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.

Referring Provider Opinions of Pediatric Cardiology Evaluations Performed by Nurse Practitioners.

In the setting of physician shortages, nurse practitioner (NP) roles have evolved, with increasing independence across most healthcare settings. We sought to characterize referring clinician perceptions of NP-performed outpatient pediatric cardiology consultations. We electronically distributed to pediatric and family medicine physicians and NPs in Arkansas our 11-item survey assessing the acceptability of pediatric cardiology consultations being completed by an NP under varying circumstances. Circumstances included seven common referral indications, and the scale offered five answer choices ranging from "definitely unacceptable" to "definitely acceptable". A total of 292 of 1756 (16.6% response rate) referring clinicians responded to the survey. Overall, 57% of responses indicated that NP-completed pediatric cardiology evaluations were either definitely or probably unacceptable. Acceptability was varied by referral indication and referring clinician characteristics. Unacceptability of NP-completed pediatric cardiology evaluations was greatest among family medicine physicians (81%), pediatricians (66%), and clinicians working in solo or two-physician practices (77%) or community hospitals/clinics (71%). If NP evaluation of a murmur included required review with a cardiologist, the unacceptability rate dropped from 50 to 24% (p < 0.0001). Unacceptability was higher in physicians who do not work with NPs (69%) compared to those who do (60%) (pp < 0.0001). Many referring physicians were willing to send patients ≥ 100 miles to ensure evaluation by a pediatric cardiologist. Most referring physicians find pediatric cardiology evaluations performed by NPs to be unacceptable. Requisite review with a cardiologist improved acceptability of NP evaluations. Many referring physicians would send patients much farther to guarantee evaluation by a cardiologist.

Health Insurance Status and Access to Healthcare Among Young Adults with Congenital Heart Disease: from the Congenital Heart Survey To Recognize Outcomes, Needs and Well-beinG (CH STRONG), 2016-2019.

Having health insurance is associated with better access to healthcare and lower rates of comorbidity in the general population, but data are limited on insurance's impact on adults with congenital heart disease (ACHD). The Congenital Heart Survey To Recognize Outcomes, Needs and well-beinG (CH STRONG) was conducted among ACHD in three locations from 2016 to 2019. We performed multivariable logistic regression to determine the associations between health insurance and both access to healthcare and presence of comorbidities. We also compared health insurance and comorbidities among ACHD to similarly-aged individuals in the Behavioral Risk Factor Surveillance System (BRFSS) as a proxy for the general population. Of 1354 CH STRONG respondents, the majority were ≤ 30 years old (83.5%), and 8.8% were uninsured versus 17.7% in the BRFSS (p < 0.01). Compared to insured ACHD, uninsured were less likely to report regular medical care (adjusted odds ratio [aOR] 0.2, 95% confidence interval [CI] 0.1-0.3) and visited an emergency room more often (aOR 1.6, CI 1.0-2.3). Among all ACHD reporting disability, uninsured individuals less frequently received benefits (aOR 0.1, CI 0.0-0.3). Depression was common among uninsured ACHD (22.5%), but insured ACHD had lower rates of depression than insured in the BRFSS (13.3% vs. 22.5%, p < 0.01). In conclusion, rates of insurance were higher among ACHD compared to the general population. Nonetheless, uninsured ACHD inconsistently accessed healthcare and benefits. Further studies are needed to determine if insurance ameliorates the risk of morbidity as ACHD age.

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.

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.

Comparison of Postoperative, In-Hospital Outcomes After Complete Repair of Tetralogy of Fallot Between 22q11.2 Deletion Syndrome and Trisomy 21.

22q11.2 deletion syndrome (22q11) and trisomy 21 (T21) are frequently associated with tetralogy of Fallot (TOF). We hypothesized that there are differences in postoperative length of stay (LOS) and occurrence of postoperative interventions after complete repair of TOF when comparing children with 22q11 to those with T21. Using the Pediatric Health Information System, we performed a retrospective cohort study of patients who underwent complete repair of TOF from 2004 to 2019. Three groups were identified: 22q11, T21, and controls (those without a coded genetic syndrome). Outcomes were postoperative LOS and composite occurrence (yes/no) of at least one postoperative intervention. Bivariate and multivariate comparisons were made among groups; odds ratios (ORs) with 95% confidence intervals (CIs) were calculated using the control group as the comparator. There were 6924 subjects (n = 493 22q11, n = 455 T21, n = 5976 controls). In bivariate analysis, 22q11 was associated with a longer LOS compared to T21 (OR 2.37 [2.16, 2.60] vs. 1.25 [1.12, 1.39], p < 0.001), and 22q11 more often underwent postoperative intervention (OR 3.42 [CI 2.56, 4.57] vs. 1.38 [CI 0.91, 2.11]; p < 0.001). In multivariate analysis, 22q11 was also associated with longer LOS (adjusted OR 1.35 [1.26, 1.44] vs. 1.12 [1.04, 1.20]; p < 0.001), but there was no difference in the adjusted odds of postoperative intervention. Children with 22q11 are more likely to experience adverse outcomes after repair of TOF compared to those with T21; the differences are most pronounced for LOS.

Comorbidities Among Young Adults with Congenital Heart Defects: Results from the Congenital Heart Survey To Recognize Outcomes, Needs, and well-beinG - Arizona, Arkansas, and Metropolitan Atlanta, 2016-2019.

An estimated 1.4 million adults in the United States live with congenital heart defects (CHDs), yet their health outcomes are not well understood (1). Using self-reported, cross-sectional data from 1,482 respondents in the 2016-2019 Congenital Heart Survey To Recognize Outcomes, Needs, and well-beinG (CH STRONG) (2), CDC and academic partners estimated the prevalence of comorbidities among adults with CHDs aged 20-38 years born in Arizona (AZ), Arkansas (AR), and metropolitan Atlanta, Georgia (GA) compared with the general population (aged 20-38 years) from the National Health and Nutrition Examination Survey (NHANES) during 2015-2018 (3) and the AZ, AR, and GA Behavioral Risk Factor Surveillance Systems (BRFSS) during 2016-2018 (4). Adults with CHDs were more likely than those in the general population to report cardiovascular comorbidities, such as a history of congestive heart failure (4.3% versus 0.2%) and stroke (1.4% versus 0.3%), particularly those with severe CHDs (2). Adults with CHDs were more likely to report current depressive symptoms (15.1% versus 8.5%), but less likely to report previous diagnoses of depression (14.2% versus 22.6%), asthma (12.7% versus 16.9%), or rheumatologic disease (3.2% versus 8.0%). Prevalence of noncardiovascular comorbidities was similar between adults whose CHD was considered severe and those with nonsevere CHDs. Public health practitioners and clinicians can encourage young adults with CHDs to seek appropriate medical care to help them live as healthy a life as possible.

Impact of Perioperative Tachydysrhythmias on Mortality and Length of Stay in Complete Repair of Tetralogy of Fallot: A Multicenter Retrospective Cohort Study from the Pediatric Health Information System.

INTRODUCTION: Tachydysrhythmias (TDS) frequently occur after complete repair of tetralogy of Fallot (TOF). However, not much is known about the effect of TDS on morbidity and mortality after TOF repair. We sought to assess the associations between TDS and mortality and morbidity after repair of TOF using a multicentre database. MATERIALS AND METHODS: We identified all children aged 0-5 years in the Pediatric Health Information System who underwent TOF repair between 2004 and 2015. Codes for TDS were used to identify cases. Outcome variables were inpatient mortality and total length of stay (LOS). Univariate and multiple logistic and linear regression analyses were used to identify the effects of multiple risk factors, including TDS, on mortality and LOS. RESULTS: A total of 7,749 patients met inclusion criteria, of which 1,493 (19%) had codes for TDS. There was no association between TDS and inpatient mortality. However, TDS were associated with 1.1 days longer LOS and accounted for 2% of the variation observed in LOS. CONCLUSION: After complete repair of TOF, TDS were not associated with mortality and appeared to have only a modest effect on LOS.

A Fetal Presentation of a Ruptured Right Ventricular Diverticulum.

Ruptured diverticula and ventricular aneurysms are rare in the fetus, with a limited number of case reports published previously. Additional fetal complications secondary to these ventricular wall abnormalities can be seen. Interventional measures can be considered and attempted either in utero or postnatally to improve the chance of survival. We present a case of a ruptured diverticulum in a fetus and the clinical course.

Maternal dietary fat intake and the risk of congenital heart defects in offspring.

BACKGROUND: Fatty acids are crucial in embryologic development, including cardiogenesis. The impact of maternal periconceptional dietary fat intake on the risk of congenital heart defects (CHDs) has not been clearly elucidated. We hypothesized that maternal dietary fat intake during pregnancy is associated with risk of CHDs in offspring. METHODS: We analyzed CHD cases and nonmalformed controls from the National Birth Defects Prevention Study, a case-control, multicenter population-based study of birth defects. We used multivariable logistic regression to analyze the association between maternal periconceptional dietary fat intake and occurrence of CHDs. RESULTS: We examined 11,393 infants with CHDs (cases) and 11,029 infants without birth defects (controls). Multivariable analysis of maternal dietary fat intake adjusted for maternal energy intake demonstrated modest change in risk for 2 of the 25 CHDs analyzed; otherwise there was no association. Maternal dietary fat intake unadjusted for total energy was associated with increased risk for several CHDs. CONCLUSIONS: After adjusting for total energy intake, maternal periconceptional dietary fat intake has a modest association with risk of a few specific CHDs. If maternal dietary fat intake does impact CHD risk, the effect is minimal. IMPACT: In this large, case-control study, after adjusting for total caloric intake, maternal periconceptional dietary fat intake was not associated with increased odds of congenital heart defects. This study investigates the hypothesis that women's periconceptional fat intake alters the risk of congenital heart defects in offspring. Our results raise questions about the role maternal fat intake may play in cardiogenesis and risk of congenital heart defects. Additionally, they raise the question about whether maternal lipid metabolism, as opposed to fat intake, may influence cardiac development.

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.

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.

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.

Real-Time Transthoracic Vector Flow Imaging of the Heart in Pediatric Patients.

In children with congenital heart defects, Doppler ultrasound is the standard, bedside imaging modality. However, precise characterization of blood flow is challenging due to angle-dependent and one-dimensional velocity estimation. Contrast agent free Vector Flow Imaging is a new ultrasound technology that enables angle-independent visualization of the detailed flow field. Two piglets, one with normal cardiac anatomy and one with congenital heart disease comprised of valvular pulmonary stenosis, a dilated main pulmonary artery, and an incomplete atrioventricular canal defect, were imaged transthoracically and epicardially using a BK Ultrasound bk5000 with built-in vector flow imaging and a 5MHz linear probe. Subsequently, two children, one with normal cardiac anatomy and one with congenital heart disease comprised of aortic valve stenosis and coarctation of the aorta were imaged transthoracically. Transthoracic two-dimensional echocardiography and vector flow imaging were readily performed in both animals and were limited only by the geometry of the porcine thorax. In addition, transthoracic vector flow imaging was successfully performed in both children, and abnormal flow secondary to cardiac anomalies was visible. Adequate penetration was obtained to a depth of 6.5 cm. Our group has previously demonstrated for the first time that transthoracic vector flow imaging echocardiography is feasible and practicable in pediatric-sized patients, and this paper describes examples of these concepts and in-depth comparisons with traditional imaging modalities. This paper demonstrates that commercially available vector flow imaging technology can be utilized in pediatric cardiac applications as a bedside transthoracic imaging modality, providing advanced detail of blood flow patterns within the cardiac chambers, across valves, and in the great arteries.

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.

Feasibility of Transthoracic Echocardiography Evaluation of Pulmonary Arteries Following Arterial Switch Operation.

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.

Fetal magnetocardiography using optically pumped magnetometers: a more adaptable and less expensive alternative?

Fetal magnetocardiography provides the requisite precision for diagnostic measurement of electrophysiological events in the fetal heart. Despite its significant benefits, this technique with current cryogenic based sensors has been limited to few centers, due to high cost of maintenance. In this study, we show that a less expensive non-cryogenic alternative, optically pumped magnetometers, can provide similar electrophysiological and quantitative characteristics when subjected to direct comparison with the current technology. Further research can potentially increase its clinical use for fetal magnetocardiography. © 2016 John Wiley & Sons, Ltd.