Left Ventricular Dysfunction Following Repair of Ventricular Septal Defects in Infants.

Left ventricular systolic dysfunction (LVSD) is frequently observed following repair of ventricular septal defects (VSD), although little is known about its incidence, time course, or risk factors. Among infants undergoing VSD repair, for postoperative LVSD, we sought to determine (1) incidence, (2) predictors, and (3) time to resolution. We queried our institution's surgical database for infants who underwent repair of isolated VSDs from November 2001 through January 2019. The primary outcome was postoperative LVSD, which was defined as a shortening fraction (SF) of <26% by M-mode. Postoperative echocardiograms were reviewed, and measurements were made using standard methods. Receiver operating characteristic analysis was generated to determine the preoperative left ventricular internal dimension (LVIDd) z-score most predictive of LVSD. Multivariable analysis was conducted to determine associations with LVSD; covariates in the model were weight percentile, genetic syndrome, preoperative diuretic, VSD type, and preoperative LVIDd z-score. Of the 164 patients who met inclusion criteria, 62 (38%) had postoperative LVSD. Fifty-eight (94%) of patients had resolution of LVSD within 9 months of surgery. Preoperative LVIDd z-score of >3.1 was associated with both an increased incidence of postoperative LVSD and prolonged time to resolution. Multivariable logistic regression analysis showed only preoperative LVIDd z-score was independently associated with postoperative LVSD. LVSD following VSD closure is common, but nearly all cases resolve by 9 months postoperatively. Elevated LVIDd prior to surgery is associated with postoperative LVSD. These data suggest VSD closure should be considered prior to the development of significant left ventricular dilation.

Infantile Hemangioma Treated with Propranolol Readmission Trends, Complications of Therapy, and Cost: A PHIS Database Study.

Objective: To examine admission trends, complications, and costs for inpatient infantile hemangioma (IH) associated with propranolol therapy utilizing the Pediatric Health Information System (PHIS) database. Study Design. A retrospective cohort study was completed using the PHIS database. The PHIS database was queried from 2008 to 2020 for children without cardiac disease and between the ages of three weeks and one year who were admitted with a diagnosis of IH and administered propranolol. Admissions were trended annually and by geographic region. Primary outcomes were length of stay (LOS), readmission, mortality, propranolol-related complications, and costs. Bivariate and multivariable analyses were employed to identify predictors of the primary outcomes. Results: A total of 2290 unique patient encounters were identified. Admissions steadily decreased after 2011, with variations by geographic region. There was no mortality and only 60 (2.6%) propranolol-related complications. African-American race (odds ratio (OR) 1.20 [95% CI: 1.02-1.41]), respiratory comorbidities (OR 2.04 [95% CI: 1.42-2.93]), neurologic conditions (OR 1.34 [95% CI: 1.09-1.59]), admission to an intensive care unit (OR 1.31 [95% CI: 1.09-1.59]), bronchospasm (OR 1.37 [95% CI: 1.22-1.55]), and hyperkalemia (OR 1.86 [95% CI: 1.08-3.20]) were associated with increased LOS. Neurologic conditions (OR 2.87 [95% CI: 1.76-4.67]) and respiratory comorbidities (OR 2.48 [CI: 1.43-4.30]) were associated with readmission. Average cost per admission was $5,158 ($3,259 to $8,560 range). Conclusion: There is an overall national decline in rate of admissions for IH propranolol therapy. Inpatient admission may be beneficial for patients with neurologic or respiratory conditions.

Left heart hypoplasia in the fetus: Echocardiographic predictors of outcome.

INTRODUCTION: Fetal left heart hypoplasia (LHH) with an apex-forming left ventricle may require neonatal intervention but it is difficult to predict. METHODS: We performed a retrospective study of fetuses with LHH defined as normal segmental anatomy, apex-/near-apex forming left ventricle, and ≥1 left-sided z-score ≤ -2 between 1997 and 2014. Fetuses with mitral or aortic atresia, critical aortic stenosis, extracardiac anomalies, or fetal intervention were excluded. Classification and regression tree analyses (CART) were performed to construct algorithms to predict postnatal circulation: no surgery versus biventricular surgery versus single ventricle (SV) palliation. RESULTS: Among 138 included fetuses, 52 (37%) underwent neonatal surgery, with 10 (7%) undergoing SV palliation. The strongest single outcome discriminator was exclusively left-to-right flow foramen ovale (FO) flow ≥32 weeks gestational age (GA) (seen in 0% with no surgery, 22% with biventricular surgery, 88% with SV palliation). On CART analysis >32 weeks GA, fetuses with right-to-left FO flow and aortopulmonary ratio >0.76 had 0% probability of neonatal surgery, while those with left-to-right FO flow and mitral valve z-score < -3.6 had a 70% probability of SV palliation. CONCLUSION: SV palliation is an uncommon outcome of fetal LHH. Fetal FO flow and other echocardiographic measures can help determine risk and type of postnatal intervention.

Maternal Smoking and Congenital Heart Defects, National Birth Defects Prevention Study, 1997-2011.

OBJECTIVES: To assess associations between maternal smoking and congenital heart defects (CHDs) in offspring. STUDY DESIGN: We performed a retrospective case-control study using data for cases of CHD (n = 8339) and nonmalformed controls (n = 11 020) from all years (1997-2011) of the National Birth Defects Prevention Study. Maternal self-reported smoking 1 month before through 3 months after conception was evaluated as a binary (none, any) and categorical (light, medium, heavy) exposure. Multivariable logistic regression was used to estimate aOR and 95% CIs. Stratified analyses were performed for septal defects according to maternal age, prepregnancy body mass index, and maternal race/ethnicity. RESULTS: Multiple CHDs displayed modest associations with any level of maternal periconceptional smoking independent of potential confounders; the strongest associations were for aggregated septal defects (OR, 1.5; 95% CI, 1.3-1.7), tricuspid atresia (OR, 1.7; 95% CI, 1.0-2.7), and double outlet right ventricle (DORV) (OR, 1.5; 95% CI, 1.1-2.1). Tricuspid atresia and DORV also displayed dose-response relationships. Among heavy smokers, the highest odds were again observed for tricuspid atresia (aOR 3.0; 95% CI, 1.5-6.1) and DORV (aOR 1.5; 95% CI, 1.1-2.2). Heavy smokers ≥35 years old more frequently had a child with a septal defect when compared with similarly aged nonsmokers (aOR 2.3; 95% CI, 1.4-3.9). CONCLUSIONS: Maternal periconceptional smoking is most strongly associated with septal defects, tricuspid atresia, and DORV; the risk for septal defects is modified by maternal age.

Implementation of a statewide, multisite fetal tele-echocardiography program: evaluation of more than 1100 fetuses over 9 years.

OBJECTIVES: We sought to describe the implementation and effectiveness of a statewide fetal tele-echocardiography program serving a resource-limited population. STUDY DESIGN: In 2009, our heart center established six satellite clinics for fetal tele-echocardiography around the state. We retrospectively reviewed all fetal tele-echocardiograms performed through 2018. Yearly statewide prenatal detection rates of operable congenital heart disease were queried from the Society of Thoracic Surgeons database. RESULT: In 1164 fetuses, fetal tele-echocardiography identified all types of congenital heart disease, with a sensitivity of 74% and specificity of 97%. For the detection of ductal-dependent congenital heart disease, fetal tele-echocardiography was 100% sensitive and specific. Between 2009 and 2018, annual statewide prenatal detection rates of congenital heart disease requiring heart surgery in the first 6 months of life rose by 159% (17-44%; R2 = 0.88, p < 0.01). CONCLUSIONS: The present study provides a framework for an effective, large-scale fetal tele-echocardiography program.

Gastrostomy and Tracheostomy After Complete Repair of Tetralogy of Fallot in Children With 22q11.2 Deletion Syndrome.

OBJECTIVES: Caring for a child with gastrostomy and/or tracheostomy can cause measurable parental stress. It is generally known that children with 22q11.2 deletion syndrome are at greater risk of requiring gastrostomy or tracheostomy after heart surgery, although the magnitude of that risk after complete repair of tetralogy of Fallot has not been described. We sought to determine the degree to which 22q11.2 deletion is associated with postoperative gastrostomy and/or tracheostomy after repair of tetralogy of Fallot. DESIGN: Retrospective cohort study. SETTING: Pediatric Health Information System. PATIENTS: Children undergoing complete repair of tetralogy of Fallot (ventricular septal defect closure and relief of right ventricular outflow tract obstruction) from 2003 to 2016. Patients were excluded if they had pulmonary atresia, other congenital heart defects, and/or genetic diagnoses other than 22q11.2 deletion. MEASUREMENTS AND MAIN RESULTS: Two groups were formed on the basis of 22q11.2 deletion status. Outcomes were postoperative tracheostomy and postoperative gastrostomy. Bivariate analysis and Kaplan-Meier analysis at 150 days postoperatively were performed. There were 4,800 patients, of which 317 (7%) had a code for 22q11.2 deletion. There were no significant differences between groups for age at surgery or sex. Patients with 22q11.2 deletion had significantly higher rates of gastrostomy (18% vs 5%; p < 0.001) and higher rates of tracheostomy (7% vs 1%; p < 0.001); there was no difference for mortality. Kaplan-Meier analyses also showed higher rates of gastrostomy (p = 0.024) and tracheostomy (p = 0.037). CONCLUSIONS: The present study establishes rates of postoperative gastrostomy and tracheostomy in children with 22q11.2 deletion after complete repair of tetralogy of Fallot. These data are useful to clinicians for providing families with preoperative counseling.

Effect of Trisomy 21 on Postoperative Length of Stay and Non-cardiac Surgery After Complete Repair of Tetralogy of Fallot.

Trisomy 21 (T21) is the most common chromosomal abnormality, and is frequently associated with congenital heart disease. Results of previous studies evaluating the effect of T21 on postoperative outcomes and complications following heart surgery have been mixed. Our goal was to determine if T21 is associated with higher frequency of adverse postoperative outcomes following repair of tetralogy of Fallot (TOF). A query of the Pediatric Health Information System was performed for patients who underwent complete repair of TOF from 2004 to 2015. Patients with a genetic syndrome other than T21 and tracheostomy and/or gastrostomy prior to heart surgery were excluded. Two groups were created on the basis of whether patients received a diagnostic code for T21. The adverse outcomes of interest were postoperative mortality, postoperative length of stay (LOS), postoperative gastrostomy, and postoperative tracheostomy. Univariate and Kaplan-Meier analysis were performed to evaluate outcomes. There were a total of 4790 patients; 430 (9%) patients had T21, and 4360 (91%) patients without a genetic diagnosis. There was no significant difference in mortality before discharge between those with and without T21 (2.3% vs 1.4%; p = 0.155). Patients with T21 had longer postoperative LOS (mean of 19.8 days vs 12.4 days; p < 0.001), and higher rates of postoperative gastrostomy (13.3% vs 5.3%; p < 0.02). There was no significant difference between groups for rates of postoperative tracheostomy (1.9% vs 1.2%; p = 0.276). Kaplan-Meier analysis confirmed that patients with T21 had longer postoperative LOS and greater incidence of gastrostomy.

Magnetocardiographic identification of prolonged fetal corrected QT interval in women receiving treatment for opioid use disorder.

AIM: Pregnant women undergoing treatment for opioid use disorder (OUD) may be exposed to multiple QT prolonging agents. We used magnetocardiography to measure fetal QT intervals in mothers with OUD on buprenorphine therapy. METHODS: Fetal and maternal magnetocardiography was performed in pregnant women receiving buprenorphine-assisted treatment (Disorder group); these were matched by gestational age to pregnant women who were opiate naïve (Reference group). Corrected QT intervals were determined using Bazett's formula and compared between groups. RESULTS: A total of eight women in the Disorder group matched to eight in the Reference group. Seven of the mothers (88%) in the Disorder group were smokers; there were no smokers in the Reference group. The average fetal corrected QT was significantly longer (P = 0.022) in the Disorder group than that in the Reference group (505 milliseconds [ms] ± 68.6 [standard deviation] vs 383 ms ± 70.3 [standard deviation]). CONCLUSION: Novel data from this small sample demonstrate prolongation of fetal corrected QT in women with OUD participating in buprenorphine assisted therapy. Additional investigation from a larger sample is needed to clarify if fetal buprenorphine and/or tobacco exposure is associated with changes in fetal QT which would warrant further prenatal and postnatal testing.

Characteristics of Non-postoperative Pediatric Pericardial Effusion: A Multicenter Retrospective Cohort Study from the Pediatric Health Information System (PHIS).

Little is known about the causes and risks of non-postoperative pericardial effusion (PCE) in pediatric patients. We sought to assess the diagnoses most frequently associated with admissions for PCE, and to determine if certain conditions were associated with higher in-hospital mortality and rates of readmission. Nationally distributed data from 44 pediatric hospitals in the 2004-2015 Pediatric Health Information System database were used to identify patients with hospital admissions for International Classification of Disease, Ninth Revision (ICD-9) codes for PCE and/or cardiac tamponade. Children with congenital heart disease were excluded. ICD-9 codes for conditions associated with PCE were grouped into eight categories: neoplastic, renal, autoimmune/inflammatory, pneumonia, viral, bacterial, hypothyroidism, and idiopathic. Multivariable models were used to evaluate odds of in-hospital mortality and readmission within 30 and 90 days. There were 9902 patients who met inclusion criteria. Total in-hospital mortality was 8.2% (n = 813); of those without a neoplastic diagnosis, mortality was 6.5% (n = 493/7543). Idiopathic PCE accounted for the most admissions (36%), followed by neoplasms (24%), pneumonia (20%), and autoimmune/inflammatory disease (19%). In multivariable models, odds of death were highest for neoplasms (adjusted odds ratio 3.83, p < 0.001) and renal disease (adjusted odds ratio 2.86, p < 0.001). Children with a neoplasm, renal disease, and those undergoing pericardiocentesis had the highest rates of readmission at 30 and 90 days. Children admitted with non-postoperative PCE have multiple associated conditions. Neoplasm and renal disease in the setting of PCE are associated with the highest odds of in-hospital mortality among concomitant conditions; children with a neoplasm, renal disease, and those undergoing pericardiocentesis have the highest odds of readmission.

Frequency of Development of Aortic Valve Disease in Unrepaired Perimembranous Ventricular Septal Defects.

We sought to determine the natural history of aortic valve disease in patients with unrepaired perimembranous ventricular septal defects (pVSDs) and to identify echocardiographic parameters predictive of increased risk of surgical repair of pVSD because of aortic valve disease. We retrospectively analyzed all echocardiograms of patients with a diagnosis of pVSD at our institution from January 1999 to January 2015. All available echocardiographic data were collected. Patients were excluded if there was another structural cardiac anomaly other than bicuspid aortic valve, small patent foramen ovale, or ductus arteriosus. The prevalences of aortic valve prolapse and regurgitation, as well as aortic valve disease progression, were determined. A total of 2,114 echocardiograms from 657 patients with unrepaired pVSD were reviewed. Median age at the time of echocardiogram was 1.9 years (interquartile range [IQR] 0.2 to 5.4). Median duration of follow-up was 1.7 years (IQR 0.2 to 7.4). pVSD-associated aortic valve disease prompted surgical intervention in 1.5% (10 of 657) of patients. Median age at the time of surgery was 4.8 years (IQR 1.7 to 8.4). A pVSD-to-aortic annulus diameter ratio of 0.66 ± 0.05 was present in 90% (9 of 10) of patients who underwent surgical closure because of pVSD-associated aortic valve disease. In conclusion, pVSD-associated aortic valve disease is uncommon, and progression of aortic regurgitation is rare. These data suggest that the majority of patients with pVSD do not require frequent follow-up and that frequent follow-up can be saved for a subset with echocardiographic markers placing them at higher risk of aortic valve diseases.

Tracheostomy Among Infants With Hypoplastic Left Heart Syndrome Undergoing Cardiac Operations: A Multicenter Analysis.

BACKGROUND: Less than 2.7% of infants undergoing congenital heart disease operations have difficulty weaning from invasive mechanical ventilation. In such instances, clinicians may choose to perform tracheostomy. Limited literature has examined tracheostomy placement specifically in infants with hypoplastic left heart syndrome (HLHS). This study evaluated the risk factors for tracheostomy placement in infants with HLHS and examined the outcomes of these infants before their first hospital discharge. METHODS: This retrospective analysis of the Pediatric Heath Information System data set included infants with HLHS who underwent stage 1 Norwood operation, a hybrid procedure, or heart transplant from 2004 through 2013. RESULTS: We identified 5721 infants with HLHS, and 126 underwent tracheostomy placement. Infants in the tracheostomy group had more morbidities and a higher mortality rate across the study period. Diagnosis of chromosomal abnormalities, anomalies of the trachea and esophagus, larynx, diaphragm and nervous system, bilateral vocal cord paralysis, and necrotizing enterocolitis, and procedures including extracorporeal membrane oxygenation support, cardiac catheterization, and gastrostomy tube were independently associated with tracheostomy placement in the study population. Despite an overall increase in rates of tracheostomy performed in infants with HLHS during the study period, the mortality rate did not improve among tracheostomy patients. CONCLUSIONS: Several risk factors were identified in infants with HLHS in whom a tracheostomy was placed during their first hospitalization. Despite an overall increase in rates of tracheostomies during the study period, the mortality rate did not improve among these patients. Appropriate family counseling and thorough preoperative case selection is suggested when discussing possible tracheostomy placement in infants with HLHS.

Older Age at Completion of Fontan Procedure Is Associated with Improved Percentage of Predicted Maximum Oxygen Uptake.

We tested the hypothesis that later completion of the Fontan procedure is associated with improved exercise capacity in the current period of staged single-ventricle palliation. We performed a retrospective study, in Fontan patients, of exercise stress test data from April 2003 through March 2011. Patients were included if they had received staged palliations in accordance with current surgical strategy, defined as the performance of a superior cavopulmonary connection at ≤1 year of age, followed in subsequent years by Fontan completion. Patients with a pacemaker or respiratory exchange ratio <1 were excluded. Early and late Fontan groups were created on the basis of whether Fontan completion had been performed at <4 or ≥ 4 years of age. The primary predictor variable was age at Fontan completion, and the primary marker of exercise performance was the percentage of predicted maximum oxygen consumption. During the study period, 55 patients were identified (mean age, 11.7 ± 2.8 yr). Older age at Fontan completion correlated positively with higher percentages of predicted maximum oxygen consumption (R=0.286, P=0.034). Patients in whom Fontan completion was performed at ≥4 years of age had higher percentages of predicted maximum oxygen consumption than did those in whom completion was at <4 years of age (84.4 ± 21.5 vs 72.9 ± 18.1; P=0.041). Later Fontan completion might be associated with improved exercise capacity in patients palliated in accordance with contemporary surgical strategy.