Adverse Effect of Bundle Branch Block on Exercise Performance in Patients with Fontan Physiology: From the Pediatric Heart Network Fontan Public Data Set.

Patients with Fontan physiology have reduced exercise performance compared to their peers as well as a higher incidence of bundle branch block (BBB). This study aims to investigate the association between BBB and exercise performance in the Fontan population through a retrospective review of the Pediatric Heart Network Fontan study public use dataset. "Low Performers" were defined as ≤ 25th percentile (for Fontan patients) for each exercise parameter at anaerobic threshold (AT) for gender and age and "Normal Performers" were all other patients. A total of 303 patients with Fontan physiology who underwent exercise testing reached AT and had complete data for BBB. BBB occurred more frequently in Low Performers for VO2 [OR (95% CI): 2.6 (1.4, 4.8)] and Work [OR (95% CI): 2.7 (1.4, 5.1)], suggesting that BBB in the Fontan population is associated with reduced exercise performance. This data adds to the existing clinical evidence of the adverse effects of conduction abnormalities on single ventricle cardiac output and adds support for consideration of cardiac resynchronization and multi-site ventricular pacing in this patient population.

Worse Hospital Outcomes for Children and Adults with COVID-19 and Congenital Heart Disease.

The aim of the current study is to investigate hospitalization outcomes of COVID-19 positive children and adults with moderate or severe congenital heart disease to children and adults without congenital heart disease. Retrospective review using the Vizient Clinical Data Base for admissions of patients with an ICD-10 code for COVID-19 from April 2020 to March 2021. Admissions with COVID-19 and with and without moderate or severe congenital heart disease (CHD) were stratified into pediatric (< 18 years) and adult (≥ 18 years) and hospital outcomes were compared. There were 9478 pediatric COVID-19 admissions, 160 (1.7%) with CHD, and 658,230 adult COVID-19 admissions, 389 (0.06%) with CHD. Pediatric admissions with COVID-19 and CHD were younger (1 vs 11 years), had longer length of stay (22 vs 6 days), higher complication rates (6.9 vs 1.1%), higher mortality rates (3.8, 0.8%), and higher costs ($54,619 vs 10,731; p < 0.001 for all). Adult admissions with COVID-19 and CHD were younger (53 vs 64 years, p < 0.001), had longer length of stay (12 vs 9 days, p < 0.001), higher complication rates (8 vs 4.8%, p = 0.003), and higher costs ($23,551 vs 13,311, p < 0.001). This appears to be the first study to report the increased hospital morbidities and costs for patients with CHD affected by COVID-19. Our hope is that these findings will help counsel patients moving forward during the pandemic.

Development of bilateral coronary artery aneurysms in a child with Noonan syndrome.

Noonan syndrome is a constellation of congenital malformations including heart defects, facial anomalies and short stature. The cardiovascular defects are variable and extensive, with the most common being pulmonary stenosis and hypertrophic cardiomyopathy. Coronary artery anomalies have only been reported in a few cases. We report a child with Noonan syndrome status post pulmonary stenosis and atrial septal defect repair, who developed bilateral coronary artery aneurysms. The aneurysms were diagnosed with both cardiac magnetic resonance imaging and coronary computed tomography angiography. There had been no evidence of them on a cardiac MR exam 5 years previously.

Cardiac function in congenital adrenal hyperplasia: a pattern of reversible cardiomyopathy.

OBJECTIVE: To evaluate cardiac function in infants with congenital adrenal hyperplasia (CAH) before and after corticosteroid replacement therapy. STUDY DESIGN: This prospective, case-control study included 9 infants with CAH. Cardiac function was assessed by echocardiography at presentation and after corticosteroid replacement therapy. Six term infants underwent 2 echocardiograms each and served as the control group. Data on fractional shortening (FS), rate-corrected velocity of circumferential fiber shortening (Vcf), wall stress, tissue Doppler indices, myocardial performance index, left ventricular mass, and Vcf/wall stress were obtained. RESULTS: The infants with CAH exhibited myocardial dysfunction at baseline and lower systolic blood pressure (SBP) compared with the control group. FS, a measure of systolic contractility, differed significantly from before to after corticosteroid treatment (mean, 32.3%±4.7% pretreatment, 39.9%±5.0% posttreatment). Vcf, a preload-independent measure of cardiac contractility, also differed significantly before and after treatment (mean, 1.23±0.16 circumferences/second pretreatment, 1.45±0.22 circumferences/second posttreatment). SBP was also lower (mean, 84±9.3 mmHg) and improved with treatment (mean, 95±4.8 mmHg). The control group demonstrated no statistically significant changes in FS, Vcf, or SBP. There was a change in left ventricular mass in the control group between the 2 studies. CONCLUSION: Newborns with CAH have evidence for cardiac dysfunction at baseline that reverses with corticosteroid replacement therapy. These data suggest that corticosteroids play a direct role in modulating cardiac function in the newborn.

Pulmonary vein atresia with severe contralateral pulmonary vein stenosis in a child.

Unilateral pulmonary vein atresia is a very rare congenital anomaly associated with high morbidity and mortality. Most cases present in infancy or childhood with recurrent respiratory infections or hemoptysis. Further, the diagnosis can often be difficult to make. We present an infant who died due to unilateral right-sided pulmonary vein atresia in conjunction with severe contralateral pulmonary vein stenosis who was diagnosed with the assistance of cardiac computed tomography scanning.

Exercise Performance in Adolescents With Fontan Physiology (from the Pediatric Heart Network Fontan Public Data Set).

In the pediatric population, exercise capacity differs between females and males and the gap widens through adolescence. However, specific age- and sex-based changes in adolescents with congenital heart disease and Fontan palliation have not been reported. The purpose of the current study is to identify age- and sex-specific changes in exercise performance at peak and ventilatory anaerobic threshold (AT) for adolescents with Fontan physiology. Retrospective review of the Pediatric Heart Network Fontan cross sectional study (Fontan 1) public use dataset. Comparisons were made for peak and AT exercise parameters for females and males at 2-year age intervals. In addition, normative values were generated by sex and age at 2-year intervals. χ2 test was used for comparison for categorical variables. Changes in exercise parameters between age groups by sex were compared by ANOVA with post-hoc analysis. Exercise testing was performed in 411 patients. AT was reached in 317 subjects (40% female), of whom, 166 (43% female) reached peak exercise. Peak oxygen consumption decreased 32% through adolescence in females and did not have the typical increase through adolescence for males. Oxygen consumption at AT also decreased with age in both sexes. In conclusion, age- and sex-based exercise performance for adolescents with Fontan physiology are predictably low, but there are additional significant decreases through adolescence for this population, especially in females. We have established normative exercise values for several parameters for this population which will better identify at risk patients and allow for earlier intervention.

Anatomically corrected malposition of the great arteries.

We present cardiac magnetic resonance images of a 6-year-old with a suspected great artery abnormality in the setting of mesocardia, atrial situs solitus, D-looping of the ventricles, and anatomically corrected malposition of the great arteries with subaortic and subpulmonary conus.

Assessment of the accuracy and reproducibility of RV volume measurements by CMR in congenital heart disease.

OBJECTIVES: The purpose of this study was to determine whether right ventricular (RV) volumes are more accurately and reproducibly measured by cardiac magnetic resonance (CMR) in an axial orientation or in a short-axis orientation in patients with congenital heart disease (CHD). BACKGROUND: There is little agreement on the most suitable imaging plane for RV volumetric analysis in the setting of abnormal RV physiology. METHODS: Measurements of RV volumes from datasets acquired in axial and short-axis orientations were made in 50 patients with CHD. RV stroke volumes (SV) calculated using these 2 methods were compared with forward flow measured in the pulmonary trunk by phase contrast (PC) imaging. Repeated volume measurements were made to assess intraobserver and interobserver reliability. Bland-Altman plots and Lin's concordance correlation coefficient (CCC) were used for all analyses of agreement. RESULTS: Analysis of all subjects revealed a statistically significant difference in interobserver reliability of RV end-systolic volume (ESV) measurements that favored the axial method (p = 0.047). The magnitude of measurement differences between observers in this case was small (-2.8 ml/m(2); 95% confidence interval: -5.6 to 0.0). There was no difference between the 2 contouring methods in terms of intraobserver reliability in measurements of RV end-diastolic volume (EDV), ESV, ejection fraction, or SV (p > 0.05 in all cases). In subjects with RV EDV ≥ 150 ml/m(2), RV SV measured using axial contours yielded better agreement with forward flow measured in the pulmonary trunk (CCC = 0.63) than did measurements made using short-axis contours (CCC = 0.56; p = 0.007). CONCLUSIONS: Trends favoring the axial orientation in terms of reproducibility were not clinically significant. In subjects with RV EDV ≥ 150 ml/m(2), the axial orientation yields RV volume measurements that agree more closely with flow measured in the pulmonary trunk than does the short-axis orientation.

Accuracy of plasma B-type natriuretic peptide to diagnose significant cardiovascular disease in children: the Better Not Pout Children! Study.

OBJECTIVES: The purpose of this study was to assess the ability of plasma B-type natriuretic peptide (BNP) to diagnose significant cardiovascular disease (CVD) in the pediatric population. BACKGROUND: BNP has been shown to be reliable in detecting ventricular dysfunction and heart failure in adults. Timely and accurate identification of significant pediatric heart disease is important but challenging. A simple blood test could aid the front-line physician in this task. METHODS: Subjects without a history of heart disease with findings possibly attributable to significant CVD in the acute care setting requiring a cardiology consult were enrolled. Clinicians were blinded to the BNP result, and confirmation of disease was made by cardiology consultation. RESULTS: Subjects were divided into a neonatal (n = 42, 0 to 7 days) and older age group (n = 58, >7 days to 19 years). CVD was present in 74% of neonates and 53% of the older age group. In neonates with disease, median BNP was 526 pg/ml versus 96 pg/ml (p < 0.001) for those without disease. In older children with disease, median BNP was 122 pg/ml versus 22 pg/ml in those without disease (p < 0.001). Subjects with disease from an anatomic defect, a longer hospital stay, or who died had higher BNP. A BNP of 170 pg/ml yielded a sensitivity of 94% and specificity of 73% in the neonatal group and 87% and 70% in the older age group, respectively, using a BNP of 41 pg/ml. CONCLUSIONS: BNP is a reliable test to diagnose significant structural or functional CVD in children. Optimal cutoff values are different from adult values.

The safety and efficacy of ibutilide in children and in patients with congenital heart disease.

BACKGROUND: The safety and efficacy of ibutilide in the cardioversion of atrial flutter and atrial fibrillation in children and in patients with congenital heart disease (CHD) is unknown. METHODS: Data from 19 patients (age 6 months to 34 years, median 16 years) who received ibutilide for atrial flutter or atrial fibrillation between 1996 and 2005 was retrospectively reviewed. There were 15 patients with CHD (14 had prior heart surgery); four children had normal heart structure. RESULTS: There were 74 episodes of atrial flutter and four episodes of atrial fibrillation (median episodes per patient was one, range 1-31). Ibutilide converted 55 of all the episodes (71%). Ibutilide was successful during its first-ever administration in 12 of 19 patients (63%). Fourteen episodes in six patients required electrical cardioversion after ibutilide failed. There were no episodes of symptomatic bradycardia. One patient went into torsade de pointes and one patient had nonsustained ventricular tachycardia. CONCLUSION: With careful monitoring, ibutilide can be an effective tool in selected patients for cardioversion of atrial flutter.