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Open in new tab Download slide OBJECTIVES Children with Alagille syndrome often have complex forms of congenital heart defects with the majority having peripheral pulmonary artery stenosis (PPAS) and pulmonary valve atresia (PA) or pulmonary valve stenosis. Children with Alagille syndrome also have variable amounts of liver dysfunction. The purpose of this study was to evaluate the impact of liver dysfunction on outcomes in children with Alagille syndrome undergoing congenital heart surgery. METHODS This was a retrospective review of 69 patients with Alagille syndrome who underwent congenital heart surgery at our institution. The underlying diagnoses included PPAS (n  = 29), tetralogy of Fallot with PPAS (n  = 14), tetralogy with PA (n  = 3), PA with ventricular septal defect and major aortopulmonary collateral arteries (n  = 21) and one each with D-transposition and supravalvar aortic stenosis. RESULTS The median age at surgery was 16 months (range 0–228 months). Procedures performed included PPAS repair (n  = 43), tetralogy with PA repair (n  = 3), unifocalization procedures (n  = 21) and other (n  = 2). Forty-two (61%) patients had mild or no liver dysfunction, while 26 (38%) had moderate or severe liver dysfunction. The median cardiopulmonary bypass time was 345 min (341 with liver dysfunction, 345 without liver dysfunction). There were a total of 8 operative (12%) deaths and 3 late (4%) deaths. Six operative and 2 late deaths occurred in patients with liver dysfunction (combined 30.7%) versus 2 operative and 1 late death (combined 7.1%) for patients without liver dysfunction (P  < 0.05). CONCLUSIONS These results suggest that liver dysfunction has a profound impact on survival in children with Alagille syndrome undergoing congenital heart surgery. [ABSTRACT FROM AUTHOR]

Background: There is emerging evidence that children with complex congenital heart defects (CHDs) are at increased risk for childhood lymphoma, but the mechanisms underlying this association are unclear. Thus, we sought to evaluate the role of DNA methylation patterns on "CHD‐lymphoma" associations. Methods: From >3 million live births (1988–2004) in California registry linkages, we obtained newborn dried bloodspots from eight children with CHD‐lymphoma through the California BioBank. We performed case–control epigenome‐wide association analyses (EWAS) using two comparison groups with reciprocal discovery and validation to identify differential methylation associated with CHD‐lymphoma. Results: After correction for multiple testing at the discovery and validation stages, individuals with CHD‐lymphoma had differential newborn methylation at six sites relative to two comparison groups. Our top finding was significant in both EWAS and indicates PPFIA1 cg25574765 was hypomethylated among individuals with CHD‐lymphoma (mean beta = 0.04) relative to both unaffected individuals (mean beta = 0.93, p = 1.5 × 10−12) and individuals with complex CHD (mean beta = 0.95, p = 3.8 × 10−8). PPFIA1 encodes a ubiquitously expressed liprin protein in one of the most commonly amplified regions in many cancers (11q13). Further, cg25574765 is a proposed marker of pre‐eclampsia, a maternal CHD risk factor that has not been fully evaluated for lymphoma risk in offspring, and the tumor microenvironment that may drive immune cell malignancies. Conclusions: We identified associations between molecular changes present in the genome at birth and risk of childhood lymphoma among those with CHD. Our findings also highlight novel perinatal exposures that may underlie methylation changes in CHD predisposing to lymphoma. [ABSTRACT FROM AUTHOR]

Background: Outcomes for congenital heart disease have dramatically improved over the past several decades. However, there are patients who encounter intraoperative or postoperative complications and ultimately do not survive. It was our hypothesis that the number of postoperative procedures (including surgical and unplanned diagnostic procedures) would correlate with hospital length of stay and operative mortality.Methods: This was a retrospective review of 938 consecutive patients undergoing congenital heart surgery at a single institution over a 2-year timeframe. The number of postoperative surgical and unplanned diagnostic procedures were counted and the impact on hospital length of stay and mortality was assessed.Results: 581 of the 938 (62%) patients had zero postoperative diagnostic or surgical procedures. These patients had a median length of stay of 6 days with a single operative mortality (0.2%). 357 of the 938 (38%) patients had one or more postoperative diagnostic or surgical procedures. These patients had a total of 1586 postoperative procedures. There was a significant correlation between the number of postoperative procedures and both hospital length of stay and mortality (p < .001). Patients who required 10 or more postoperative procedures had a median hospital length of stay of 89 days and had a 50% mortality. There were no survivors in patients who had 15 or more postoperative procedures.Conclusions: The data demonstrate that the number of postoperative procedures was highly correlated with both hospital length of stay and mortality. [ABSTRACT FROM AUTHOR]

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (MAPCAs) is a relatively rare and complex form of congenital heart disease. Unifocalization of MAPCAs has been advocated by some groups for the treatment of this condition. The purpose of this study was to assess the midterm fate of unifocalized MAPCAs in a cohort of patients with retroesophageal MAPCAs. This was a retrospective review of 37 patients who underwent a unifocalization procedure. All patients in this study had 1 or more retroesophageal MAPCAs, and detailed mapping of the MAPCAs was made based on a combination of the cardiac catheterization and surgical findings. The 37 patients had a total of 166 MAPCAs, or 4.5 MAPCAs per patient. One hundred twenty-nine (78%) MAPCAs were unifocalized, whereas 37 (22%) were ligated because they were dual supply. Median follow-up was 69 months. At follow-up cardiac catheterization, evaluation of the 129 unifocalized MAPCAs demonstrated that 123 (95%) had antegrade flow, whereas 6 were occluded. For the 123 MAPCAs with antegrade flow, 97 (80%) were widely patent, whereas 26 were stenotic. Thirteen of the 37 patients have subsequently undergone reintervention on MAPCAs that were determined to be stenotic following unifocalization. Seven of these patients had mild disease and had complete resolution with balloon (n = 5) or surgical revision (n = 2). Six patients with moderate or severe disease underwent surgical revision with confirmed resolution in 4 of 6. The data demonstrate that the majority of unifocalized MAPCAs remain widely patent following unifocalization. However, one-quarter of unifocalized MAPCAs develop stenoses or occlusion. These results suggest the fate for most unifocalized MAPCAs is favorable but highlight the need for close vigilance. Midterm fate of unifocalized MAPCAs. Ninety-five percent of unifocalized MAPCAs had antegrade flow at follow-up evaluation. Of these, 80% were widely patent, whereas 20% had some degree of stenosis. Reinterventions on MAPCAs with stenoses were 100% successful. [Display omitted] [ABSTRACT FROM AUTHOR]

Congenital heart disease is exceedingly prevalent in trisomy 13 and 18. Improved survival following congenital heart surgery has been reported, however, mortality remains significantly elevated. Utilizing inpatient data on trisomy 13 and 18 from the 2003–2016 Pediatric Health Information System database, a survival model was developed and validated using data from the California Perinatal Quality Care Collaborative and the California Office of Statewide Health Planning and Development. The study cohort included 1,761 infants with trisomy 13 and 18. Two models predicting survival to 6 months of age were developed and tested. The initial model performed excellently, with a c‐statistic of 0.87 and a c‐statistic of 0.76 in the validation cohort. After excluding procedures performed on the day of death, the revised model's c‐statistic was 0.76. Certain variables, including cardiac surgery, gastrostomy, parenteral nutrition, and mechanical ventilation, are predictive of survival to 6 months of age. This study presents a model, which potentially can inform decision‐making regarding congenital heart surgery. [ABSTRACT FROM AUTHOR]

Peripheral pulmonary artery stenosis is a relatively rare form of congenital heart disease typically associated with genetic syndromes, such as Williams or Alagille syndromes. However, some patients present with severe stenosis without associated syndromes. The purpose of the study was to review our surgical experience in such patients. This was a retrospective review of 30 patients who underwent surgical repair for peripheral pulmonary artery stenosis. Concomitant anatomical diagnoses in 20 patients (67%) included supravalvar aortic stenosis (n = 8), tetralogy of Fallot (n = 4), d-transposition of the great arteries (n = 2), truncus arteriosus (n = 2), hypoplastic left heart syndrome (n = 2), ventricular septal defect (n = 1), and patent ductus arteriosus (n = 1). Additional medical diagnoses in 15 patients (50%) included elastin arteriopathy (n = 9), pulmonary artery calcinosis (n = 1), arterial tortuosity syndrome (n = 1), DiGeorge syndrome (n = 1), and Noonan syndrome (n = 1). Median age at surgery was 3.6 years (interquartile range 1.6-7.4 years). Seventeen patients (57%) had prior cardiac operations, and 16 patients (53%) previously underwent percutaneous intervention. With surgery, mean right ventricle-to-aortic systolic pressure ratio decreased from 0.95 ± 0.2 to 0.28 ± 0.08 (P< 0.0001). Median duration of cardiopulmonary bypass was 369 minutes. There was 100% survival to hospital discharge, with no mortality at mean follow-up of 2.3 years. No patient required reoperation, while 4 underwent balloon dilation. Freedom from pulmonary artery catheter-based reintervention was 95% and 80% at 12 and 36 months, respectively. Patients with peripheral pulmonary artery stenosis without either Williams or Alagille syndrome can successfully undergo surgical repair with a significant reduction in right ventricle-to-aortic pressure ratios. [ABSTRACT FROM AUTHOR]

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease, making up 10% of all congenital heart defects. Annual follow-up echocardiograms are recommended in patients with repaired ToF, but evidence-based guidelines do not exist. We hypothesized that most echocardiograms performed in asymptomatic patients with repaired ToF and no physical exam change do not result in an actionable change (AC) in management. We retrospectively reviewed records of all patients with ToF and prior complete repair at our institution between January 2000 and September 2015. Changes in echocardiograms resulting in hospital admission, medication addition/change, cardiac catheterization, or surgical procedure were identified via chart review. These changes were referred to as an AC. A total of 1135 echocardiograms were reviewed from 233 patients (160 with initial complete repair, 70 with prior shunt, and 3 with other initial surgery). The median number of echocardiograms per patient was 5. Of the 1135 echocardiograms, 15 (1.3%) were associated with AC. Of the 15 patients with AC echocardiograms, 9 underwent a shunt prior to complete repair (9/70, 12.9%) and 6 had undergone an initial complete repair (6/160, 3.8%). The median age at AC was 6.3 years (IQR 4.4, 6.8) in the shunt group and 0.90 years (IQR 0.87, 1.1) in the initial complete repair group. In asymptomatic patients with repaired ToF and no physical exam change, echocardiograms rarely lead to a change in clinical management. In conclusion, the likelihood and timing of AC echocardiograms and reinterventions vary based on the type of initial surgery. [ABSTRACT FROM AUTHOR]

Pulmonary artery (PA) stenosis is the most common late sequela following arterial switch for d-transposition of the great arteries. The purpose of this study was to assess the effectiveness of transthoracic echocardiography in evaluating the pulmonary arteries following repair. This was a retrospective, cross-sectional analysis of all echocardiograms performed on patients following arterial switch operation. A numerical scoring system was devised and used to quantify PA visualization based on 2D images, color mapping, and spectral Doppler. The study cohort included 150 patients. The ability to visualize at least one PA was poorer in patients who were older [> 10 years (47%) vs ≤ 10 years (89%) (p < 0.001)], and who had larger body surface area (BSA) (> 1.25 m2 (40%) vs ≤ 1.25 m2 (90%) (p < 0.001)]. Regardless of age, 2D visualization of the pulmonary arteries was poor for the entire cohort. Of those with at least one non-visualized PA, only 54% had alternative imaging performed or ordered within the 5 years at or prior to their last echocardiogram. In conclusion, PA visualization following arterial switch is worse in patients who are older and in those with larger BSA. In such patients, alternative forms of imaging are more likely to be necessary. [ABSTRACT FROM AUTHOR]

Background: Childhood cancer is increased in those with birth defects, including those with congenital heart disease (CHD). Lymphoma risk is increased in children with CHD. This study analyzes the effect of CHD and CHD severity on childhood cancer risk. Methods: We analyzed cancer risk in a population‐based cohort of children with and without CHD born between 1988 and 2004 by linking data from the California Birth Defects Monitoring Program with data from the California Cancer Registry. We compared cancer risk in children with and without CHD, excluding children with chromosomal anomalies. Results: Of >3 million children in the birth cohort, 65,585 had birth defects (2%), 25,981 with CHD. Cancer occurred in 4,781 (0.15%) children, 43 (0.17%) with CHD. Cancer risk in CHD was increased (hazard ratio [HR]) 2.63, 95% CI: 1.95, 3.55). Leukemia was the most common cancer in those without CHD (1,722/4,738, 36%), central nervous system tumors were second (1,073/4,738, 23%), and lymphoma third (410/4,738, 9%). Among children with CHD, lymphoma and leukemia occurred with the same frequency (12/43, 28% for each). HR for lymphoma was 8.37 (CI: 4.71, 14.86) with CHD versus without. HR for leukemia was 2.05 (CI: 1.16, 3.61) with CHD versus without. CHD complexity was higher in lymphoma (3, interquartile range [IQR]: 2–3) than those with leukemia (1, IQR, 1–2; p < .02). Conclusion: Cancer risk is increased in children with CHD. Lymphoma risk is increased in CHD and is correlated with more complex CHD. These results suggest a shared developmental origin for CHD and lymphoma may be present. [ABSTRACT FROM AUTHOR]

Unlabelled: Aortic dilation at the level of the aortic root can be caused by a variety of congenital or acquired conditions that lead to weakening of the aortic wall. In this retrospective study, we sought to determine the frequency of different associated diagnoses from children with aortic dilation seen at a single institution. A total of 377 children (68 % male) met study inclusion criteria. Patients were classified based on the suspected or confirmed associated diagnosis in one of the following categories: congenital heart disease (241/377, 64 %), chromosomal (34/377, 9 %), Marfan syndrome (26/377, 7 %), other genetic and non-genetic (22/377, 6 %), Loeys-Dietz syndrome (6/377, 2 %), and unknown (48/377, 13 %). Bicuspid aortic valve was by far the most prevalent congenital heart defect (206/241, 85 %), while Turner syndrome was the most frequent chromosomal abnormality (12/34, 35 %). Patients with Marfan syndrome were more likely to have severe dilation of the ascending aorta (p = 0.002) and to require aortic root replacement surgery (p < 0.001) compared to those in other diagnosis categories.Conclusion: The differential diagnosis of aortic dilation is broad and requires a careful assessment of cardiac anatomy. Evaluation by a clinical geneticist in this setting should be strongly considered given the high frequency of associated genetic conditions.What Is Known: • Aortic dilation is frequent in bicuspid aortic valve and other congenital heart defects. • Aortic dilation can be seen in several connective tissue disorders. Limited information is available in regard to the differential diagnosis of aortic dilation in children.What Is New: • In patients with aortic dilation concurrent congenital heart disease is frequently diagnosed. • Almost 18 % of cases in the present study had a defined presumptive or confirmed genetic diagnosis. We suggest considering a genetics evaluation in the setting of aortic dilation. [ABSTRACT FROM AUTHOR]

The purpose of this study was to assess the safety and indications for cardiac magnetic resonance (CMR) with myocardial perfusion imaging (MPI) in a cohort of children and young adults. A retrospective review of 178 children and young adults who underwent CMR with MPI was performed. Studies were categorized based on study protocols as MPI with resting perfusion only, adenosine stress MPI, exercise-induced stress MPI, and MPI for cardiac mass diagnosis. Relevant clinical history, exam indications, and adverse reactions following gadolinium-based contrast agent and adenosine administration were recorded. Studies were reviewed for the presence of myocardial perfusion defects, wall motion abnormalities, and delayed myocardial enhancement. The most common indications from MPI were congenital heart disease (CHD), Kawasaki disease, anomalous coronary artery, or myocardial mass characterization. Of these, 51% were protocoled with adenosine stress, 23% without stress, 6% with exercise stress, and 20% for cardiac mass evaluation. Excluding patients for myocardial mass evaluation, MPI defects were present in 16% (14 with adenosine stress, 1 with exercise stress, 8 on resting studies only). For cardiac mass evaluation, a mass was confirmed in 58%. No adverse reactions occurred with intravenous administration of a gadolinium-based contrast agent. Three self-limited adverse reactions, 2 patients with chest pain, and 1 patient with bradycardia, occurred following adenosine administration. MPI is a safe modality for the evaluation of pediatric and young adults with minimal adverse events. The most common indications for MPI were for the evaluation of CHD, Kawasaki disease, anomalous coronary artery, or myocardial mass characterization. [ABSTRACT FROM AUTHOR]

Peripheral pulmonary artery stenosis (PPAS) is a relatively rare form of congenital heart disease often associated with Williams syndrome, Alagille syndrome, and elastin arteriopathy. This disease is characterized by stenoses at nearly all lobar and segmental ostia and results in systemic-level right ventricular pressures. The current study summarizes our experience with the surgical treatment of PPAS. This was a retrospective review of 145 patients who underwent surgical repair of PPAS. This included 43 patients with Williams syndrome, 39 with Alagille syndrome, and 21 with elastin arteriopathy. Other diagnoses include tetralogy of Fallot with PPAS (n = 21), truncus arteriosus (n = 5), transposition (n = 3), double-outlet right ventricle (n = 2), arterial tortuosity syndrome (n = 3), and other (n = 8). The median preoperative right ventricle to aortic peak systolic pressure ratio was 1.01 (range, 0.50-1.60) which was reduced to 0.30 (range, 0.17-0.60) postoperatively. The median number of ostial repairs was 17 (range, 6-34) and median duration of cardiopulmonary bypass was 398 minutes (range, 92-844). There were 3 in-hospital deaths (2.1%). The median duration of follow-up was 26 months (range, 1-220) with 4 late deaths (2.9%). Eighty-two patients have subsequently undergone catheterization and 74 had a pressure ratio <0.50. The surgical treatment of PPAS resulted in a 70% reduction in right ventricular pressures. At 3 years, freedom from death was 94% and 90% of those evaluated maintained low pressures. These results suggest that the surgical treatment of PPAS is highly effective in most patients. [Display omitted] [Display omitted] [ABSTRACT FROM AUTHOR]

Myocardial dysfunction and heart failure are common in pediatric patients with congenital and acquired heart disease. Alkaline phosphatase (AP) has been suggested as a biomarker for myocardial dysfunction after Fontan operation. We hypothesized that pediatric patients with myocardial dysfunction requiring orthotopic heart transplant (OHT) have diminished AP compared to normal. A retrospective review was performed in all patients who underwent OHT at Arkansas Children's Hospital between January 2007 and October 2012. Anatomic diagnoses, therapeutic interventions, and ventricular ejection fraction (EF) were recorded. Z scores for AP levels in the study group were determined by comparing the observed AP levels to age- and gender-matched normative values. T tests were performed to compare the mean AP Z score prior to and after OHT. p values <0.05 were considered statistically significant. During the study period, 124 OHTs were performed. Complete study data were available and analyzed from 71/124 patients (mean age at OHT 3.9 years; 51% female). The mean AP Z score was significantly lower in the study group prior to OHT compared to normal ( p < 0.0001). The initiation of ACE inhibitor therapy prior to OHT was associated with a significant increase in AP and the ventricular EF ( p < 0.001 for both). Treatment with milrinone was associated with an increase in EF. AP is significantly lower in pediatric patients with myocardial dysfunction prior to OHT compared to normal. AP increases significantly after the initiation of therapies to improve myocardial function. Diminished AP is an indicator of myocardial dysfunction in pediatric patients. [ABSTRACT FROM AUTHOR]

This special edition of I Heart Failure Reviews i focuses on myocardial dysfunction, or "heart failure", in the setting of adult congenital heart disease (ACHD). The field of congenital heart disease care began with and remains a symbiotic relationship between pediatric cardiologists and congenital heart surgeons. While advanced practice providers are an integral part of what we do, it is clear that for the foreseeable future, if ACHD patients are to receive cardiological care, a lot of them will have to be cared for by non-ACHD-trained cardiologists. [Extracted from the article]

Arrhythmia in Adults with Single Ventricles Objectives The study sought to identify the impact of cardiac arrhythmias on hospitalizations in adults with single ventricle (SV) congenital heart disease (CHD). Background Surgical advances have dramatically improved survival in patients with CHD. Cardiac arrhythmias and sudden cardiac death are common in adults with CHD. Methods and Results Data from 43 pediatric hospitals in the 2004 to 2011 Pediatric Health Information System database were used to identify patients ≥18 years of age admitted with International Classification of Diseases-9th Revision codes for a diagnosis of either hypoplastic left heart syndrome (HLHS), tricuspid atresia (TA) or common ventricle (CV), and a cardiac arrhythmia. Primary and secondary diagnoses, length of stay (LOS), hospital charges, and interventional procedures were determined. Multilevel models were used to evaluate differences in demographics, diagnoses, and clinical outcomes among the 3 subgroups (HLHS, TA, and CV). Interactions of charges with arrhythmia and admission year were examined using ANOVA. There were 642 admissions in 424 patients with SV CHD and an arrhythmia diagnosis. A single arrhythmia diagnosis was present in 454 admissions (71%). Total hospital charges were $80.7 million with mean charge per admission of $127,296 ± 243,094. The mean charge per hospital day was $16,653 ± 17,516 and increased across the study period (P < 0.01). Arrhythmia distributions were impacted by SV anatomic subtype (P < 0.001). Hospital resource utilization was significantly different among arrhythmia groups (P < 0.001). Conclusions In adults with SV CHD, arrhythmias are affected by SV anatomic subtype and impact adversely upon hospital resource utilization. [ABSTRACT FROM AUTHOR]

Data investigating the impact of household income and other factors on SV patient status‐post‐Fontan palliation after heart transplantation are lacking. We aim to evaluate factors affecting outcomes after OHT in this population. The PHIS database was interrogated for either SV or myocarditis/primary CM who were 4 years or older who underwent a single OHT. There were 1599 patients with a median age of 13.2 years (IQR: 9.3‐16.1). Total hospital costs were significantly higher in the SV group ($408 000 vs $294 000, P < 0.0001), but as median household income increased, the risk of inhospital mortality, post‐transplant LOS, and LOS‐adjusted total hospital costs all decreased. The risk of inhospital mortality increased 6.5% per 1 year of age increase at the time of transplant. Patients in the SV group had significantly more diagnoses than those in the CM group (21 vs 15, P < 0.0001) and had longer total hospital LOSs as a result of longer post‐transplant courses (25 days vs 15, P < 0.0001). Increased median household income and younger age are associated with decreased resource utilization and improved inhospital mortality in SV CHD patients who undergo OHT. In conclusion, earlier consideration of OHT in this population, coupled with improved selection criteria, may increase survival in this population. [ABSTRACT FROM AUTHOR]

Introduction: We sought to describe characteristics of children admitted with pericardial effusion (PCE) and Systemic Lupus Erythematosus (SLE) and determine the association between PCE and outcomes of interest. Methods: We performed a retrospective cohort study of all patients 18 years of age or younger with an International Classification of Disease, Ninth Revision code for SLE from 2004 to 2015 in the Pediatric Health Information System (PHIS). Children with congenital heart disease or who had undergone heart surgery were excluded. Multivariable analysis evaluated the effect of PCE on mortality, length of stay (LOS), and readmission within 30 days. Results: There were 5679 admissions, in which 705 (12.4%) had PCE. Median age at admission was 15 years (interquartile range: 13-17). There were no differences in age or sex between patients with or without PCE. The percentage of black children was higher in the PCE group (43% vs. 31%, p < 0.001). The odds of a black patient having PCE were 1.7 higher than non-black patients (p < 0.001). In-hospital mortality was 2.5 times higher in children with PCE (p = 0.027). Those with PCE had 1.5 greater odds of readmission within 30 days (p < 0.001). PCE was not associated with increased LOS (0.99, p = 0.753). Conclusion: PCE is common in admissions of children with SLE. There are disproportionately more black patients with SLE affected by PCE. PCE is associated with significantly higher mortality and rates of readmission. [ABSTRACT FROM AUTHOR]

Introduction: Congenital heart disease (CHD) is the most common birth defect and leading cause of neonatal and infant death. CHD with single ventricle anatomy, such as double-inlet left ventricle, also known as common ventricle (CV), are the most complex with the highest mortality. In contrast to other forms of CHD, prenatal risk factors associated with CV have not been previously studied. Methods: We analyzed 200 CV cases and 11,063 nonmalformed controls in the National Birth Defects Prevention Study, a case-control, multicenter population-based study of birth defects among 32,200 cases from 9 United States. Mothers completed surveys by trained interviewers 6 weeks to 24 months after the estimated date of delivery (October 1997 to December 2011). Random forest analysis identified potential predictor variables for CV among >200 variables. These potential predictor variables were included in multivariable models to estimate effects. Odds ratios (OR) and 95% confidence intervals were estimated using logistic regression for CV among all cases, cases with isolated CV, and non-isolated cases, defined as CV with at least 1 additional major non-cardiac malformation. Results: Random forest analysis identified pre-pregnancy diabetes, any history of maternal insulin use, maternal total lipid intake, and paternal race as potential predictors of CV. Table 1 reports risk factors identified via logistic regression. Higher maternal fat intake was associated with reduced risk of CV. Conclusions: Maternal pre-pregnancy diabetes and history of insulin use were associated with increased risk of having an infant with CV while maternal lipid intake had an inverse association. These novel data provide multiple metabolic pathways for investigation to better identify the developmental etiologies of CHD and suggest that public health interventions targeting balanced metabolism in women of childbearing age could reduce CHD risk. [ABSTRACT FROM AUTHOR]