Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery in a Neonate with Turner Syndrome and Aortic Arch Hypoplasia

Anomalous origin of the right coronary artery from the pulmonary artery, a rare congenital cardiac defect, is typically not diagnosed during infancy. On the other hand, Turner syndrome is usually diagnosed early, and it is classically associated with bicuspid aortic valve and aortic coarctation. Individuals with Turner syndrome are also at increased risk for coronary artery anomalies. We present a case of anomalous right coronary artery from the pulmonary artery in a week-old neonate who also had Turner syndrome, patent ductus arteriosus, transverse aortic arch hypoplasia, and impaired ventricular function. Prostaglandin therapy through the ductus increased the patient\u27s myocardial perfusion. Four months after corrective surgery, she was doing well. We discuss the reperfusion phenomenon in our patient\u27s case, as well as other considerations in this combination of congenital defects

mRNA Coronavirus Disease 2019 Vaccine-Associated Myopericarditis in Adolescents: A Survey Study

In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA coronavirus disease 2019 (COVID-19) vaccine-associated myopericarditis was noted. Only one adolescent with life-threatening complications was reported, with no deaths at any of the participating institutions

High burden of premature ventricular contractions in structurally normal hearts: To worry or not in pediatric patients?

BACKGROUND: There is paucity of data regarding the significance of high percentage of premature ventricular contractions (PVCs) in healthy children and their impact on left ventricular (LV) function and the risk of ventricular arrhythmias. The purpose of this study was to assess the prevalence of LV systolic dysfunction in children with frequent PVCs and determine whether PVC characteristics were predictive of LV dysfunction. METHODS: We performed a single-center retrospective review to examine the prevalence of PVC-induced cardiomyopathy and natural history of PVC burden in children with frequent PVCs. Children aged 6 months-21 years with PVCs noted on 24-hr Holter monitoring studies were enrolled. The four categories included those with a PVC burden of >10%, 10%-20%, and those with more than 20% PVC burden. RESULTS: A total of 134 children were included, 65 with more than 10% PVCs and 31 with more than 20% PVCs. Median age of the patients was 10.5 years (IQR 6.1-14.8 years), with 79 males (54.5%). Median PVC burden was 8.7% (IQR 4.2%-16.9%) with median follow-up of 2.8 years (IQR 1.2-4.6 years). During 2.8 years (1.3-4.3 years) of follow-up, the PVC burden decreased by 67% of baseline PVC burden in those who did not undergo any intervention. There were no deaths. CONCLUSION: PVCs in children with structurally normal hearts are associated with a relatively benign course, with trend toward spontaneous resolution

Pediatric noncompaction patients with high spatial QRS-T angles are at increased risk for ventricular tachycardia.

INTRODUCTION: Noncompaction cardiomyopathy (NCCM) patients may develop sustained ventricular arrhythmias (VA). Currently no known electrocardiogram (ECG) parameter has demonstrated predictive value for VA development. The spatial QRS-T angle has demonstrated ability to identify VA in other cardiomyopathy populations. METHODS: A total of 39 patients with NCCM, defined by compact to non-compact ratio of >2.3 by magnetic resonance imaging, were assessed. The first ECG taken at time of MRI was assessed utilizing the heart rate, the QRS duration (QRSd), the corrected QT interval (QTc), and the spatial QRS-T angle (SPQRS-T angle, three-dimensional angle between the QRS and T-wave vectors) were assessed. RESULTS: Eight patients developed VA (20.5%). Median time to event was 3 months (95% CI 1.0 to 24.0 months). There were no significant differences between baseline ejection fraction or fractional shortening. Baseline median heart rate, spatial QRS-T angles, and indexed left ventricular end-diastolic volumes were all significantly higher in patients with VA development (p-value <0.05). Only heart rate and the SPQRS-T angle had significant univariate hazard ratios (HR) for VA at 1.031/beat per minute (1.001-1.071) and at a cut-off of 147 degrees the SPQRS-T angle gave a hazard ratio of HR of 5.773 (95% CI 1.161 to 28.702). The multivariate hazard ratio was only significant for the SPQRS-T angle, 1.031/degree (1.001-1.066). Survival analysis by Kaplan-Meier yielded a significant difference at a cutoff of 147 degrees. CONCLUSION: The SPQRS-T angle identified those at risk for VA development. Future studies are warranted with larger populations of noncompaction patients

High burden of premature ventricular contractions in structurally normal hearts : To worry or not in pediatric patients?

Background: There is paucity of data regarding the significance of high percentage of premature ventricular contractions (PVCs) in healthy children and their impact on left ventricular (LV) function and the risk of ventricular arrhythmias. The purpose of this study was to assess the prevalence of LV systolic dysfunction in children with frequent PVCs and determine whether PVC characteristics were predictive of LV dysfunction. Methods: We performed a single-center retrospective review to examine the prevalence of PVC-induced cardiomyopathy and natural history of PVC burden in children with frequent PVCs. Children aged 6 months–21 years with PVCs noted on 24-hr Holter monitoring studies were enrolled. The four categories included those with a PVC burden of >10%, 10%–20%, and those with more than 20% PVC burden. Results: A total of 134 children were included, 65 with more than 10% PVCs and 31 with more than 20% PVCs. Median age of the patients was 10.5 years (IQR 6.1–14.8 years), with 79 males (54.5%). Median PVC burden was 8.7% (IQR 4.2%–16.9%) with median follow-up of 2.8 years (IQR 1.2–4.6 years). During 2.8 years (1.3–4.3 years) of follow-up, the PVC burden decreased by 67% of baseline PVC burden in those who did not undergo any intervention. There were no deaths. Conclusion: PVCs in children with structurally normal hearts are associated with a relatively benign course, with trend toward spontaneous resolution

Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery in a Neonate with Turner Syndrome and Aortic Arch Hypoplasia

Anomalous origin of the right coronary artery from the pulmonary artery, a rare congenital cardiac defect, is typically not diagnosed during infancy. On the other hand, Turner syndrome is usually diagnosed early, and it is classically associated with bicuspid aortic valve and aortic coarctation. Individuals with Turner syndrome are also at increased risk for coronary artery anomalies. We present a case of anomalous right coronary artery from the pulmonary artery in a week-old neonate who also had Turner syndrome, patent ductus arteriosus, transverse aortic arch hypoplasia, and impaired ventricular function. Prostaglandin therapy through the ductus increased the patient's myocardial perfusion. Four months after corrective surgery, she was doing well. We discuss the reperfusion phenomenon in our patient's case, as well as other considerations in this combination of congenital defects.</jats:p