Severe obstruction to systemic blood flow in congenitally corrected transposition (discordant atrioventricular and ventriculo-arterial connexions): an analysis of 14 patients.

The more common associated cardiac anomalies in any cohort of patients with discordant atrioventricular and ventriculo-arterial connexions (congenitally corrected transposition) include ventricular septal defect, left ventricular (subpulmonary) outlet obstruction, and displacement and/or dysplasia of the morphologically tricuspid valve. There is scant mention in the literature of severe obstruction to systemic blood flow at aortic valve level or beyond in these hearts. The present study reviews those pertinent morphological-clinical and follow-up data on 14 patients with congenitally corrected transposition and aortic valve atresia (one patient); aortic arch interruption or aortic arch atresia (two patients) and coarctation of the aorta (11 patients). All but one patient (the patient with aortic valve atresia) had a perimembranous ventricular septal defect. Mild outflow tract obstruction of the morphologically left ventricle was identified in one patient. Nine patients, however, demonstrated a significant structural and functional disturbance (Ebstein-like with dysplasia) of the systemic morphologically tricuspid valve. No patient in this series was identified with isolated aortic coarctation.