Project Details

Early Career

Status: Funded - Open

Natural History and Consequences of Aortic Dilatation in Children with Conotruncal Malformations

Aditya Sengupta, MD


BACKGROUND: Aortic or neoaortic root and ascending aortic dilatation (AAD) are common sequelae of conotruncal cardiac malformations (CTM) in children. GAP: The natural history of pathologic aortic dilatation in children with CTM is incompletely understood, with adverse implications for clinical management of the consequences of AAD. HYPOTHESIS: We hypothesize that 1) echocardiographically-derived aortic dimensions can be used to develop z-score equations that are adjusted for various patient-related variables; 2) nomograms derived for children with CTM using the aforementioned z-scores will be significantly different from that of the normal age-matched population, and a subset of children with CTM will display high-risk behavior in longitudinal z-score analyses; and 3) high-risk patterns of AAD will be significantly associated with the occurrence of adverse clinical events. METHODS: Data from patients with CTM who have undergone corrective repair at Boston Children’s Hospital from 1/1/2000 to 12/31/2020 will be retrospectively reviewed. Within each study group (categorized by age, gender, and diagnosis), z-score equations will be determined for all aortic dimensions of interest using linear regression analysis of the log-transformed values. Abnormal patterns of growth acceleration at various age categories will be identified. Logistic regression will be used to analyze the association between high-risk z-score characteristics and the occurrence of adverse outcomes. RESULTS: Pending. IMPACT: The use of normative aortic dimensions will allow clinicians to better understand the natural history of aortic dilatation in children with CTM, and assess the optimal timing of eventual surgical or medical therapy for the complications of pathologic AAD.