Clinical Focus


  • Adult Congenital Heart Disease

Academic Appointments


Professional Education


  • Fellowship: University of Chicago Medical Center Internal Medicine Residency (2010) IL
  • Residency: University of Chicago Medical Center Internal Medicine Residency (2006) IL
  • Board Certification: American Board of Internal Medicine, Adult Congenital Heart Disease (2017)
  • Medical Education: Rosalind Franklin University The Chicago Medical School (2002) IL
  • Board Certification: American Board of Internal Medicine, Cardiovascular Disease (2011)
  • Fellowship: Boston Children's Hospital (2013) MA
  • Fellowship: Boston Children's Hospital (2012) MA

All Publications


  • Heart Failure and Patient-Reported Outcomes in Adults With Congenital Heart Disease from 15 Countries. Journal of the American Heart Association Lu, C., Wang, J., Yang, H., Kovacs, A. H., Luyckx, K., Ruperti-Repilado, F. J., Van De Bruaene, A., Enomoto, J., Sluman, M. A., Jackson, J. L., Khairy, P., Cook, S. C., Chidambarathanu, S., Alday, L., Oechslin, E., Eriksen, K., Dellborg, M., Berghammer, M., Johansson, B., Mackie, A. S., Menahem, S., Caruana, M., Veldtman, G., Soufi, A., Fernandes, S. M., White, K., Callus, E., Kutty, S., Apers, S., Moons, P., APPROACH‐IS consortium, t. I., Alday, L., Maisuls, H., Vega, B., Menahem, S., Eaton, S., Wang, Q. F., Larion, R., Budts, W., Van Deyk, K., Apers, S., Goossens, E., Rassart, J., Luyckx, K., Moons, P., Rempel, G., Mackie, A., Ballantyne, R., Rankin, K., Norris, C., Taylor, D., Vondermuhll, I., Windram, J., Heggie, P., Lasiuk, G., Khairy, P., Proietti, A., Dore, A., Mercier, L., Mongeon, F., Marcotte, F., Ibrahim, R., Mondesert, B., Cote, M., Kovacs, A., Oechslin, E., Bandyopadhyay, M., Soufi, A., Di Filippo, S., Sassolas, F., Bozio, A., Chareyras, C., Chidambarathanu, S., Farzana, F., Lakshmi, N., Callus, E., Quadri, E., Chessa, M., Campioni, G., Giamberti, A., Enomoto, J., Mizuno, Y., Caruana, M., Grech, V., Vella, S., Mifsud, A., Borg, N., Chircop, D., Balbi, M. M., Critien, R. V., Farrugia, J., Gatt, Y., Muscat, D., Eriksen, K., Estensen, M., Dellborg, M., Berghammer, M., Mattsson, E., Strandberg, A., Karlstrom-Hallberg, P., Johansson, B., Kronhamn, A., Schwerzman, M., Thomet, C., Huber, M., Wang, J., Lu, C., Yang, H., Hua, Y. C., Mulder, B., Sluman, M., Post, M., Pieper, E., Peels, K., Waskowsky, M., Veldtman, G., Faust, M., Lozier, C., Reed, C., Hilfer, J., Daniels, C., Jackson, J., Kutty, S., Chamberlain, C., Warta, S., Cook, S., Hindes, M., Cedars, A., White, K., Fernandes, S., Romfh, A., MacMillen, K. 2022: e024993

    Abstract

    Background Heart failure (HF) is the leading cause of mortality and associated with significant morbidity in adults with congenital heart disease. We sought to assess the association between HF and patient-report outcomes in adults with congenital heart disease. Methods and Results As part of the APPROACH-IS (Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study), we collected data on HF status and patient-reported outcomes in 3959 patients from 15 countries across 5 continents. Patient-report outcomes were: perceived health status (12-item Short Form Health Survey), quality of life (Linear Analogue Scale and Satisfaction with Life Scale), sense of coherence-13, psychological distress (Hospital Anxiety and Depression Scale), and illness perception (Brief Illness Perception Questionnaire). In this sample, 137 (3.5%) had HF at the time of investigation, 298 (7.5%) had a history of HF, and 3524 (89.0%) had no current or past episode of HF. Patients with current or past HF were older and had a higher prevalence of complex congenital heart disease, arrhythmias, implantable cardioverter-defibrillators, other clinical comorbidities, and mood disorders than those who never had HF. Patients with HF had worse physical functioning, mental functioning, quality of life, satisfaction with life, sense of coherence, depressive symptoms, and illness perception scores. Magnitudes of differences were large for physical functioning and illness perception and moderate for mental functioning, quality of life, and depressive symptoms. Conclusions HF in adults with congenital heart disease is associated with poorer patient-reported outcomes, with large effect sizes for physical functioning and illness perception. Registration URL: https://clinicaltrials.gov; Unique identifier: NCT02150603.

    View details for DOI 10.1161/JAHA.121.024993

    View details for PubMedID 35470715

  • 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

    Abstract

    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

  • Waitlist and post-transplant outcomes for eisenmenger syndrome: A comparison of transplant strategies. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation Kearney, K., Lau, E. M., Darley, D., Romfh, A., Bart, N., Kotlyar, E., Hayes, D. J., Khush, K., Keogh, A. 2021

    Abstract

    BACKGROUND: End-stage Eisenmenger syndrome (ES) due to unrepaired atrial septal defect (ASD) or ventricular septal defect (VSD) is an indication for lung transplantation (LTx) or heart-lung transplantation (HLTx). Limited evidence exists as to the optimal transplant strategy for this unique population.AIM: To describe waitlist characteristics and post-transplant outcomes in patients with ES-ASD or ES-VSD.METHODS: Using the ISHLT Registry, data were extracted for all ES-ASD or ES-VSD patients who underwent transplantation between 1987 and 2018. Additional data were sought for patients listed for LTx or HLTx in the OPTN Registry during the same period. Early era was defined as 1987-2004, and current era was defined as 2005-2018.RESULTS: In the current era, patients with ES-ASD or ES-VSD represented a lessening proportion of all LTx and HLTx. Compared to LTx for other indications, the odds of transplantation were significantly less for both ES-ASD 0.18 [0.07-0.50] and ES-VSD 0.03 [0.004-0.22]. In the early era, an equivalent survival was observed for ES-ASD who underwent HLTx versus LTx (p=0.47), and superior survival for ES-VSD (p=0.015). In contrast, ES-ASD patients who underwent LTx from the current era displayed better survival compared with HLTx, 10-year survival 52% vs 30% p=0.036. Similar survival were observed for ES-VSD for both transplant strategies (p=0.68).CONCLUSION: LTx shows superior survival outcomes in the current era for ES ASD patients, and equivalent outcomes for ES-VSD. In the current era, ES-ASD or ES-VSD patients were less likely to be transplanted than other candidates for LTx.

    View details for DOI 10.1016/j.healun.2021.04.005

    View details for PubMedID 34112578

  • 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

    Abstract

    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

  • 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

    Abstract

    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

  • 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

  • General principles of heart failure management in adult congenital heart disease. Heart failure reviews Tompkins, R., Romfh, A. 2019

    Abstract

    The population of adults with congenital heart disease is now an estimated 1.4 million in the United States alone and growing. Unfortunately, survival to adulthood does not equate to a normal life expectancy, and heart failure (HF) has now emerged as the leading cause of death for the adult congenital heart disease (ACHD) patient. As this population continues to grow in number and advance in age, the prevalence of heart failure in the adult with congenital heart disease (ACHD-HF) will undoubtedly continue to increase. However, much of our current understanding of mechanism, diagnosis, and management of ACHD-HF remains limited. We aim to review the current understanding of the proposed definitions, mechanisms, clinical impact, and general management considerations of ACHD-HF while also recognizing the large number of knowledge gaps that persist.

    View details for DOI 10.1007/s10741-019-09895-x

    View details for PubMedID 31788738

  • Patient-reported outcomes of adults with congenital heart disease from eight European countries: scrutinising the association with healthcare system performance EUROPEAN JOURNAL OF CARDIOVASCULAR NURSING Van Bulck, L., Luyckx, K., Goossens, E., Apers, S., Kovacs, A. H., Thomet, C., Budts, W., Sluman, M. A., Eriksen, K., Dellborg, M., Berghammer, M., Johansson, B., Caruana, M., Soufi, A., Callus, E., Moons, P., Alday, L., Maisuls, H., Vega, B., Menahem, S., Eaton, S., Wang, Q., Larion, R., Van Deyk, K., Apers, S., Rassart, J., Rempel, G., Mackie, A., Ballantyne, R., Rankin, K., Norris, C., Taylor, D., Vondermuhll, I., Windram, J., Heggie, P., Lasiuk, G., Khairy, P., Proietti, A., Dore, A., Mercier, L., Mongeon, F., Marcotte, F., Ibrahim, R., Mondesert, B., Cote, M., Kovacs, A., Oechslin, E., Bandyopadhyay, M., Di Filippo, S., Sassolas, F., Bozio, A., Chareyras, C., Chidambarathanu, S., Farzana, F., Lakshmi, N., Quadri, E., Chessa, M., Campioni, G., Giamberti, A., Enomoto, J., Mizuno, Y., Grech, V., Vella, S., Mifsud, A., Borg, N., Chircop, D., Balbi, M., Critien, R., Farrugia, J., Gatt, Y., Muscat, D., Estensen, M., Mattsson, E., Strandberg, A., Karlstrom-Hallberg, P., Kronhamn, A., Schwerzman, M., Huber, M., Wang, J., Lu, C., Yang, H., Hua, Y., Mulder, B., Sluman, M., Post, M., Pieper, E., Peels, K., Waskowsky, M., Veldtman, G., Faust, M., Lozier, C., Reed, C., Hilfer, J., Daniels, C., Jackson, J., Kutty, S., Chamberlain, C., Warta, S., Cook, S., Hindes, M., Cedars, A., White, K., Fernandes, S., Romfh, A., MacMillen, K., APPROACH-IS Consortium, ISACHD 2019; 18 (6): 465–73

    Abstract

    Inter-country variation in patient-reported outcomes of adults with congenital heart disease has been observed. Country-specific characteristics may play a role. A previous study found an association between healthcare system performance and patient-reported outcomes. However, it remains unknown which specific components of the countries' healthcare system performance are of importance for patient-reported outcomes.The aim of this study was to investigate the relationship between components of healthcare system performance and patient-reported outcomes in a large sample of adults with congenital heart disease.A total of 1591 adults with congenital heart disease (median age 34 years; 51% men; 32% simple, 48% moderate and 20% complex defects) from eight European countries were included in this cross-sectional study. The following patient-reported outcomes were measured: perceived physical and mental health, psychological distress, health behaviours and quality of life. The Euro Health Consumer Index 2015 and the Euro Heart Index 2016 were used as measures of healthcare system performance. General linear mixed models were conducted, adjusting for patient-specific variables and unmeasured country differences.Health risk behaviours were associated with the Euro Health Consumer Index subdomains about patient rights and information, health outcomes and financing and access to pharmaceuticals. Perceived physical health was associated with the Euro Health Consumer Index subdomain about prevention of chronic diseases. Subscales of the Euro Heart Index were not associated with patient-reported outcomes.Several features of healthcare system performance are associated with perceived physical health and health risk behaviour in adults with congenital heart disease. Before recommendations for policy-makers and clinicians can be conducted, future research ought to investigate the impact of the healthcare system performance on outcomes further.

    View details for DOI 10.1177/1474515119834484

    View details for Web of Science ID 000477647400005

    View details for PubMedID 30808198

  • 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

  • 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
  • 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

    Abstract

    BACKGROUND: Although lower-complexity cardiac malformations constitute the majority of adult congenital heart disease (ACHD), the long-term risks of adverse cardiovascular events and relationship with conventional risk factors in this population are poorly understood. We aimed to quantify the risk of adverse cardiovascular events associated with lower-complexity ACHD that is unmeasured by conventional risk factors.METHODS: A multi-tiered classification algorithm was used to select individuals with lower-complexity ACHD and individuals without ACHD for comparison amongst >500,000 British adults in the UK Biobank (UKB). ACHD diagnoses were sub-classified as "isolated aortic valve (AoV)" and "non-complex" defects. Time-to-event analyses were conducted for primary endpoints of fatal or non-fatal acute coronary syndrome (ACS), ischemic stroke, heart failure (HF), and atrial fibrillation, and a secondary combined endpoint for major adverse cardiovascular event (MACE). Maximum follow-up time for the study period was 22 years using retrospectively and prospectively collected data from the UKB.RESULTS: We identified 2,006 individuals with lower-complexity ACHD and 497,983 unexposed individuals in the UKB (median [IQR] age at enrollment 58 [51,63]). Of the ACHD-exposed group, 59% were male; 51% were current or former smokers; 30% were obese; 69%, 41%, and 7% were diagnosed or treated for hypertension, hyperlipidemia, and diabetes respectively. After adjustment for 12 measured cardiovascular risk factors, ACHD remained strongly associated with the primary endpoints, with hazard ratios (HR) ranging from 2.0 (95% confidence interval [CI] 1.5-2.8, p<0.001) for ACS to 13.0 (95% CI 9.4-18.1, p<0.001) for HF. ACHD-exposed individuals with ≤2 cardiovascular risk factors had a 29% age-adjusted incidence rate of MACE in contrast to 13% in non-ACHD individuals with ≥5 risk factors.CONCLUSIONS: Individuals with lower-complexity ACHD had higher burden of adverse cardiovascular events relative to the general population that was unaccounted for by conventional cardiovascular risk factors. These findings highlight the need for closer surveillance of patients with mild to moderate ACHD and further investigation into management and mechanisms of cardiovascular risk unique to this growing population of high-risk adults.

    View details for PubMedID 30813762

  • 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

    Abstract

    BACKGROUND: Arrhythmias are a leading cause of death in adults with congenital heart disease (ACHD). While 24-48-hour monitors are often used to assess arrhythmia burden, extended continuous ambulatory rhythm monitors (ECAM) can record 2 weeks of data. The utility of this device and the arrhythmia burden identified beyond 48-hour monitoring have not been evaluated in the ACHD population. Additionally, the impact of ECAM has not been studied to determine management recommendations.OBJECTIVE: To address the preliminary question, we hypothesized that clinically significant arrhythmias would be detected on ECAM beyond 48hours and this would lead to clinical management changes.METHODS: A single center retrospective cohort study of ACHD patients undergoing ECAM from June 2013 to May 2016 was performed. The number and type of arrhythmias detected within and beyond the first 48hours of monitoring were compared using Kaplan-Meier curves and Cox proportional hazard models.RESULTS: Three hundred fourteen patients had monitors performed [median age 31 (IQR 25-41) years, 61% female). Significant arrhythmias were identified in 156 patients (50%), of which 46% were noted within 48 hours. A management change based on an arrhythmia was made in 49 patients (16%).CONCLUSIONS: ECAM detects more clinically significant arrhythmias than standard 48-hour monitoring in ACHD patients. Management changes, including medication changes, further testing or imaging, and procedures, were made based on results of ECAM. Recommendations and guidelines have been made based on arrhythmias on 48-hour monitoring; the predictive ability and clinical consequence of arrhythmias found on ECAM are not yet known.

    View details for PubMedID 30604934

  • Transitioning the Adult Congenital Heart Disease Patient from the Cardiovascular Intensive Care Unit to the Ward INTENSIVE CARE OF THE ADULT WITH CONGENITAL HEART DISEASE Sillman, C., Romfh, A., Tompkins, R., Fernandes, S. M., DaCruz, E., Macrae, D., Webb, G. 2019: 521-534
  • 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
  • CONGENITAL HEART DISEASE CONFERS SUBSTANTIAL RISK OF ACQUIRED CARDIOVASCULAR DISEASE AMONGST BRITISH ADULTS Saha, P., Potiny, P., Tcheandjieu, C., Fernandes, S. M., Romfh, A., Bernstein, D., Lui, G. K., Ingelsson, E., Priest, J. ELSEVIER SCIENCE INC. 2018: 553
  • 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

    Abstract

    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

  • Incidence, risk factors, and outcomes of acute kidney injury in adults undergoing surgery for congenital heart disease. Cardiology in the young Kwiatkowski, D. M., Price, E., Axelrod, D. M., Romfh, A. W., Han, B. S., Sutherland, S. M., Krawczeski, C. D. 2016: 1-8

    Abstract

    Acute kidney injury after cardiac surgery is a frequent and serious complication among children with congenital heart disease (CHD) and adults with acquired heart disease; however, the significance of kidney injury in adults after congenital heart surgery is unknown. The primary objective of this study was to determine the incidence of acute kidney injury after surgery for adult CHD. Secondary objectives included determination of risk factors and associations with clinical outcomes.This single-centre, retrospective cohort study was performed in a quaternary cardiovascular ICU in a paediatric hospital including all consecutive patients ⩾18 years between 2010 and 2013.Data from 118 patients with a median age of 29 years undergoing cardiac surgery were analysed. Using Kidney Disease: Improving Global Outcome creatinine criteria, 36% of patients developed kidney injury, with 5% being moderate to severe (stage 2/3). Among higher-complexity surgeries, incidence was 59%. Age ⩾35 years, preoperative left ventricular dysfunction, preoperative arrhythmia, longer bypass time, higher Risk Adjustment for Congenital Heart Surgery-1 category, and perioperative vancomycin use were significant risk factors for kidney injury development. In multivariable analysis, age ⩾35 years and vancomycin use were significant predictors. Those with kidney injury were more likely to have prolonged duration of mechanical ventilation and cardiovascular ICU stay in the univariable regression analysis.We demonstrated that acute kidney injury is a frequent complication in adults after surgery for CHD and is associated with poor outcomes. Risk factors for development were identified but largely not modifiable. Further investigation within this cohort is necessary to better understand the problem of kidney injury.

    View details for PubMedID 27869053

  • Quality of Life of Adults With Congenital Heart Disease in 15 Countries Evaluating Country-Specific Characteristics JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Apers, S., Kovacs, A. H., Luyckx, K., Thomet, C., Budts, W., Enomoto, J., Sluman, M. A., Wang, J., Jackson, J. L., Khairy, P., Cook, S. C., Chidambarathanu, S., Alday, L., Eriksen, K., Dellborg, M., Berghammer, M., Mattsson, E., Mackie, A. S., Menahem, S., Caruana, M., Veldtman, G., Soufi, A., Romfh, A. W., White, K., Callus, E., Kutty, S., Fieuws, S., Moons, P. 2016; 67 (19): 2237-2245

    Abstract

    Measuring quality of life (QOL) is fundamental to understanding the impact of disease and treatment on patients' lives.This study aimed to explore QOL in an international sample of adults with congenital heart disease (CHD), the association between patient characteristics and QOL, and international variation in QOL and its relationship to country-specific characteristics.We enrolled 4,028 adults with CHD from 15 countries. QOL was assessed using a linear analog scale (LAS) (0 to 100) and the Satisfaction with Life Scale (SWLS) (5 to 35). Patient characteristics included sex, age, marital status, educational level, employment status, CHD complexity, and patient-reported New York Heart Association (NYHA) functional class. Country-specific characteristics included general happiness and 6 cultural dimensions. Linear mixed models were applied.Median QOL was 80 on the LAS and 27 on the SWLS. Older age, lack of employment, no marriage history, and worse NYHA functional class were associated with lower QOL (p < 0.001). Patients from Australia had the highest QOL (LAS: 82) and patients from Japan the lowest (LAS: 72). Happiness scores and cultural dimensions were not associated with variation in QOL after adjustment for patient characteristics and explained only an additional 0.1% of the variance above and beyond patient characteristics (p = 0.56).This large-scale, international study found that overall QOL in adults with CHD was generally good. Variation in QOL was related to patient characteristics but not country-specific characteristics. Hence, patients at risk for poorer QOL can be identified using uniform criteria. General principles for designing interventions to improve QOL can be developed.

    View details for DOI 10.1016/j.jacc.2016.03.477

    View details for Web of Science ID 000375406600007

    View details for PubMedID 27173035