Aortic atresia: morphologic characteristics affecting survival and operative palliation

Abstract

A quantitative anatomic study of 54 cardiac specimens with aortic atresia is related to surgical treatment of patients with aortic atresia and hypoplastic left heart syndrome. Coarctation of the aorta was present in 80% of patients with aortic atresia and should influence the extent of aortic arch reconstruction when present. Other associated cardiac defects were uncommon but, when present, may be contraindications to operative palliation because of added complexity of the operation. Anatomic abnormalities of the tricuspid or pulmonary valve were present in 7% of cases and could be important in the outcome of palliative procedures. Natural survival was related to thickness of the right ventricular wall so that selection of those infants with thicker and better functioning right ventricles may improve short-term and long-term operative results in aortic atresia.