studied 153 adults with transposition of the great arteries, Ebstein anomaly, tetralogy of Fallot, double outlet right ventricle, and single ventricle from 2 ACHD centers. The primary outcome was cardiovascular death, with a secondary composite outcome of death, transplant, ventricular assist device, cardiovascular admission, and treatment for arrhythmia. We defined risk groups based on SHFM 5-year predicted survival: high (predicted survival <70%), intermediate (70% to 85%), and low risk (>85%). Ten patients had the primary outcome of death, and 46 the combined end point. The hazard of death in the SHFM high- versus the intermediate-risk group was 7.09 (95% confidence interval 1.5 to 33.4, p = 0.01; no deaths in the low-risk group) and the hazard of the composite outcome between the high- versus low-risk group was 6.64 (95% confidence interval 2.5 to 17.6, p = 0.0001). Kaplan-Meier survival analysis showed greater probability of all-cause mortality (p = 0.003) in the high-risk group. In conclusion, the SHFM can help identify subjects with ACHD at risk for adverse outcome and poor cardiopulmonary efficiency. This may add to the care of patients with ACHD in the community and streamline care at tertiary centers.

    View details for DOI 10.1016/j.amjcard.2013.11.043

    View details for Web of Science ID 000332142200019

  • Anti-thrombotic strategies in the third trimester of pregnancy: A case of spontaneous coronary dissection requiring emergent PCI INTERNATIONAL JOURNAL OF CARDIOLOGY Paruchuri, V., Hafiz, A. M., Gubernikoff, G., De Leon, J., Lui, G., Naidu, S. S., Di Biase, L. 2013; 167 (1): E22-E25

    View details for DOI 10.1016/j.ijcard.2013.03.017

    View details for Web of Science ID 000320603100010

    View details for PubMedID 23582436

  • Caring for the Adult with Congenital Heart Disease in an Adult Catheterization Laboratory by Pediatric InterventionalistsSafety and Efficacy CONGENITAL HEART DISEASE Sutton, N. J., Greenberg, M. A., Menegus, M. A., Lui, G., Pass, R. H. 2013; 8 (2): 111-116


    The purpose of this study is to describe the outcomes of cardiac catheterizations performed by pediatric interventional cardiologists in an adult catheterization laboratory on adult patients with congenital heart disease (CHD).With improved survival rates, the number of adults with CHD increases by ∼5%/year; this population often requires cardiac catheterization.From January 2005 to December 2009, two groups of patients were identified, an adult group (>21 years) and an adolescent group (13-21 years), who had catheterizations performed by pediatric interventional staff.Fifty-seven catheterizations were performed in 53 adults, while 59 were performed in 47 adolescents. The male to female ratio differed significantly between groups; only 15/53 (28%) of adults were male vs. 26/47 (55%) of adolescents (P =.006). Among adults, 27 had previously corrected CHD, 16 with atrial septal defect (ASD), and six with patent foramen ovale (PFO). This differed significantly from the adolescents, where only 30 had previously corrected CHD, seven with ASD, and one with PFO (P =.012). Among adults who were catheterized, interventions were performed on 28/53 (53%). All interventions were successful and included ASD/PFO closure, patent ductus arteriosus occlusion, coarctation dilation, pulmonary artery dilations, and one saphenous vein graft aneurysm closure. Nineteen adults had coronary angiography performed by adult interventionalists in consult with pediatric interventionalists. Two complications occurred among adults (3.8%) vs. one complication (2%; P = 1) among adolescents. No femoral vessel complications or catheterization-associated mortality occurred.Cardiac catheterizations can be performed effectively and safely in adults with CHD by pediatric interventional cardiologists in an adult catheterization laboratory.

    View details for DOI 10.1111/chd.12004

    View details for Web of Science ID 000317140100013

    View details for PubMedID 23006871

  • Left and Right Ventricular Diastolic Function in Adults With Surgically Repaired Tetralogy of Fallot: A Multi-Institutional Study. The Canadian journal of cardiology Aboulhosn, J. A., Lluri, G., Gurvitz, M. Z., Khairy, P., Mongeon, F. P., Kay, J., Valente, A. M., Earing, M. G., Opotowsky, A. R., Lui, G., Gersony, D. R., Cook, S., Child, J., Ting, J., Webb, G., Landzberg, M., Broberg, C. S. 2013


    BACKGROUND: Our purpose was to assess the prevalence and clinical implications of left ventricular (LV) and right ventricular (RV) diastolic dysfunction (DD) in patients with repaired tetralogy of Fallot (TOF). METHODS: Adults with repaired TOF at 11 North American congenital heart disease centres were included. Of 556 patients analyzed, 325 had sufficient Doppler data to evaluate for LV DD, defined as mitral lateral e' < 10 cm/s and E/e' ratio > 9. Abnormal RV diastolic function was defined as tricuspid E/A ratio of 0.8 to 2.1 with E/e' ratio > 6 or a tricuspid ratio E/A > 2.1 with a deceleration time < 120 milliseconds, and 105 patients were found to have sufficient Doppler data. RESULTS: Abnormal LV diastolic Doppler indices were prevalent in 13.8% and associated with hypertension, dyslipidemia, 3 or more cardiac operations, and a history of ventricular tachycardia. Abnormal RV indices were prevalent in 52.4% and associated with hypertension, dyslipidemia, diabetes mellitus, and 3 or more cardiac operations. Ventricular tachycardia was more common in those with abnormal RV diastolic function. CONCLUSION: Further prospective studies are needed to evaluate these associations with DD and the impact of treatment risk factors on diastolic function and arrhythmia burden.

    View details for DOI 10.1016/j.cjca.2012.11.003

    View details for PubMedID 23369488

  • Aortic Root Dilatation in Adults with Surgically Repaired Tetralogy of Fallot A Multicenter Cross-Sectional Study CIRCULATION Mongeon, F., Gurvitz, M. Z., Broberg, C. S., Aboulhosn, J., Opotowsky, A. R., Kay, J. D., Valente, A. M., Earing, M. G., Lui, G. K., Fernandes, S. M., Gersony, D. R., Cook, S. C., Ting, J. G., Nickolaus, M. J., Landzberg, M. J., Khairy, P. 2013; 127 (2): 172-?


    Although aortic root pathology has been described in patients with tetralogy of Fallot, the scope of the problem remains poorly defined. We sought to determine the prevalence and predictors of aortic root dilatation in adults with repaired tetralogy of Fallot.A multicenter cross-sectional study was conducted with standardized reassessment of echocardiographic parameters in 474 adults (≥18 years) with surgically repaired tetralogy of Fallot or pulmonary atresia with ventricular septal defect. The aortic root was measured in a parasternal long-axis view, in diastole, at the level of the sinus of Valsalva. Prevalence and predictors of an absolute diameter ≥40 mm and of an observed-to-expected ratio >1.5 were assessed. The aortic root dimension was ≥40 mm in 28.9% (95% confidence interval, 26.9%-30.9%). In multivariate analyses, the only independently associated factor was male sex (odds ratio, 4.48; 95% confidence interval, 1.55-12.89; P=0.006). The prevalence of an observed-to-expected aortic root dimension ratio >1.5 was 6.6% (95% confidence interval, 5.3%-7.9%). It was associated with pulmonary atresia and moderate or severe aortic regurgitation in univariate analyses, but no independent predictor was identified. The side of the aortic arch was not associated with aortic root dimension. The prevalence of moderate or severe aortic regurgitation was 3.5% (95% confidence interval, 2.7%-4.2%).Although nearly one third of adults with repaired tetralogy of Fallot have an aortic root diameter ≥40 mm, the prevalence of a dilated aortic root, when defined by an indexed ratio of observed-to-expected values, is low. Similarly, moderate or severe aortic regurgitation is uncommon.

    View details for DOI 10.1161/CIRCULATIONAHA.112.129585

    View details for Web of Science ID 000313637200013

    View details for PubMedID 23224208

  • Not All Obstructive Cardiac Lesions Are Created Equal: Double-Chamber Right Ventricle In Pregnancy ECHOCARDIOGRAPHY-A JOURNAL OF CARDIOVASCULAR ULTRASOUND AND ALLIED TECHNIQUES Murthy, S., Lui, G., Raiszadeh, F., Boxt, L., Taub, C. 2012; 29 (8): E197-E200


    Double-chambered right ventricle (DCRV) is a rare form of right ventricular outflow tract (RVOT) obstruction accounting for approximately 1% of patients with congenital heart disease. It consists of an anomalous muscle bundle that divides the right ventricle usually between the sinus (inlet) and the infundibulum (outlet). This division creates a proximal chamber with high pressure and a distal chamber with low pressure. The hemodynamic obstruction of the RVOT is usually an acquired phenomenon, however the substrate for the anomalous muscle bundle is likely congenital. The diagnosis of DCRV should be considered in the young patient with an elevated right ventricular systolic pressure and intracavitary gradient. Echocardiography and cardiac MRI are the principal diagnostic tools for the assessment of DCRV. This entity is often misdiagnosed as pulmonary hypertension in the young patient, and can often go overlooked and untreated for many years. Definitive therapy involves surgical resection of the muscle bundle. This can often be curative and if done in a timely fashion, may prevent right ventricular remodeling. We describe the unique diagnostic dilemma, the course and management of a young adult with DCRV during pregnancy.

    View details for DOI 10.1111/j.1540-8175.2012.01721.x

    View details for Web of Science ID 000308466700006

    View details for PubMedID 22639818

  • Making Complex Adult Congenital Heart Disease a Little Simpler SEMINARS IN ROENTGENOLOGY Zwany, S. K., Lui, G. K., Scheinfeld, M. H., Levsky, J. M. 2012; 47 (3): 289-301

    View details for DOI 10.1053/

    View details for Web of Science ID 000305048900011

    View details for PubMedID 22657118

  • Prevalence of Left Ventricular Systolic Dysfunction in Adults With Repaired Tetralogy of Fallot AMERICAN JOURNAL OF CARDIOLOGY Broberg, C. S., Aboulhosn, J., Mongeon, F., Kay, J., Valente, A. M., Khairy, P., Earing, M. G., Opotowsky, A. R., Lui, G., Gersony, D. R., Cook, S., Ting, J. G., Webb, G., Gurvitz, M. Z. 2011; 107 (8): 1215-1220


    Left ventricular (LV) systolic dysfunction has been observed in patients with repaired tetralogy of Fallot (TOF), although its clinical associations are unknown. Adults with repaired TOF were identified from 11 adult congenital heart disease centers. Clinical history was reviewed. Patients with pulmonary atresia were excluded. Echocardiograms were reanalyzed to estimate LV ejection fraction. LV function was defined as normal (LV ejection fraction ≥ 55%) or mildly (45% to 54%), moderately (35% to 44%), or severely (< 35%) decreased. Right ventricular (RV) and LV dimensions and Doppler parameters were remeasured. Function of all valves was qualitatively scored. Of 511 patients studied, LV systolic dysfunction was present in 107 (20.9%, 95% confidence interval 17.4 to 24.5). Specifically, 74 (14.4%) had mildly decreased and 33 (6.3%) had moderately to severely decreased systolic function. Presence of moderate to severe LV dysfunction was associated with male gender, LV enlargement, duration of shunt before repair, history of arrhythmia, QRS duration, implanted cardioverter-defibrillator, and moderate to severe RV dysfunction. Severity or duration of pulmonary regurgitation was not different. In conclusion, LV systolic dysfunction was found in 21% of adult patients with TOF and was associated with shunt duration, RV dysfunction, and arrhythmia.

    View details for DOI 10.1016/j.amjcard.2010.12.026

    View details for PubMedID 21349477

  • Heart Rate Response During Exercise and Pregnancy Outcome in Women With Congenital Heart Disease CIRCULATION Lui, G. K., Silversides, C. K., Khairy, P., Fernandes, S. M., Valente, A. M., Nickolaus, M. J., Earing, M. G., AboulHosn, J. A., Rosenbaum, M. S., Cook, S., Kay, J. D., Jin, Z., Gersony, D. R. 2011; 123 (3): 242-248


    Cardiopulmonary exercise testing is often used to evaluate exercise capacity in adults with congenital heart disease including women who are considering pregnancy. The relationship between cardiopulmonary exercise testing parameters and pregnancy outcome has not been defined.We conducted a multicenter retrospective observational study of women with congenital heart disease who had undergone cardiopulmonary exercise testing within 2 years of pregnancy or during the first trimester. Cardiopulmonary exercise testing variables included peak oxygen consumption and measures of chronotropic response: peak heart rate, percentage of maximum age predicted heart rate, heart rate reserve (peak heart rate-resting heart rate), and chronotropic index [(peak heart rate-resting heart rate)/(220-age-resting heart rate)]. We identified 89 pregnancies in 83 women. There were 4 spontaneous abortions and 1 termination. One or more adverse cardiac events occurred in 18%; congestive heart failure in 14%, and sustained arrhythmia in 7%. Peak heart rate (odds ratio [OR] 0.71; 95% confidence interval [CI] [0.53, 0.94]; P=0.02), percentage of maximum age predicted heart rate (OR 0.93; 95% CI [0.88, 0.98]; P=0.01), and chronotropic index (OR 0.65; 95% CI [0.47, 0.90]; P=0.01) were associated with a cardiac event. Neonatal events occurred in 20%. Peak heart rate (OR 0.75; 95% CI [0.58, 0.98]; P=0.04), percentage of maximum age predicted heart rate (OR 0.94; 95% CI [0.89, 0.99]; P=0.02), heart rate reserve (OR 0.8; 95% CI [0.64, 0.99]; P=0.04), and chronotropic index (OR 0.73; 95% CI [0.54, 0.98]; P=0.04) correlated with a neonatal event. Peak oxygen consumption was not associated with an adverse pregnancy outcome.Abnormal chronotropic response correlates with adverse pregnancy outcomes in women with congenital heart disease and should be considered in refining risk stratification schemes.

    View details for DOI 10.1161/CIRCULATIONAHA.110.953380

    View details for Web of Science ID 000286507800010

    View details for PubMedID 21220738

  • Arrhythmia Burden in Adults With Surgically Repaired Tetralogy of Fallot A Multi-Institutional Study CIRCULATION Khairy, P., Aboulhosn, J., Gurvitz, M. Z., Opotowsky, A. R., Mongeon, F., Kay, J., Valente, A. M., Earing, M. G., Lui, G., Gersony, D. R., Cook, S., Ting, J. G., Nickolaus, M. J., Webb, G., Landzberg, M. J., Broberg, C. S. 2010; 122 (9): 868-875


    The arrhythmia burden in tetralogy of Fallot, types of arrhythmias encountered, and risk profile may change as the population ages.The Alliance for Adult Research in Congenital Cardiology (AARCC) conducted a multicenter cross-sectional study to quantify the arrhythmia burden in tetralogy of Fallot, to characterize age-related trends, and to identify associated factors. A total of 556 patients, 54.0% female, 36.8+/-12.0 years of age were recruited from 11 centers. Overall, 43.3% had a sustained arrhythmia or arrhythmia intervention. Prevalence of atrial tachyarrhythmias was 20.1%. Factors associated with intraatrial reentrant tachycardia in multivariable analyses were right atrial enlargement (odds ratio [OR], 6.2; 95% confidence interval [CI], 2.8 to 13.6), hypertension (OR, 2.3; 95% CI, 1.1 to 4.6), and number of cardiac surgeries (OR, 1.4; 95% CI, 1.2 to 1.6). Older age (OR, 1.09 per year; 95% CI, 1.05 to 1.12), lower left ventricular ejection fraction (OR, 0.93 per unit; 95% CI, 0.89 to 0.96), left atrial dilation (OR, 3.2; 95% CI, 1.5 to 6.8), and number of cardiac surgeries (OR, 1.5; 95% CI, 1.2 to 1.9) were jointly associated with atrial fibrillation. Ventricular arrhythmias were prevalent in 14.6% and jointly associated with number of cardiac surgeries (OR, 1.3; 95% CI, 1.1 to 1.6), QRS duration (OR, 1.02 per 1 ms; 95% CI, 1.01 to 1.03), and left ventricular diastolic dysfunction (OR, 3.3; 95% CI, 1.5 to 7.1). Prevalence of atrial fibrillation and ventricular arrhythmias markedly increased after 45 years of age.The arrhythmia burden in adults with tetralogy of Fallot is considerable, with various subtypes characterized by different profiles. Atrial fibrillation and ventricular arrhythmias appear to be influenced more by left- than right-sided heart disease.

    View details for DOI 10.1161/CIRCULATIONAHA.109.928481

    View details for Web of Science ID 000281502900004

    View details for PubMedID 20713900

  • The CALF (Congenital Heart Disease in Adults Lower Extremity Systemic Venous Health in Fontan Patients) Study JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Valente, A. M., Bhatt, A. B., Cook, S., Earing, M. G., Gersony, D. R., Aboulhosn, J., Opotowsky, A. R., Lui, G., Gurvitz, M., Graham, D., Fernandes, S. M., Khairy, P., Webb, G., Gerhard-Herman, M., Landzberg, M. J. 2010; 56 (2): 144-150


    The objective of this study was to document the prevalence of chronic venous insufficiency (CVI) and its associated factors in adults with Fontan physiology.As the population of adults with complex congenital heart disease and Fontan physiology increases, so does the occurrence of highly morbid and mortal outcomes, including heart failure and thromboembolism. The presence of abnormal peripheral hemodynamic conditions in this population and their potential contribution to adverse outcomes is not well known. The primary objective of this study was to document the prevalence of CVI in adults with Fontan physiology.A total of 159 adults with Fontan physiology from 7 adult congenital heart centers were prospectively assessed for lower extremity CVI, with the assignment of clinical, etiological, anatomical, and pathophysiological classification grades, and compared with age-matched and sex-matched controls. Leg photographs were independently reassessed to confirm interobserver reliability.The prevalence of CVI was significantly greater in the Fontan population (60%; 95% confidence interval [CI]: 52% to 68%) compared with healthy controls (32%; 95% CI: 15% to 54%) (p = 0.008). Strikingly, the prevalence of severe CVI (clinical, etiological, anatomical, and pathophysiological grade > or = 4) was significantly higher in the Fontan group (22%; 95% CI: 16% to 29%) versus the healthy controls (0%; 95% CI: 0% to 14%) (p = 0.005). In a multivariate analysis, several factors were independently associated with severe CVI, including increased numbers of catheterizations with groin venous access, lower extremity itching, and deep venous thrombosis.CVI is common in adult patients with congenital heart disease with Fontan physiology. The contribution of abnormal peripheral hemodynamic conditions to comorbidities, including thromboembolism and heart failure, and interventions to improve peripheral hemodynamic conditions require further exploration.

    View details for DOI 10.1016/j.jacc.2010.02.048

    View details for Web of Science ID 000279313600009

    View details for PubMedID 20620728