Outcomes of left atrial isomerism over a a 28-year period at a single institution

OBJECTIVES We determined long-term outcomes in a large cohort With left atr ial isomerism (LAI). BACKGROUND Left atrial isomerism is associated with a complex spectrum of c ardiac and noncardiac anomalies that may impact on outcomes. METHODS The records of all patients with LAI, born between 1970 and 1998, a nd treated at one center were reviewed. Kaplan-Meier survival was estimated , and independent factors associated with time-related death were identifie d. RESULTS There were 163 patients (63% women), and extracardiac anomalies wer e noted in 36%, including biliary atresia in 10%. Cardiac defects included interrupted inferior caval vein in 92%, anomalous pulmonary veins in 56%, a trioventricular septal defect in 49%, pulmonary atresia or stenosis in 28% and aortic coarctation in 16%, with congenital atrioventricular block in 7% . Of 22 patients with a normal heart, 18% died of extracardiac anomalies. O f 71 patients with hearts suitable for biventricular repair, 62 (87%) had s urgery, with survival of 80% at one year, 7146 at fit.e years, 66% at 10 ye ars and 63% after 15 years. Of 70 patients with unbalanced cardiac defects suitable for single-ventricle palliation, il 47 (67%) had surgery, with sur vival of 73% at one year, 61% at five years, 53% at 10 years and 48% at 15 years (p < 0.001). Independent factors associated with time-related death i ncluded congenital atrioventricular block, aortic coarctation, single ventr icle, biliary atresia and other gastrointestinal malformations. CONCLUSIONS Both cardiac and noncardiac anomalies contribute to a high mort ality with LAI. Cardiac transplantation may need to be a considered a prima ry option for selected high-risk patients. (C) 2000 by the American College of Cardiology.