Santosh Uppu

Use of 3-Dimensional Printing to Demonstrate Complex Intracardiac Relationships in Double-Outlet Right Ventricle for Surgical Planning

Double-outlet right ventricle falls under the category of congenital heart disease known as conotruncal defects, which possess abnormal ventriculoarterial relationships.1 For complex cases, the surgeon must determine whether the left ventricle and one of the great arteries can be aligned using the ventricular septal defect to construct an unobstructed pathway or baffle, resulting in a 2-ventricle repair.2 Creation of the baffle can be complicated by anatomic obstructions because of prominent conal septum, straddling atrioventricular valve attachments, or location of the ventricular septal defect in the inlet septum, remote from any great artery. Three-dimensional (3D) printing has been applied in the management of many different congenital heart diseases.3 In this specific patient population, in whom communicating the complex intracardiac anatomy to the surgeon is so critical, the use of 3D modeling and printing is invaluable. We used this approach in a patient with dextrocardia, complex double-outlet right ventricle (S,L,A)1 and supratricuspid ring. She underwent pulmonary artery banding in infancy and had been doing relatively well clinically; so that any further surgical intervention was deferred until she was 8 years old. Although she was growing well and required no medication, …

Temporal Variation of Birth Prevalence of Congenital Heart Disease in the United States

BACKGROUND 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. METHODS 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. RESULTS 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. CONCLUSIONS 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.

Prevalence of congenital anomalies in newborns with congenital heart disease diagnosis.

Background: 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. Materials and Methods: 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. Results: 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. Conclusions: 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.

Congenital malformations in the newborn population: A population study and analysis of the effect of sex and prematurity

BACKGROUND 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. METHODS 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. RESULTS 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. CONCLUSION 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.

Primary tetralogy of Fallot repair: Predictors of intensive care unit morbidity

Background 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. Methods 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). Results 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. Conclusion 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.

CMR techniques and findings in children with myocarditis: a multicenter retrospective study

Background CMR is increasingly used to diagnose myocarditis in adults but its use in pediatric-age pts is not well estab- lished. We sought to describe the clinical presentation, CMR imaging protocols, CMR findings, and outcomes in a multicenter cohort of children with myocarditis. Methods 71 studies (66%) and was abnormal in 49 (69%). First pass contrast perfusion (FPP) imaging was performed in 42 studies (45%) and was abnormal in 4 (10%). T1- weighted imaging for early gadolinium enhancement (EGE) was performed in 35 studies (37%) and was abnor- mal in 19 (51%). Late gadolinium enhancement (LGE) imaging was performed in all studies, and was abnormal in 93 (83%) with the following reported distributions: 89% subepicardial or midwall, 6% patchy, 3% sub-endo- cardial, 1% transmural, and 1% diffuse. The CMR study was interpreted as positive for myocarditis in 96 pts (87%), negative in 11 (10%), and equivocal in 4 (4%), yielding a sensitivity of 86% for an ultimate clinical diag- nosis of myocarditis. There was significant practice varia- tion in the use of T2W, FPP, and EGE imaging among the participating institutions (Figure 1). At a median fol- low-up time from CMR of 6 mo (0.2-217), all patients were alive and 3 had undergone cardiac transplantation, all of whom had CMR studies positive for myocarditis. Conclusions This is the largest study to date describing the CMR findings in children with myocarditis. The CMR techni- ques used, from most to least common, were LGE, T2W, FPP, and EGE. Abnormalities were most often seen with LGE followed by T2W, EGE, and FPP. There was significant practice variation in the CMR protocol between institutions. The information from this study should be useful in planning a prospective study to eval- uate the diagnostic and predictive performance of CMR in children with suspected myocarditis. Funding None.