Deborah Y. Ho

All Publications

• Echocardiographic Predictors of Readiness for Double Switch Operation and Postoperative Ejection Fraction in Patients with Congenitally Corrected Transposition of the Great Arteries Undergoing Left Ventricular Retraining. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Duong, S. Q., Ho, D., Punn, R., Sganga, D., Mainwaring, R., Ma, M., Hanley, F. L., Lee, K. J., Maskatia, S. A. 2024

  Abstract

  In patients with congenitally corrected transposition of the great arteries (ccTGA), assessment of readiness for double switch operation (DSO) after pulmonary arterial band (PAB) placement involves cardiac MRI (cMRI) to measure LV ejection fraction (EF) and mass and cardiac catheterization (cath) to assess LV:RV pressure ratio (LV:RVp). We described the relationships between echocardiographic and cath/cMRI measures of readiness for DSO, and developed risk factors for LV dysfunction after DSO based on echocardiographic measures of ventricular arterial coupling (VAC).We reviewed patients with ccTGA undergoing LV retraining at a DSO referral center. We compared EF measured by echo to cMRI, and LV:RVp measured by echo to cath with Bland-Altman (BA) analysis. We analyzed the relationship between preoperative VAC markers and postoperative echocardiogram using end-systolic elastance (EES), and a novel marker consisting of the product of EF and the LV:RVp (EFPR).We included 31 patients with 56 evaluations for DSO, 24 of which underwent DSO. Echo EF correlated well with cMRI (r= 0.79), and BA slightly overestimated cMRI (mean difference +3%). Echo EF had moderate ability to identify normal cMRI EF (AUC of 0.80) and at optimal cutpoint of echo EF threshold of 61%, there was 71% sensitivity and 76% specificity to detect cMRI LVEF >=55%. Echo LV:RVp correlated well with cath (r=0.77) and slightly underestimated cath (mean difference of -0.11). Echo LV:RVp had good ability to identify adequate LV:RVp by cath (AUC=0.95) and at optimal echo cutpoint of 0.75 had 100% Sensitivity and 85% specificity to detect a catheterization LV:RVp above 0.9. Echo-based criteria for DSO readiness (echo EF of 61% and LV:RVp of 0.75) demonstrated specificity of 97% and positive predictive value of 96% for published criteria of DSO readiness (cMRI EF of 55% and cath LV:RVp of 0.9). EES and EFPR correlated with post DSO EF (rho= 0.72 and 0.60). EFPR of 0.51 demonstrated 78% sensitivity and 100% specificity for post DSO LV dysfunction (EF < 55%). Age at first PAB also strongly correlated with post DSO EF (rho=0.75). No patient with first PAB under age 1 years exhibited post DSO LV dysfunction.Echocardiographic measures of EF and LV:RVp are reliable indicators of reference standard modalities, and can guide management during retraining. Preoperative VAC markers EES and EFPR may be useful markers of post-DSO LV dysfunction. Echo LV:RVp > 0.75 are likely to meet pressure-generation criteria for DSO and should be considered for referral for cath and cMRI evaluation for DSO. PAB placement before 1 year of life may optimize LV outcomes in patients considered for DSO.

  View details for DOI 10.1016/j.echo.2024.08.011

  View details for PubMedID 39218369

• SURGICAL OUTCOMES IN PATIENTS UNDERGOING A DOUBLE SWITCH OPERATION FOR CORRECTED TRANSPOSITION. The Annals of thoracic surgery Mainwaring, R. D., Felmly, L. M., Ho, D. Y., Arunamata, A., Algaze, C., Ma, M., Hanley, F. L. 2024

  Abstract

  BACKGROUND: Congenitally corrected transposition of the great arteries (CC-TGA) is a rare and complex form of congenital heart disease. Results of physiologic repair proved disappointing due to late right ventricular dysfunction and/or tricuspid regurgitation. The current study was performed to evaluate surgical outcomes in patients undergoing a double switch for CC-TGA.METHODS: This was a retrospective review of 121 patients who underwent a double switch over a two-decade time frame (2002-2023). The median age of patients was 32 months. Forty-nine of 121 patients (40%) had undergone left ventricular retraining prior to double switch.RESULTS: Sixty-seven patients underwent an arterial switch whereas 54 underwent a Rastelli procedure. There were four in-hospital deaths (3.3%) including three who had a Rastelli procedure (5.6%) and one who had an arterial switch (1.5%). At a median follow-up of 30 months, there have been four late deaths (two Rastelli and two arterial switch). Combined early and late mortality was 9.3% for the Rastelli and 4.5% for arterial switch. Combined mortality was 2.0% for patients who required left ventricular retraining versus 9.7% for those who did not. For the 117 patients discharged from the hospital, 93% have normal or low normal left ventricular function and 96% have mild or less neo-aortic insufficiency.CONCLUSIONS: Surgical outcomes in patients undergoing a double switch procedure have been excellent both in the short- and mid-term. However, the Rastelli procedure was associated with a more than two-fold increase in mortality risk compared with the arterial switch.

  View details for DOI 10.1016/j.athoracsur.2024.04.022

  View details for PubMedID 38750683

• Results of the Double Switch Operation in Patients Who Previously Underwent Left Ventricular Retraining. World journal for pediatric & congenital heart surgery Mac Felmly, L., Mainwaring, R. D., Ho, D. Y., Arunamata, A., Algaze, C., Hanley, F. L. 2024: 21501351231224329

  Abstract

  BACKGROUND: Congenitally corrected transposition of the great arteries (CC-TGA) is a complex form of congenital heart disease that has numerous subtypes. While most patients with CC-TGA have a large ventricular septal defect (VSD) and pulmonary stenosis, there are some patients who have either no VSD or a highly restrictive VSD. These patients will require left ventricular (LV) retraining prior to double switch. The purpose of this study was to review our experience with the double switch procedure in patients who had previously undergone LV retraining.METHODS: This was a retrospective review of a single institution experience with the double switch procedure in patients who had undergone LV retraining (2002-present).RESULTS: Forty-five patients underwent double switch following LV retraining. Of these, 39 had an arterial switch with hemi-Mustard/bidirectional Glenn and six had a Senning. The median cross-clamp time was 135 min (range 71-272) and median bypass time was 202 min (range 140-430 min). Median hospital length of stay was eight days (range 4-108). There were no in-hospital deaths. Median duration of follow-up was 30 months (range 0-175). One patient subsequently underwent heart transplantation and died 65 months following double switch. At follow-up, 41 of the 44 survivors (93%) have normal or low normal LV function and 40 of the 44 survivors (91%) have no or trace mitral regurgitation.CONCLUSIONS: The data demonstrate early and mid-term survival of 100% and 97%. Ninety-three percent had preserved LV function. These results suggest that patients with CC-TGA who undergo LV retraining and double switch can have excellent clinical outcomes.

  View details for DOI 10.1177/21501351231224329

  View details for PubMedID 38321756

• Cardiac magnetic resonance predictors for successful primary biventricular repair of unbalanced complete common atrioventricular canal. Cardiology in the young Jones, A. L., White, B. R., Ghosh, R. M., Mondal, A., Ampah, S., Ho, D. Y., Whitehead, K., Harris, M. A., Biko, D. M., Partington, S., Fuller, S., Cohen, M. S., Fogel, M. A. 2023: 1-8

  Abstract

  BACKGROUND: Patients with unbalanced common atrioventricular canal can be difficult to manage. Surgical planning often depends on pre-operative echocardiographic measurements. We aimed to determine the added utility of cardiac MRI in predicting successful biventricular repair in common atrioventricular canal.METHODS: We conducted a retrospective cohort study of children with common atrioventricular canal who underwent MRI prior to repair. Associations between MRI and echocardiographic measures and surgical outcome were tested using logistic regression, and models were compared using area under the receiver operator characteristic curve.RESULTS: We included 28 patients (median age at MRI: 5.2 months). The optimal MRI model included the novel end-diastolic volume index (using the ratio of left ventricular end-diastolic volume to total end-diastolic volume) and the left ventricle-right ventricle angle in diastole (area under the curve 0.83, p = 0.041). End-diastolic volume index ≤ 0.18 and left ventricle-right ventricle angle in diastole ≤ 72° yield a sensitivity of 83% and specificity of 81% for successful biventricular repair. The optimal multimodality model included the end-diastolic volume index and the echocardiographic atrioventricular valve index with an area under the curve of 0.87 (p = 0.026).CONCLUSIONS: Cardiac MRI can successfully predict successful biventricular repair in patients with unbalanced common atrioventricular canal utilising the end-diastolic volume index alone or in combination with the MRI left ventricle-right ventricle angle in diastole or the echocardiographic atrioventricular valve index. A prospective cardiac MRI study is warranted to better define the multimodality characteristic predictive of successful biventricular surgery.

  View details for DOI 10.1017/S1047951123001786

  View details for PubMedID 37462049

• Congenitally Corrected Transposition of the Great Arteries: Fetal Diagnosis, Associations, and Postnatal Outcome: A Fetal Heart Society Research Collaborative Study. Journal of the American Heart Association Cohen, J., Arya, B., Caplan, R., Donofrio, M. T., Ferdman, D., Harrington, J. K., Ho, D. Y., Hogan, W., Hornberger, L. K., Jhaveri, S., Killen, S. A., Lindblade, C. L., Michelfelder, E., Moon-Grady, A. J., Patel, S., Quezada, E., Ronai, C., Sanchez Mejia, A. A., Schidlow, D. N., Stiver, C., Thakur, V., Srivastava, S. 2023: e029706

  Abstract

  Background Fetal diagnosis of congenitally corrected transposition of the great arteries (ccTGA) has been increasingly reported; however, predictors of clinical outcomes remain underexplored. We undertook a multicenter, retrospective study to investigate natural history, associated anomalies, and outcomes of fetal ccTGA. Methods and Results Fetuses with ccTGA diagnosed from January 2004 to July 2020 within 20 North American programs were included. Fetuses with severe ventricular hypoplasia thought to definitively preclude biventricular repair were excluded. We included 205 fetuses diagnosed with ccTGA at a median gestational age of 23 (interquartile range, 21-27) weeks. Genetic abnormalities were found in 5.9% tested, with extracardiac anomalies in 6.3%. Associated cardiac defects were diagnosed in 161 (78.5%), with atrioventricular block in 23 (11.3%). On serial fetal echocardiogram, 39% demonstrated a functional or anatomic change, most commonly increased tricuspid regurgitation (6.7%) or pulmonary outflow obstruction (11.1%). Of 194 fetuses with follow-up, 26 were terminated, 3 experienced fetal death (2 with atrioventricular block), and 165 were live-born. Of 158 with postnatal data (median follow-up 3.7years), 10 (6.6%) had death/transplant before 1year. On univariable analysis, fetal factors associated with fetal death or death/transplant by 1year included ≥ mild tricuspid regurgitation, pulmonary atresia, aortic obstruction, fetal arrhythmia, and worsening hemodynamics on serial fetal echocardiogram (defined as worse right ventricular function, tricuspid regurgitation, or effusion). Conclusions Associated cardiac lesions and arrhythmias are common in fetal ccTGA, and functional changes commonly occur through gestation. Worse outcomes are associated with fetal tricuspid regurgitation (≥mild), any arrhythmia, pulmonary atresia, aortic obstruction, and worsening hemodynamics on serial echocardiograms. These findings can inform prenatal counseling and perinatal management planning.

  View details for DOI 10.1161/JAHA.122.029706

  View details for PubMedID 37259984

• Mid-Gestational Fetal Placental Blood Flow is Diminished in the Fetus with Congenital Heart Disease. Prenatal diagnosis Ho, D. Y., Josowitz, R. n., Katcoff, H. n., Griffis, H. M., Tian, Z. n., Gaynor, J. W., Rychik, J. n. 2020

  Abstract

  Data suggest fetuses with congenital heart disease (CHD) have placental abnormalities. Their abnormal placental vasculature may affect fetal placental blood flow, which has not previously been explored.We performed a retrospective cross-sectional study comparing umbilical venous volume flow (UVVF) of single ventricle, D-transposition of the great arteries, and tetralogy of Fallot fetuses with fetuses without CHD. UVVF and combined cardiac output (CCO) were calculated from fetal echocardiography and compared using t-tests, Chi-square and Fisher's exact tests.Mean gestational age and fetal weight were greater in CHD fetuses (26.5 weeks, 1119.4g; n=81, p<0.001) compared to controls (23.1 weeks, 675g; n=170, p<0.001). UVVF/fetal weight was nevertheless decreased among cases (99.8 versus 115.3 mL/min/kg, p<0.001). Subgroup analysis of 20- to 25-week fetuses demonstrated no significant differences in case and control baseline characteristics. In CHD fetuses (n=31) compared to controls (n=144), absolute UVVF (50.8 versus 62.1 mL/min, p=0.006), and UVVF/fetal weight (98.8 versus 118.5 mL/min/kg, p<0.001) were decreased. Findings were similar in single ventricle (n=24) and hypoplastic left heart syndrome (n=14).Mid-gestational placental blood flow in CHD fetuses is decreased compared to controls. Further study is needed to explore the relationship between UVVF and placental pathology, and impact on outcomes. This article is protected by copyright. All rights reserved.

  View details for DOI 10.1002/pd.5791

  View details for PubMedID 32673414

• Left Valvar Morphology is Associated with Late Regurgitation in Atrioventricular Canal Defect. The Annals of thoracic surgery Ho, D. Y., Katcoff, H. n., Griffis, H. M., Mercer-Rosa, L. n., Fuller, S. M., Cohen, M. S. 2020

  Abstract

  Left atrioventricular valvar regurgitation (LAVVR) following atrioventricular canal (AVC) repair remains a significant cause of morbidity. Papillary muscle arrangement may be important. The implications of left mural leaflet morphology have not been investigated. We examined anatomic characteristics of the LAVV to determine possible associations with postoperative LAVVR.All patients with biventricular AVC repair at our institution between 1/1/11 and 12/31/16 with necessary imaging were retrospectively reviewed. Papillary muscle structure and novel measures of the left mural leaflet were assessed from preoperative echocardiograms, and degree of LAVVR from the first and last available follow-up echocardiograms. Associations with degree of early and late postoperative LAVVR were assessed with t-tests, ANOVA or Chi-square/Fisher's exact tests, and multivariable logistic regression.Fifty-eight patients (37% of 156) had significant (moderate or severe) early postoperative LAVVR. Thirty (32% of 93) had significant LAVVR after 6 or more months. Fewer patients with closely-spaced or asymmetric papillary muscles had moderate or severe late LAVVR versus those with widely-spaced papillary muscles (17% versus 40%, P=0.019). Controlling for weight at surgery, genetic syndromes and bypass time, widely-spaced papillary muscles increased the odds ratio for late LAVVR to 3.6 (P=0.026). Larger mural leaflet area was also associated with late LAVVR on univariable and multivariable analyses (P=0.019; P=0.023). A third of patients with significant late LAVVR had no significant early postoperative regurgitation.Mural leaflet and papillary muscle anatomy are associated with late LAVVR after AVC repair. Late regurgitation can develop in the absence of early LAVVR, suggesting different mechanisms.

  View details for DOI 10.1016/j.athoracsur.2020.01.012

  View details for PubMedID 32088289

• A standardized imaging protocol improves quality and reduces practice variability in echocardiographic assessment of ventricular function by first-year pediatric cardiology fellows. Echocardiography (Mount Kisco, N.Y.) White, B. R., Ho, D. Y., Rogers, L. S., Natarajan, S. S. 2019

  Abstract

  Echocardiography education for pediatric cardiology fellows has been a recent focus leading to the implementation of "boot camps." Less is described about continuing education through fellowship and improving image quality. We noticed practice variation in echocardiograms assessing ventricular function performed on nights and weekends. Thus, we implemented a standardized protocol and assessed its impact on imaging and reporting completeness.We created an imaging protocol for the assessment of ventricular function in the acute setting. The protocol included demographic information, a list of images to be obtained, and the methods to quantify ventricular function. The protocol was explained to first-year fellows and distributed on an electronic quick reference card. Echocardiograms independently performed by first-year fellows during their first 4 months of on-call time were assessed pre- and postintervention using a standard rubric.Compliance with demographic reporting was high pre- and postintervention, but significantly improved after the standardized protocol (P < 0.001). Use of the protocol increased the median number of unique images obtained per echocardiogram from 13 to 17 (out of 23 required views, P < 0.001). Particularly improved was the performance of quantitative evaluations of function, including Simpson's method for left ventricular ejection fraction (four chamber: 40% vs 67%, P < 0.001; two chamber: 33% vs 67%, P < 0.001) and tricuspid annular plane systolic excursion (45% vs 80%, P < 0.001).The introduction of a standardized imaging protocol and its distribution to first-year fellows resulted in improvements in echocardiographic reporting completeness and increased the quality of information obtained by providing more quantitative assessments of ventricular function.

  View details for DOI 10.1111/echo.14441

  View details for PubMedID 31385382

• Repair of Total Anomalous Pulmonary Venous Connection: Risk Factors for Postoperative Obstruction. The Annals of thoracic surgery White, B. R., Ho, D. Y., Faerber, J. A., Katcoff, H., Glatz, A. C., Mascio, C. E., Stephens, P., Cohen, M. S. 2019; 108 (1): 122-129

  Abstract

  Pulmonary venous obstruction after repair of total anomalous pulmonary venous connection (TAPVC) results in substantial morbidity and mortality. Risk factors for postoperative obstruction remain ambiguous. In addition, the existing literature has no standard definition for preoperative obstruction, making patient counseling difficult.All patients undergoing repair of TAPVC at our institution from January 1, 2006, to October 23, 2017, were identified. The primary outcome was the development of postoperative obstruction, analyzed as a time-to-event outcome. Clinical information was extracted to assess risk factors. Degrees of preoperative obstruction were defined based on echocardiographic, catheterization, and clinical findings. Univariable and multivariable Cox proportional hazard regression methods were used to identify factors associated with the primary outcome.During the study interval, 119 patients underwent repair of TAPVC (40% single ventricle), and postoperative obstruction developed in 25 patients (21%). Risk factors associated with obstruction were heterotaxy syndrome, single-ventricle heart disease, additional procedures at the time of vein repair, mixed-type TAPVC, and preoperative obstruction. Having even mild preoperative obstruction (≥1.2 m/s by Doppler echocardiography) was predictive of postoperative obstruction. A multivariable model showed mixed-type TAPVC and the presence of preoperative obstruction were associated with a more than twofold greater hazard of obstruction.TAPVC in the setting of heterotaxy and a single ventricle remains challenging, with high rates of postoperative obstruction. Mixed-type TAPVC is an independent risk factor for postoperative obstruction, particularly in patients with isolated TAPVC. Even mild preoperative obstruction is a risk factor for postoperative obstruction. These results may help risk-stratify TAPVC patients.

  View details for DOI 10.1016/j.athoracsur.2019.02.017

  View details for PubMedID 30885849

  View details for PubMedCentralID PMC6591098

• Postoperative Obstruction of the Pulmonary Veins in Mixed Total Anomalous Pulmonary Venous Connection. Pediatric cardiology Ho, D. Y., White, B. R., Glatz, A. C., Mascio, C. E., Stephens, P., Cohen, M. S. 2018; 39 (7): 1489-1495

  Abstract

  Total anomalous pulmonary venous connection (TAPVC) is a rare form of congenital heart disease in which the pulmonary veins drain by various pathways to the right atrium instead of the left atrium. Postoperative obstruction of the pulmonary veins is a known complication. Identifying risk factors for morbidity and mortality is important for counseling and monitoring. We describe a pattern of postoperative obstruction in a specific arrangement of mixed TAPVC. Five patients with a type of mixed TAPVC, namely, three pulmonary veins connecting to the coronary sinus and the left upper pulmonary vein (LUPV) connecting to the innominate vein, were identified over an 11-year period at our institution. Two additional patients with this TAPVC arrangement were cared for at our institution after having surgery at other institutions. Of these, one patient received only comfort care at birth due to other clinical issues. The six other patients underwent surgical unroofing of the coronary sinus. The anomalous LUPV was not addressed during the initial surgery in any of these cases. Following repair, one patient died from non-cardiac reasons. The remaining five patients all developed obstruction of the repaired pulmonary veins with decompression through the unrepaired LUPV, requiring surgical revision. Three patients underwent a second reoperation as well. Three of the six repaired patients also developed refractory atrial arrhythmias. This cohort suggests that this mixed TAPVC pattern predisposes patients to obstruction after surgical repair. Further investigation may aid pediatric cardiologists in risk-stratifying and counseling these patients. Alternative surgical approaches may need to be considered.

  View details for DOI 10.1007/s00246-018-1921-9

  View details for PubMedID 29872881

• The Parasternal Short-Axis View Improves Diagnostic Accuracy for Inferior Sinus Venosus Type of Atrial Septal Defects by Transthoracic Echocardiography. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Snarr, B. S., Liu, M. Y., Zuckerberg, J. C., Falkensammer, C. B., Nadaraj, S., Burstein, D., Ho, D., Gardner, M. A., Butto, A., Ewing, S. G., Pandian, N. G., Banerjee, A. 2017; 30 (3): 209-215

  Abstract

  Sinus venosus defects (SVD) of the inferior vena cava (IVC) type, or inferior SVDs, are an uncommon form of atrial communication located outside the confines of the fossa ovalis and involve override of the IVC. Despite numerous studies describing the anatomical and echocardiographic features of the inferior SVD, distinguishing this defect from a large secundum atrial septal defect (ASD) by echocardiography is often challenging. Accurate diagnosis of an inferior SVD and correct differentiation from a secundum ASD is essential for appropriate presurgical planning. Absence of the posterior rim in the parasternal short-axis views may serve as a useful clue in diagnosing inferior SVDs. We sought to determine the utility of using the presence or absence of a posterior atrial rim in the parasternal short-axis view to help distinguish an inferior SVD from a secundum ASD. This sign may help clinch the diagnosis when subcostal imaging is suboptimal.We retrospectively reviewed transthoracic echocardiograms from 15 patients with a known surgical diagnosis of an inferior SVD between 2004 and 2015. The presence or absence of a posterior rim in the parasternal short-axis view was determined by two primary investigators. The posterior rim was also evaluated in 14 patients with a secundum ASD repair as controls. Echocardiograms were then reviewed blindly by attending-level echocardiographers and cardiology fellows in training. Diagnostic accuracy was assessed both with and without the use of the posterior rim criterion. Statistical analysis was used to determine the effect of using the rim criterion on inferior SVD diagnosis. We also reviewed all surgically diagnosed secundum ASDs that were incorrectly diagnosed as inferior SVD by preoperative imaging and determined whether use of the posterior rim criterion would have resulted in the correct diagnosis.The posterior rim was absent in all 15 patients with a surgical diagnosis of inferior SVD and present in all 14 patients with a secundum ASD. For all observers, there was a statistically significant increase in diagnostic accuracy of inferior SVDs with the use of the rim criterion (P < .0001). We noted that secundum ASDs with inferior extension also have persistent posterior rims. The rim criterion correctly classified all large secundum ASDs with inferior extension that were previously misdiagnosed by echocardiogram preoperatively.Absence of the posterior rim ("bald" posterior wall) is a consistent finding in patients with an inferior SVD and distinguishes an inferior SVD from a large secundum ASD with inferior extension. Parasternal short-axis evaluation of the posterior atrial rim is a helpful tool for all levels of physician training in improving diagnostic accuracy for detecting inferior SVDs and in distinguishing them from secundum ASDs.

  View details for DOI 10.1016/j.echo.2016.12.007

  View details for PubMedID 28139440

  View details for PubMedCentralID PMC5837475

• Effect of Race on the Prevalence of Congenital Malformations among Newborns in the United States. Ethnicity & disease Egbe, A., Lee, S., Ho, D., Uppu, S. 2015; 25 (2): 226-31

  Abstract

  Racial variability in certain prenatal risk factors, such as prenatal vitamin supplementation and termination of pregnancy for fetal anomaly, has altered the racial prevalence of congenital malformation (CM). Analysis of a single large representative population is required to analyze current racial differences in prevalence of CM in the United States.This is a population-based cross-sectional study to analyze racial differences in prevalence of CM diagnoses. We reviewed all live births in the 2008 Nationwide Inpatient Sample (NIS) database and determined birth prevalence of 55 selected CM diagnoses in Caucasians. We then calculated the relative risk of these CM diagnoses in African American, Hispanics and Asians relative to Caucasians.Overall CM prevalence was 29.2 per 1,000 in a cohort of 1,048,252 live births of which 51% were Caucasians. Compared to Caucasian, risk of overall CM was lower in African Americans (RR = .9, CI .8-9) and Hispanics (RR = .9, CI .8-.9). Risk of overall CM was similar in Caucasians and Asians. Relative to the Caucasians, African Americans had lower risk of cardiac, genitourinary, and craniofacial malformations but higher risk of musculoskeletal malformations. Hispanics had lower risk of genitourinary and gastrointestinal malformation. Asians had higher risk of craniofacial and musculoskeletal malformation.This is a comprehensive description of racial difference in risk of CM in the United States. Observed racial differences in risk of CM may be related to genetic susceptibilities, to cultural or social differences that could modify exposures, or to the many potential combinations between susceptibilities and exposures.

  View details for PubMedID 26118153

• Congenital malformations in the newborn population: a population study and analysis of the effect of sex and prematurity. Pediatrics and neonatology Egbe, A., Uppu, S., Lee, S., Stroustrup, A., Ho, D., Srivastava, S. 2015; 56 (1): 25-30

  Abstract

  Congenital malformation (CM) is a leading cause of infant mortality. We hypothesized that the current estimates of the prevalence of CM are obsolete because of the increased rate of terminating fetuses with severe CMs and the widespread use of prenatal vitamins.This population-based cross-sectional study analyzed the effect of sex and prematurity on CM prevalence. All data were derived from birth entries in the 2008 Nationwide Inpatient Sample (NIS) database. Our objectives were to determine the prevalence of CM diagnoses among all birth hospitalizations in 2008 and to analyze the effect of sex and gestational maturity on CM prevalence.We identified 29,312 patients with CMs from among 1,014,261 live births, which yielded a CM prevalence of 28.9 per 1000 live births. Associated genetic syndromes were present in 1172 (4%) patients. Among newborns with nonsyndromic CM, 91% of newborns had an isolated CM and 9% of newborns had multiple CMs. The cardiovascular system was the most commonly involved organ system. The risk of CM was significantly higher in preterm newborns for an isolated CM [odds ratio (OR), 1.5; confidence interval (CI), 1.4-1.5]; multiple CMs (OR, 2.1; CI, 2.0-2.3); and overall CMs (OR, 1.4; CI, 1.3-1.5). Males had higher risk of isolated CMs (OR, 1.3; CI, 1.2-1.5). However, there was no sex difference in the risk of overall CM.We reported up-to-date national estimates of the prevalence of CM, which is important for monitoring trends, determining service planning, and assessing disease burden because of congenital malformations in the United States of America. We also showed a strong association between CM and prematurity. Further study of this association is needed to provide insight into the etiology of these relatively common public health problems.

  View details for DOI 10.1016/j.pedneo.2014.03.010

  View details for PubMedID 25267275

• Racial/ethnic differences in the birth prevalence of congenital anomalies in the United States. Journal of perinatal medicine Egbe, A., Lee, S., Ho, D., Uppu, S., Srivastava, S. 2015; 43 (1): 111-7

  Abstract

  This work aimed to analyze a single large representative population to determine racial/ethnic differences in the prevalence of congenital anomalies (CA).This is a population-based comprehensive analysis of racial/ethnic differences in the prevalence of CA diagnoses. We reviewed all live births in the 2008 Nationwide Inpatient Sample (NIS) database and determined the birth prevalence of 55 selected CA diagnoses in Caucasians. We then calculated the relative risk of these CA diagnoses in African American, Hispanics, and Asians relative to Caucasians.Overall CA prevalence was 29.2 per 1000 in a cohort of 1,048,252 live births, of which 51% were Caucasians. Compared with Caucasians, risk of overall CA was lower in African Americans (RR=0.9, CI: 0.8-0.9) and Hispanics (RR=0.9, CI: 0.8-0.9). Risk of overall CA was similar in Caucasians and Asians. Relative to the Caucasians, African Americans had lower risk of cardiac, genitourinary, and craniofacial CA but higher risk of musculoskeletal anomalies. Hispanics had lower risk of genitourinary and gastrointestinal CA. Asians had higher risk of craniofacial and musculoskeletal CA.Observed racial/ethnic differences in risk of CA may be related to genetic susceptibilities, cultural or social differences that could modify exposures, or many potential combinations between susceptibilities and exposures.

  View details for DOI 10.1515/jpm-2013-0344

  View details for PubMedID 24837488

• Prevalence of associated extracardiac malformations in the congenital heart disease population. Pediatric cardiology Egbe, A., Uppu, S., Lee, S., Ho, D., Srivastava, S. 2014; 35 (7): 1239-45

  Abstract

  The authors hypothesized that changes in prenatal factors such as termination of pregnancy for fetal anomalies and prenatal vitamin supplementation have altered the epidemiology of patients with multiple congenital anomalies and may have had an impact on their prevalence in the current era. This study reviewed the Nationwide Inpatient Sample database from 1998 to 2008 and compared the prevalence of ECM among live births with a CHD diagnosis (case) and that among live births without a CHD diagnosis (control). For this study, 42 ECM and 10 CHD diagnoses were selected for subanalysis. Longitudinal analysis also was performed to determine temporal variation of ECM prevalence in the CHD population during the 11-year study period. The cohort in this study consisted of 97,154 patients in the case group and 12,078,482 subjects in the control group. The prevalences in the CHD population were 11.4 % for nonsyndromic congenital malformation (NSCM), 2.2 % for genetic syndrome (GS), and 13.6 % for overall extracardiac congenital malformation (ECM). The prevalences in the control group were 6.7 % for NSCM, 0.3 % for GS, and 7.0 % for ECM. The findings showed a strong association of NSCM [odds ratio (OR) 1.88; 95 % confidence interval (CI) 1.73-1.94], GS (OR 2.52; 95 % CI 2.44-2.61), and overall ECM (OR 2.01; 95 % CI 1.97-2.14) with CHD. The prevalences of GS and multiple organ system CM decreased significantly during the study period. This study was the largest and most comprehensive population-based study to evaluate the association between CHD and ECM in newborns.

  View details for DOI 10.1007/s00246-014-0922-6

  View details for PubMedID 24823885

• Changing prevalence of severe congenital heart disease: a population-based study. Pediatric cardiology Egbe, A., Uppu, S., Lee, S., Ho, D., Srivastava, S. 2014; 35 (7): 1232-8

  Abstract

  Although the epidemiology of congenital heart disease (CHD) has been described, the authors believe changes in prenatal factors such as termination of pregnancy for fetal anomaly and prenatal vitamin supplementation have altered the birth prevalence of severe CHD. This population-based study reviewed the Nationwide Inpatient Sample (NIS) database and identified all cases with a severe CHD diagnosis among all live birth entries from 1999 to 2008. A time trend analysis then was performed for specific severe CHD diagnoses stratified by race, socioeconomic status, and geographic location. Overall, severe CHD prevalence was 147.4 per 100,000 live births, with a temporal decrease in prevalence from 168.9 per 100,000 in 1999 to 129.3 per 100,000 in 2008 (p = 0.03). Among the 12 severe CHD diagnoses included in our cohort, the prevalence of truncus arteriosus (p = 0.02), tetralogy of Fallot (p = 0.001), hypoplastic left heart syndrome (p = 0.001), and pulmonary atresia (p = 0.01) decreased significantly during the study period. The observed prevalence trends varied significantly by race (Caucasians), socioeconomic class (upper income quartiles), and geographic location (Northeast and West regions). The study findings showed a temporal decrease in severe CHD prevalence, which varied by race, socioeconomic status, and geographic location. The authors speculated that the observed trend might be due to increased termination of fetuses with prenatally diagnosed CHD. The impact of sociodemographic variables on the observed prevalence trend might be due to differences in access to specialized perinatal care and fetal heart programs or because of variability in termination of pregnancy.

  View details for DOI 10.1007/s00246-014-0921-7

  View details for PubMedID 24823884

• Primary tetralogy of Fallot repair: predictors of intensive care unit morbidity. Asian cardiovascular & thoracic annals Egbe, A. C., Uppu, S. C., Mittnacht, A. J., Joashi, U., Ho, D., Nguyen, K., Srivastava, S. 2014; 22 (7): 794-9

  Abstract

  Primary repair of tetralogy of Fallot has low surgical mortality, but some patients still experience significant postoperative morbidity. Our objectives were to review our institutional experience with primary tetralogy of Fallot repair, and identify predictors of intensive care unit morbidity.We reviewed all patients with tetralogy of Fallot who underwent primary repair in infancy from 2001 to 2012. Preoperative, operative, and postoperative demographic and morphologic data were analyzed. Intensive care unit morbidity was defined as prolonged intensive care unit stay (≥ 7 days) and/or prolonged duration of mechanical ventilation (≥ 48 h).97 patients who underwent primary surgical repair during the study period were included in the study. The median age was 4.9 months (range 1-9 months) and the median weight was 5.3 kg (range 3.1-9.8 kg). There was no early surgical mortality. The incidence of junctional ectopic tachycardia and persistent complete heart block was 2% and 1%, respectively. The median intensive care unit stay was 6 days (range 2-21 days) and the median duration of mechanical ventilation was 19 h (range 0-136 h). Age and weight were independent predictors of intensive care unit stay, while surgical era predicted the duration of mechanical ventilation.Primary tetralogy of Fallot repair is a safe procedure with low mortality and morbidity in a medium-sized program with outcomes comparable to national standards. Age and weight at the time of surgery were significant predictors of morbidity.

  View details for DOI 10.1177/0218492313513773

  View details for PubMedID 24887913

• Incidences and sociodemographics of specific congenital heart diseases in the United States of America: an evaluation of hospital discharge diagnoses. Pediatric cardiology Egbe, A., Uppu, S., Stroustrup, A., Lee, S., Ho, D., Srivastava, S. 2014; 35 (6): 975-82

  Abstract

  Current estimates of the incidence of congenital heart disease (CHD) are derived from small clinical studies and metaanalyses. For the true incidence of CHD in the United States of America to be estimated, a single large representative population must be analyzed. All the data in this study were derived from the Nationwide Inpatient Sample database. The study determined the overall and lesion-specific incidences of CHD diagnoses among all birth hospitalizations in 2008, stratified by race, gender, socioeconomic status, and hospital geographic location. The study identified 13,093 CHD diagnoses among 1,204,887 birth hospitalizations, yielding an incidence of 10.8 per 1,000, with a predominance of mild lesions and septal defects. Atrial septal defect (ASD) and pulmonic stenosis were more common among females, whereas aortic stenosis, coarctation of the aorta, hypoplastic left heart syndrome, and d-transposition of great arteries were more common among males. No racial difference was observed in the overall CHD incidence. However, isolated patent ductus arteriosus (PDA) and ventricular septal defects (VSDs) were more common among Caucasians, whereas ASDs were more common among Hispanics. The incidences of CHD diagnoses were similar for all socioeconomic classes except the lowest socioeconomic class, which had a significantly lower CHD incidence. There was no geographic or seasonal variation in CHD incidence. This study demonstrated the incidence of echocardiographically confirmed CHD diagnosis to be 10.8 per 1,000 live births, marked by a high proportion of mild cardiac lesions and isolated PDAs. The high incidence of isolated PDAs in this study may be explained by the inclusion of only CHD diagnoses during birth hospitalization.

  View details for DOI 10.1007/s00246-014-0884-8

  View details for PubMedID 24563074

• Prevalence of congenital anomalies in newborns with congenital heart disease diagnosis. Annals of pediatric cardiology Egbe, A., Lee, S., Ho, D., Uppu, S., Srivastava, S. 2014; 7 (2): 86-91

  Abstract

  There is a known association between congenital heart disease (CHD) and other congenital anomalies (CA). These associations have been altered by changes in prenatal factors in recent time. We reviewed the largest database of inpatient hospitalization information and analyzed the current association between common CHD diagnoses and other congenital anomalies.Case-control study design. We reviewed the Nationwide Inpatient Sample (NIS) database from 1998 to 2008 and identified all live births with CHD diagnosis (case) and live births without CHD diagnosis (control). We compared prevalence of associated congenital anomalies between the case and control groups.Our cohort consisted of 97,154 and 12,078,482 subjects in the case and control groups, respectively. In the CHD population, prevalence of non-syndromic congenital anomaly (NSCA), genetic syndrome (GS), and overall extra-cardiac congenital anomaly (CA) were 11.4, 2.2, and 13.6%, respectively. In the control group, prevalence of NSCA, GS, and CA were 6.7, 0.3, and 7.0%, respectively. NSCA (odds ratio (OR): 1.88, confidence interval (CI): 1.73-1.94), GS (OR 2.52, CI 2.44-2.61), and overall CA (OR: 2.01, CI: 1.97-2.14) were strongly associated with CHD. Prevalence of GS and multiple organ-system CA decreased significantly over the study period.This is the largest and most comprehensive population-based study evaluating association between CHD and extra-cardiac malformation (ECM) in newborns. There was significant decrease in prevalence of GS and multiple CA over the study period.

  View details for DOI 10.4103/0974-2069.132474

  View details for PubMedID 24987252

  View details for PubMedCentralID PMC4070214

• Temporal variation of birth prevalence of congenital heart disease in the United States. Congenital heart disease Egbe, A., Uppu, S., Lee, S., Stroustrup, A., Ho, D., Srivastava, S. 2014; 10 (1): 43-50

  Abstract

  This is a longitudinal analysis of the largest and most comprehensive inpatient care database in the United States to determine temporal variation of birth prevalence of congenital heart disease (CHD) diagnosis, adjusting for potentially confounding factors.We compared all entries of CHD diagnoses in the Nationwide Inpatient Sample (NIS) database in 1998 and 2008 to determine differences in birth prevalence of overall CHD and specific CHD phenotypes stratified by race, gender, socioeconomic status, and geographical location.CHD prevalence was 10.2/1000 in 1998 and 10.8/1000 live births in 2008, without significant changes in prevalence (P = .09). Prevalence of isolated patent ductus arteriosus increased from 1.9 to 2.8 per 1000 (P < .001), and this temporal increase remained statistically significant after stratification by race, income status, and geographic location. Prevalence of mild CHD increased from 8.0 to 9.1 per 1000 (P = .01), with most of this increase occurring among Caucasians and the upper socioeconomic class. There was a decrease in prevalence of severe CHD from 1.5 to 0.9 (P = .03), while prevalence of moderate CHD and all other specific CHD phenotypes remained unchanged in both cohorts.We report increased prevalence of isolated patent ductus arteriosus and mild CHD; decreased prevalence of severe CHD; and unchanged prevalence of overall CHD and all other specific CHD phenotype. We speculate that increased prevalence of mild CHD was due to increased case detection because of improvement in echocardiography. Decrease in diagnosis of severe CHD could be due to the impact of pregnancy termination.

  View details for DOI 10.1111/chd.12176

  View details for PubMedID 24612877

• High-molecular-weight and total adiponectin levels and incident symptomatic peripheral artery disease in women: a prospective investigation. Circulation Ho, D. Y., Cook, N. R., Britton, K. A., Kim, E., Creager, M. A., Ridker, P. M., Pradhan, A. D. 2011; 124 (21): 2303-11

  Abstract

  Adiponectin is linked to reduced diabetes risk and may be antiatherogenic, yet clinical data show no consistent relationship with incident cardiovascular events, especially among women. To our knowledge, no prior prospective studies have evaluated adiponectin, including high-molecular-weight (HMW) adiponectin, and incident peripheral artery disease (PAD).We evaluated the relationship of total adiponectin, HMW adiponectin, and the HMW-to-total adiponectin ratio with incident symptomatic PAD in a prospective, nested case-control study conducted within the Women's Health Study (n=110 cases, n=230 controls, frequency matched in strata defined by 5-year age categories, smoking, fasting status, and follow-up time; median cohort follow-up=13.2 years). Baseline median levels of HMW and total adiponectin were significantly lower in women developing PAD than in those remaining event free (HMW: 3.3 versus 3.8 μg/mL, P=0.0005; total: 5.6 versus 7.4 μg/mL, P<0.0001). The ratio did not differ significantly between groups. Age-adjusted PAD odds ratios (95% confidence intervals) across tertiles were 1.0, 0.66 (0.39-1.13), and 0.40 (0.22-0.74) for HMW and 1.0, 0.74 (0.43-1.25), and 0.35 (0.18-0.65) for total adiponectin (P(trend)=0.004 and 0.001, respectively). Results were similar after adjustment for traditional cardiovascular risk factors, use of postmenopausal hormone therapy, high-sensitivity C-reactive protein, soluble intercellular adhesion molecule-1, leptin, hemoglobin A(1c), and fasting insulin (adjusted odds ratio and 95% confidence interval for HMW: 1.0, 0.62 [0.29-1.34], 0.30 [0.12-0.74]; total: 1.0, 0.46 [0.22-1.00], 0.30 [0.12-0.76]; P(trend)=0.01 for both).Total and HMW adiponectin are inversely associated with incident PAD among initially healthy women. These prospective data support a protective role for this adipokine in peripheral atherosclerosis development.

  View details for DOI 10.1161/CIRCULATIONAHA.111.045187

  View details for PubMedID 22025604

  View details for PubMedCentralID PMC3256987

• Determining and addressing obstacles to the effective use of long-lasting insecticide-impregnated nets in rural Tanzania. Malaria journal Widmar, M., Nagel, C. J., Ho, D. Y., Benziger, P. W., Hennig, N. 2009; 8: 315

  Abstract

  The objective of this project was to achieve high, sustainable levels of net coverage in a village in rural Tanzania by combining free distribution of long-lasting insecticide-impregnated nets (LLINs) with community-tailored education. In Tanzania, malaria is the leading cause of morbidity and mortality. Although malaria bed nets have a well-established role in reducing disease burden, few rural households have access to nets, and effective use depends on personal practices and attitudes.Five practices and attitudes inconsistent with effective LLIN use were identified from household interviews (n = 10). A randomized survey of villagers (n = 132) verified local prevalence of these practices and attitudes. Community leaders held an educational session for two members of every household addressing these practice and attitudes, demonstrating proper LLIN use, and emphasizing behaviour modification. Attendees received one or two LLINs per household. Surveys distributed three weeks (n = 104) and 15 months (n = 104) post-intervention assessed corrected practices and attitudes. Project efficacy was defined by correction of baseline practices and attitudes as well as high rates of reported daily net use, with statistical significance determined by chi-square test.Baseline interviews and surveys revealed incorrect practices and attitudes regarding 1) use of nets in dry season, 2) need to retreat LLINs, 3) children napping under nets, 4) need to repair nets, and 5) net procurement as a priority, with 53- 88.6% incorrect responses (11.4-47% correct responses). A three-week follow-up demonstrated 83-95% correct responses. Fifteen-month follow-up showed statistically significant (p < 0.01) corrections from baseline in all five practice and attitudes (39.4-93.3% correct answers). 89.4% of respondents reported using their nets every night, and 93.3% affirmed purchase of nets as a financial priority.Results suggest that addressing community-specific practices and attitudes prior to LLIN distribution promotes consistent and correct use, and helps change attitudes towards bed nets as a preventative health measure. Future LLIN distributions can learn from the paradigm established in this project.

  View details for DOI 10.1186/1475-2875-8-315

  View details for PubMedID 20043830

  View details for PubMedCentralID PMC2809069