Background— The goal of this statement was to review the available literature on surveillance, screening, evaluation, and management strategies and put forward a scientific statement that would comprehensively review the literature and create recommendations to optimize neurodevelopmental outcome in the pediatric congenital heart disease (CHD) population. Methods and Results— A writing group appointed by the American Heart Association and American Academy of Pediatrics reviewed the available literature addressing developmental disorder and disability and developmental delay in the CHD population, with specific attention given to surveillance, screening, evaluation, and management strategies. MEDLINE and Google Scholar database searches from 1966 to 2011 were performed for English-language articles cross-referencing CHD with pertinent search terms. The reference lists of identified articles were also searched. The American College of Cardiology/American Heart Association classification of recommendations and levels of evidence for practice guidelines were used. A management algorithm was devised that stratified children with CHD on the basis of established risk factors. For those deemed to be at high risk for developmental disorder or disabilities or for developmental delay, formal, periodic developmental and medical evaluations are recommended. A CHD algorithm for surveillance, screening, evaluation, reevaluation, and management of developmental disorder or disability has been constructed to serve as a supplement to the 2006 American Academy of Pediatrics statement on developmental surveillance and screening. The proposed algorithm is designed to be carried out within the context of the medical home. This scientific statement is meant for medical providers within the medical home who care for patients with CHD. Conclusions— Children with CHD are at increased risk of developmental disorder or disabilities or developmental delay. Periodic developmental surveillance, screening, evaluation, and reevaluation throughout childhood may enhance identification of significant deficits, allowing for appropriate therapies and education to enhance later academic, behavioral, psychosocial, and adaptive functioning.

Overall, children with cardiovascular disease perceived lower quality of life than healthy children across all age groups. As perceived by parents, overall quality of life was not significantly different in young children with cardiovascular disease, but children with more severe cardiovascular disease have worse physical and psychosocial quality of life. One in 5 children with cardiovascular disease perceives impaired psychosocial functioning, including children with mild disease severity.

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There is suboptimal weight gain between neonatal discharge and the bidirectional Glenn procedure. A lower weight-for-age z score and younger age at the time of the bidirectional Glenn procedure affects length of hospital stay independent of hemodynamic or echocardiographic variables.

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Background Multicenter longitudinal objective data for Fontan patients surviving into adulthood are lacking. Objectives Describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. Methods Exercise testing, echocardiography, B-type natriuretic peptide (BNP), functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) compared to previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation. Results From the original cohort of 546 subjects, 466 were recontacted and 373 (80%) were enrolled at 21.2 ± 3.5 years of age. Among subjects with paired testing, percent predicted maximum VO2 decreased (69 ± 14 vs. 61 ± 16, p <0.001, n = 95), ejection fraction decreased (58 ± 11 vs. 55 ± 10, p <0.001, n=259), and BNP increased (Median (IQR) 13 (7,25) vs. 18 (9,36) pg/mol, p <0.001, n = 340). At latest follow-up lower Pediatric Quality of Life Inventory (PedsQL) physical summary score was associated with poorer exercise performance (R2 adjusted = 0.20, p <0.001, n = 274). Cumulative complications since the Fontan included additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%), and protein losing enteropathy (8%). Since Fontan 1, 54 subjects (10%) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death/transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but was not associated with ventricular morphology, subject age or type of Fontan connection. Conclusions Interim transplant-free survival over 12 years in this Fontan cohort was 90% and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Clinical Trials Registration #: NCT00132782)

Overall, boys with DMD reported significantly lower QoL than their healthy peers. Despite decreased physical functioning, older boys seem to perceive better psychosocial QoL than perceived by their parents and by younger boys, unrelated to their need for mobility aids.