Original articleCardiovascularNatural History of Pulmonary Atresia With Intact Ventricular Septum and Right-Ventricle–Dependent Coronary Circulation Managed by the Single-Ventricle Approach
Section snippets
Patient Selection
Using the Cardiovascular Program’s database at Children’s Hospital Boston, after approval of this study by the Institutional Review Board, we identified all patients who were admitted to our institution from January 1, 1989, until December 31, 2004, with either a new diagnosis of PA/IVS-RVDCC or who were referred from an outside institution with an established diagnosis of PA/IVS-RVDCC having already undergone modified Blalock-Taussig shunt (BTS) or bidirectional Glenn (BDG) shunt following
Results
There were 32 patients admitted to our institution with PA/IVS-RVDCC, including 10 patients referred from outside institutions. Five patients were referred for BDG after BTS, 3 had already completed BDG and were referred for Fontan completion, and 2 patients who had new-onset LV dysfunction after BTS (at 11 and 82 days, respectively) were referred for transplant evaluation.
Patient characteristics are depicted in Table 1. In this cohort, median age at BTS was 2 days (range, 0 to 38), and median
Comment
Pulmonary atresia and intact ventricular septum is an uncommon congenital cardiac lesion that accounts for 3% of serious congenital heart defects, and the subset with RVDCC is even less common [22]. Whether the inciting developmental event occurs early or late in fetal life is unknown; however, it has been postulated that right ventricular outflow tract obstruction later during fetal life results in underdevelopment of the pulmonary valve, leading to a spectrum of associated abnormalities
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