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Objective: The objective of this study was to assess the relationship of prenatal diagnosis of critical congenital heart disease (CHD) to preoperative and postoperative patient findings. Method: Retrospective analysis of neonates with critical CHD who underwent cardiothoracic surgery at one of four centers in North Carolina between 2008-2013. Surgical data collected by sites for submission to the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) and the North Carolina CHD Lifespan Database were queried. Results: There were 715 patients with STS records; 566 linked to the NC CHD database. Patients with prenatal diagnosis had a lower incidence of preoperative risk factors, including need for mechanical ventilation and presence of shock. However, prenatally diagnosed patients had worse short-term outcomes, including higher operative mortality, higher incidence of select postoperative complications, and longer LOS. There was no difference in one-year mortality. Conclusion: Our findings are consistent with current literature which suggests that prenatal diagnosis of critical CHD is associated with a more optimized preoperative clinical status. However, we found that patients with prenatal diagnoses had less favorable postoperative outcomes. This needs to be investigated further, but may be secondary to patient-specific factors, such as CHD disease severity.

BACKGROUND We aimed to assess safety, tolerability, and improvement in weight gain with an energy- and protein-enriched formula (EPEF) in infants with poor growth. METHODS Infants aged 1-8 months with poor growth were enrolled to receive EPEF for 16 weeks. PRIMARY OBJECTIVE improvement in weight as measured by change in weight-for-age z-score (WAZ) and weight gain velocity (g/day) ≥ median weight gain velocity for age based on WHO growth standards. Secondary objectives: improvements in other anthropometric z-scores, formula tolerance, and safety. RESULTS 26 subjects with poor growth due to congenital heart disease (15/26), other organic causes (9/26), and non-organic causes (2/26) completed the study per protocol. Mean calorie intake was 123 ± 32 kcals/kg body weight per day with >90% of calories coming from EPEF. Weight gain velocity exceeded the WHO median for 83% (20/24) and 67% (16/24) of infants at ≥1 time point and for the overall study period, respectively. Mean ± SD WAZ improved from -2.92 ± 1.04 at baseline to -2.01 ± 1.12 at 16 weeks (p = 0.0001). Z-scores for weight-for-length and head circumference (p = 0.0001) and for length-for-age (p = 0.003) were significantly improved at 16 weeks. There were no significant differences from baseline in vomiting, fussiness, or number of stools per day. Compared to baseline, stool consistency was different at 2,4, and 16 weeks (p < 0.05). There was a decrease or no change in spit-up, flatulence, crying or gassiness. CONCLUSION EPEF is safe, well tolerated, and improves weight gain in infants with poor growth. This article is protected by copyright. All rights reserved.

Background The AHA/ACC Adult Congenital Heart Disease guidelines recommend that most adults with congenital heart disease (CHD) follow-up with CHD cardiologists every 1 to 2 years because longer gaps in care are associated with adverse outcomes. This study aimed to determine the proportion of patients in North Carolina who did not have recommended follow-up and to explore predictors of loss to follow-up. Methods Patients ages ≥18 years with a healthcare encounter from 2008 to 2013 in a statewide North Carolina database with an ICD-9 code for CHD were assessed. The proportion with cardiology follow-up within 24 months following index encounter was assessed with Kaplan-Meier estimates. Cox regression was utilized to identify demographic factors associated with differences in follow-up. Results 2822 patients were identified. Median age was 35 years; 55% were female. 70% were white, 22% black, and 3% Hispanic; 36% had severe CHD. The proportion with 2-year cardiology follow-up was 61%. Those with severe CHD were more likely to have timely follow-up than those with less severe CHD (72% vs 55%, P Conclusion 39% of adults with CHD in North Carolina are not meeting AHA/ACC recommendations for follow-up. Younger and minority patients and those with non-severe CHD were particularly vulnerable to inadequate follow-up; targeted efforts to retain these patients in care may be helpful.

With improved surgical outcomes, infants and children with congenital heart disease (CHD) may die from other causes of death (COD) other than CHD. We sought to describe the COD in youth with CHD in North Carolina (NC). Patients from birth to 20 years of age with a healthcare encounter between 2008 and 2013 in NC were identified by ICD-9 code. Patients who could be linked to a NC death certificate between 2008 and 2016 were included. Patients were divided by CHD subtypes (severe, shunt, valve, other). COD was compared between groups. Records of 35,542 patients

Background: Children with congenital heart defects (CHDs) are at risk for poor academic performance. The degree to which receipt of health care services is associated with adverse academic outcomes is not known. We examined the association between episodes of cardiac care and third-grade performance in children with CHD. Methods: We identified subjects between 1/1/2008 and 4/30/2012 among 5 centers in North Carolina. We classified children by CHD type and linked subjects to the state educational records. Any inpatient or outpatient cardiac encounter on a date of service was considered an encounter. We calculated the number of encounters by adding the number of inpatient or outpatient cardiac visits prior to the date of the end of grade (EOG) tests. We estimated the odds of failing third-grade reading or math EOG tests by episodes of care stratified at the 50thpercentile, controlling for CHD type, maternal education, sex, race/ethnicity, birth weight and gestational age. Results: A total of 184 children had third-grade EOG scores linked to health care records. The median number of episodes of care was 4 (range: 1-60). Those with visits ˃50th percentile (>4 encounters over 4.3 years) had 2.09 (95% CI: 1.04, 4.21) greater odds of failing the math EOG compared to those ≤50th percentile (1-4 encounters). The third-grade math score declined by 1.5 points (pConclusion: Children with CHD with >4 episodes of cardiac care over 4.3 years may be at risk for delays in third-grade academic performance. Strategies to minimize school absenteeism may improve academic success in this population.

Anomalous coronary arteries from the pulmonary artery are uncommon causes of heart failure in the adult population. This case demonstrates the unusual presentation in a patient with anomalous right coronary artery from the pulmonary artery and discusses the complex pathophysiology of this lesion and the role of guideline-directed medical therapy in the management of these patients.

Background Loeys-Dietz syndrome (LDS) is a connective tissue disorder that commonly presents with vascular abnormalities. Owing to the rarity and severity of the condition, consensus guidelines for aortic surgery thresholds vary. In addition, evaluation of coronary arteries in patients with LDS (either routinely or before aortic root surgery) remain undefined. In this case report, we discuss a patient with LDS who found to have an ectatic aortic root and a coronary artery aneurysm and discuss guidelines for evaluation and management in this patient population. Case summary A 48-year-old woman was incidentally found to have a 45 mm ectatic aortic root during evaluation for a neck mass. As part of pre-operative evaluation for aortic root replacement, left heart catheterization revealed a left main coronary artery aneurysm. Family history revealed aortic aneurysms, sudden cardiac death, and tall height. Physical examination was notable for pectus excavatum and elongated limbs. Workup for inflammatory aetiologies of aortic root dilation was negative. Genetic testing revealed a heterozygous pathogenic TGBF3 variant, consistent with LDS Type 5. She subsequently underwent two-vessel coronary artery bypass, excision of her left main coronary artery aneurysm, and ascending aortic replacement. Discussion In this case, we describe a patient with LDS who was noted to have a coronary artery aneurysm, a rare finding in the initial presentation of disease. In addition, we examine guidelines regarding evaluation of management of aortic root disease and coronary aneurysms.

The diagnosis of vascular rings is challenging and may be delayed as symptoms overlap with more common conditions associated with childhood. Underlying genetic associations of this condition remain largely undiscovered. In this report, we present a patient with a double aortic arch and highlight the importance of diagnostic imaging. We also engage in a review of the important genetic considerations.

With improved surgical outcomes, infants and children with congenital heart disease (CHD) may die from other causes of death (COD) other than CHD. We sought to describe the COD in youth with CHD in North Carolina (NC). Patients from birth to 20 years of age with a healthcare encounter between 2008 and 2013 in NC were identified by ICD-9 code. Patients who could be linked to a NC death certificate between 2008 and 2016 were included. Patients were divided by CHD subtypes (severe, shunt, valve, other). COD was compared between groups. Records of 35,542 patients 20 years old were evaluated. There were 15,277 infants with an annual mortality rate of 3.5 deaths per 100 live births. The most frequent COD in infants (age 1 year) were CHD (31.7%), lung disease (16.1%), and infection (11.4%). In 20,265 children (age 1 to 20 years), there was annual mortality rate of 9.7 deaths per 1000 at risk. The most frequent COD in children were CHD (34.2%), neurologic disease (10.2%), and infection (9.5%). In the severe subtype, CHD was the most common COD. In infants with shunt-type CHD disease, lung disease (19.5%) was the most common COD. The mortality rate in infants was three times higher when compared to children. CHD is the most common underlying COD, but in those with shunt-type lesions, extra-cardiac COD is more common. A multidisciplinary approach in CHD patients, where development of best practice models regarding comorbid conditions such as lung disease and neurologic disease could improve outcomes in this patient population.

Background Myocardial injury is associated with excess mortality in severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections, and the mechanisms of injury are diverse. Coagulopathy associated with this infection may have unique cardiovascular implications. Case summary We present a case of 62-year-old male who presented after experiencing syncope and cardiac arrest. Given the clinical presentation and electrocardiographic findings, there was concern for acute coronary syndrome. However, coronary angiogram did not reveal significant coronary obstruction. Due to the unclear nature of his presentation, a bedside echocardiogram was rapidly performed and was indicative of right ventricular strain. Due to these findings, a pulmonary angiogram was performed that revealed massive pulmonary embolism. He successfully underwent catheter-directed thrombolysis and, after a prolonged hospital stay, was discharged home on lifelong anticoagulation. Discussion The impact of coronavirus disease-2019 (COVID-19) on the cardiovascular system has been prominent and multifaceted. COVID-19 can have wide-ranging effects on the cardiovascular system due to coagulopathy with resultant venous and arterial thrombo-embolism. Due to the critical condition of many patients affected by COVID-19, imaging for thrombo-embolic events is often delayed. With the use of bedside echocardiogram, observation of right ventricular strain may be critical in raising suspicion for pulmonary embolism, especially when atypical features are noted on electrocardiogram.

Background:Adults with CHD have reduced work participation rates compared to adults without CHD. We aimed to quantify employment rate among adult CHD patients in a population-based registry and to describe factors and barriers associated with work participation.Methods:We retrospectively identified adults with employment information in the North Carolina Congenital Heart Defects Surveillance Network. Employment was defined as any paid work in a given year. Logistic regression was used to examine patients’ employment status during each year.Results:The registry included 1,208 adult CHD patients with a health care encounter between 2009 and 2013, of whom 1,078 had ≥1 year of data with known employment status. Overall, 401 patients (37%) were employed in their most recent registry year. On multivariable analysis, the odds of employment decreased with older age and were lower for Black as compared to White patients (odds ratio = 0.78; 95% confidence interval: 0.62, 0.98; p = 0.030), and single as compared to married patients (odds ratio = 0.50; 95% confidence interval: 0.39, 0.63; p < 0.001).Conclusion:In a registry where employment status was routinely captured, only 37% of adult CHD patients aged 18–64 years were employed, with older patients, Black patients, and single patients being less likely to be employed. Further work is needed to consider how enhancing cardiology follow-up for adults with CHD can integrate support for employment.

Background— Prospective data on the medium-term safety and effectiveness of the AMPLATZER Septal Occluder in clinical practice are not available. The objective of this study was to prospectively evaluate the risk of hemodynamic compromise and obtain medium-term survival data on patients implanted with the AMPLATZER Septal Occluder for percutaneous closure of secundum atrial septal defects. Methods and Results— Subjects were enrolled prospectively at 50 US sites and followed for 2 years. Between 2008 and 2012, atrial septal defect closure with the AMPLATZER Septal Occluder was attempted in 1000 patients (aged 0.3–83.6 years, mean 21±22 years). Procedural closure occurred in 97.9%, with 1-month and 2-year closure 98.5% and 97.9%, respectively. Hemodynamic compromise occurred in 6 subjects (0.65%), because of dysrhythmia in 2, device embolization in 1, and cardiac erosion in 3. The rate of cardiac erosion was 0.3% (average 83, range 12–171 days from implant). Conclusions— Closure of atrial septal defect with the AMPLATZER Septal Occluder is safe and effective. The rate of hemodynamic compromise and cardiac erosion is rare. The risk factors for cardiac erosion after device closure are not yet clear. Clinical Trial Registration— URL: http://www.clinicaltrials.gov . Unique identifier: NCT00650936.

BackgroundChildren born with heterotaxy syndromes have poorer outcomes compared with children born with comparable cardiac lesions requiring similar surgical palliation. Heterotaxy has been reported as a separate risk factor for mortality and increased morbidity in a series of Fontan operations reported from single centres. Little is known, however, about the functional state of surviving patients with heterotaxy following a Fontan operation.MethodsIn the multicentric cross-sectional study carried out by the Pediatric Heart Network of 546 survivors of the Fontan procedure, the patients, aged from 6 to 18 years, underwent evaluation by echocardiography, exercise testing, electrocardiography, magnetic resonance imaging, and functional health status questionnaires compiled by the patients and their parents. Heterotaxy was identified in 42 patients (8%). Medical and patient characteristics were compared between those with heterotaxy and the remaining 504 patients who did not have heterotaxy.ResultsPatients with heterotaxy had their Fontan procedure performed at a later age, with a median of 3.9 years versus 2.8 years (p = 0.001) and had volume-unloading surgery performed later, at a median age of 1.4 versus 0.9 years (p = 0.008). These patients had significantly different ventricular and atrioventricular valvar morphology, as well as a higher incidence of systemic and pulmonary venous abnormalities. They had a higher incidence of prior surgery to the pulmonary veins, at 21 versus 0.4%. The type of Fontan procedure was different, but no difference was detected in length of stay in hospital, or the number of postoperative complications. Sinus rhythm was less common, at 44 versus 71%, (p = 0.002), and history of atrial arrhythmias more common, at 19 versus 8%, (p = 0.018) in those with heterotaxy. Echocardiography revealed a greater degree atrioventricular valvar regurgitation, lower indexed stroke volume, and greater Tei index. Exercise performance, levels of brain natriuretic peptide in the serum, and summary and domain scores from health status questionnaires, were not different from those not having heterotaxy.ConclusionsThe study illustrates a profile of characteristics, medical history, functional health state, and markers of ventricular performance in patients with heterotaxy after the Fontan procedure. Despite obvious anatomic differences, and some differences in echocardiography and heart rhythm, there were no important differences in exercise performance or functional health state between these patients and other survivors of the Fontan procedure.

Calcium channel blockers as a group are responsible for significant morbidity and mortality with toxic exposures. Isradipine, a cardioselective dihydropyridine calcium channel blocker, rarely has been implicated, with only two reports in the literature of significant toxic reactions, one in an adult and another in a child. To our knowledge, we describe the first case of life-threatening isradipine poisoning in a child and provide documentation of serum drug levels. On arrival at the hospital, a 5-year-old girl had abdominal distention and bradycardia that rapidly progressed to asystole. She received 73 minutes of cardiopulmonary resuscitation and transvenous cardiac pacing and survived with an intact neurologic recovery. Serum concentrations of isradipine were 30-100 times those found with therapeutic use.

The practice of coiling aortopulmonary collaterals (APCs) before Fontan completion is controversial, and published data are limited. We sought to compare outcomes in subjects with and without pre-Fontan coil embolization of APCs using the Pediatric Heart Network Fontan Cross-Sectional Study database which enrolled survivors of prior Fontan palliation.We compared hospital length of stay after Fontan in 80 subjects who underwent APC coiling with 459 subjects who did not. Secondary outcomes included post-Fontan complications and assessment of health status and ventricular performance at cross-sectional evaluation (mean 8.6 ± 3.4 years after Fontan).Centers varied markedly in frequency of pre-Fontan APC coiling (range 0%-30% of subjects, P.001). The coil group was older at Fontan (P = .004) and more likely to have single right ventricular morphology (P = .054) and pre-Fontan atrioventricular valve regurgitation (P = .03). The coil group underwent Fontan surgery more recently (P.001), was more likely to have a prior superior cavopulmonary anastomosis (P.001), and more likely to undergo extracardiac Fontan connection (P.001) and surgical fenestration (P.001). In multivariable analyses, APC coiling was not associated with length of stay (hazard ratio for remaining in-hospital 0.91, 95% CI 0.70-1.18, P = .48) or postoperative complications, except more post-Fontan catheter interventions (hazard ratio 1.74, 95% CI 1.04-2.91, P = .03), primarily additional APC coils. The groups had similar outcomes at cross-sectional evaluation.Management of APCs before Fontan shows marked practice variation. We did not find an association between pre-Fontan coiling of APCs and shorter postoperative hospital stay or with better late outcomes. Prospective studies of this practice are needed.