Congenital heart disease (CHD) is the most common birth defect in the United States. Neonates with CHD are often cared for by neonatologists in addition to cardiologists. However, there is a paucity of rigorous evidence and limited clinical trials regarding the management of neonates with CHD. In this review, we will describe some of the challenges of research in this field. The Pediatric Heart Network serves as an example of how a research network can effectively overcome barriers to conduct and execute well-designed multicenter studies.
Objectives Recent literature examining insurance administrative data suggests that a selective approach, with concurrent history and physical exam (H&P), for obtaining an electrocardiogram ( ECG ) as a part of a preparticipation examination ( PPE ) for pediatric athletes is commonly used in the primary care setting demonstrating a high rate of disease detection. We sought to understand practice patterns of providers with regard to usage of ECG as a part of PPE . Methods Utilizing an online survey, we queried primary care providers regarding their practice patterns, rationale, and concerns regarding use of ECG s as a part of the PPE . Results A total of 125 pediatricians completed the survey; 73.7% selectively use the ECG , 24.6% never use the ECG , and only 1.7% always obtain an ECG as part of the PPE . The most common rationale for selectively or never using the ECG is the belief that the H&P is sufficient to identify cardiac disease (70%). The most common H&P findings that lead to ECG screening include chest pain or syncope with exertion, family history of sudden cardiac death, an irregular heart rate, and a diastolic murmur. Among the diseases associated with sudden cardiac death, most pediatricians fear missing hypertrophic cardiomyopathy. Conclusion Based on a survey of primary care providers, most practitioners are utilizing a selective approach of obtaining an ECG as a part of a PPE for athletic participation, which is in agreement with the current American Heart Association guidelines. Significant practice variation continues to exist, and may represent an area for future resource optimization.
Knowledge gaps persist about the incidence of and risk factors for sudden death in the young (SDY). The SDY Case Registry is a collaborative effort between the National Institutes of Health, the Centers for Disease Control and Prevention, and the Michigan Public Health Institute. Its goals are to: (1) describe the incidence of SDY in the United States by using population-based surveillance; (2) compile data from SDY cases to create a resource of information and DNA samples for research; (3) encourage standardized approaches to investigation, autopsy, and categorization of SDY cases; (4) develop partnerships between local, state, and federal stakeholders toward a common goal of understanding and preventing SDY; and (5) support families who have lost loved ones to SDY by providing resources on bereavement and medical evaluation of surviving family members. Built on existing Child Death Review programs and as an expansion of the Sudden Unexpected Infant Death Case Registry, the SDY Case Registry achieves its goals by identifying SDY cases, providing guidance to medical examiners/coroners in conducting comprehensive autopsies, evaluating cases through child death review and an advanced review by clinical specialists, and classifying cases according to a standardized algorithm. The SDY Case Registry also includes a process to obtain informed consent from next-of-kin to save DNA for research, banking, and, in some cases, diagnostic genetic testing. The SDY Case Registry will provide valuable incidence data and will enhance understanding of the characteristics of SDY cases to inform the development of targeted prevention efforts.
udden cardiac death (SCD) in the young (SCDY) has a devastating impact on families, care providers, and the community and attracts significant public and media attention.Sudden cardiac death is defined as an abrupt and unexpected death due to a cardiovascular cause, typically occurring Ͻ1 hour from the onset of symptoms.Depending on the source, "young" is variably defined as those less than 25, 30, 35, or 40 years of age.][3] Sudden infant death syndrome (SIDS) may be related to SCD in some infants.Sudden infant death syndrome is defined as the sudden death of an infant Ͻ1 year of age that cannot be explained after a thorough investigation is conducted, including an autopsy, death scene evaluation, and review of the clinical history.The incidence of SIDS ranges from 50 to 100 in 100 000, 4 and emerging data suggest that as many as 10% to 15% of SIDS deaths are associated with functional cardiac ion channelopathy gene variants. 5he most common diagnoses that increase risk for SCDY include hypertrophic cardiomyopathy (HCM), coronary artery anomalies of wrong sinus origin, myocarditis, arrhythmogenic right ventricular cardiomyopathy, and ion channelopathies. 6The latter category includes hereditary diseases such as the congenital long-QT syndromes (LQTS), catecholaminergic polymorphic ventricular tachycardia, and Brugada syndrome, among other less common channelopathies.These diseases are typically undetected before the SCD event.Estimated prevalence
Importance Multisystem inflammatory syndrome in children (MIS-C) is a life-threatening complication of COVID-19 infection. Data on midterm outcomes are limited. Objective To characterize the frequency and time course of cardiac dysfunction (left ventricular ejection fraction [LVEF] <55%), coronary artery aneurysms ( z score ≥2.5), and noncardiac involvement through 6 months after MIS-C. Design, Setting, and Participants This cohort study enrolled participants between March 2020 and January 2022 with a follow-up period of 2 years. Participants were recruited from 32 North American pediatric hospitals, and all participants met the 2020 Centers for Disease Control and Prevention case definition of MIS-C. Exposure MIS-C after COVID-19 infection. Main Outcomes and Measures Outcomes included echocardiography core laboratory (ECL) assessments of LVEF and maximum coronary artery z scores (zMax); data collection on cardiac and noncardiac sequelae during hospitalization and at 2 weeks, 6 weeks, and 6 months after discharge; and age-appropriate Patient-Reported Outcomes Measurement Information Systems (PROMIS) Global Health Instruments at follow-up. Descriptive statistics, linear regression models, and Kaplan-Meier analysis were used. Results Of 1204 participants (median [IQR] age, 9.1 [5.6-12.7] years; 724 male [60.1%]), 325 self-identified with non-Hispanic Black race (27.0%) and 324 with Hispanic ethnicity (26.9%). A total of 548 of 1195 participants (45.9%) required vasoactive support, 17 of 1195 (1.4%) required extracorporeal membrane oxygenation, and 3 (0.3%) died during hospitalization. Of participants with echocardiograms reviewed by the ECL (n = 349 due to budget constraints), 131 of 322 (42.3%) had LVEF less than 55% during hospitalization; of those with follow-up, all but 1 normalized by 6 months. Black race (vs other/unknown race), higher C-reactive protein level, and abnormal troponin level were associated with lowest LVEF (estimate [SE], −3.09 [0.98]; R 2 = 0.14; P =.002). Fifteen participants had coronary artery z scores of 2.5 or greater at any time point; 1 participant had a large/giant aneurysm. Of the 13 participants with z scores of 2.5 or greater during hospitalization, 12 (92.3%) had normalized by 6 months. Return to greater than 90% of pre–MIS-C health status (energy, sleep, appetite, cognition, and mood) was reported by 711 of 824 participants (86.3%) at 2 weeks, increasing to 548 of 576 (95.1%) at 6 months. Fatigue was the most common symptom reported at 2 weeks (141 of 889 [15.9%]), falling to 3.4% (22 of 638) by 6 months. PROMIS Global Health parent/guardian proxy median T scores for fatigue, global health, and pain interference improved significantly from 2 weeks to 6 months (fatigue, 56.1 vs 48.9; global health, 48.8 vs 51.3; pain interference, 53.0 vs 43.3; P < .001) and by the 6-week visit were at least equivalent to prepandemic population norms. Conclusions and Relevance Results of this cohort study suggest that although children and young adults with MIS-C can have severe disease during the acute phase, most recovered quickly and had a reassuring midterm prognosis.
