OBJECTIVES: We determined long-term outcomes in a large cohort with left atrial isomerism (LAI). BACKGROUND: Left atrial isomerism is associated with a complex spectrum of cardiac and noncardiac anomalies that may impact on outcomes. METHODS: The records of all patients with LAI, born between 1970 and 1998, and treated at one center were reviewed. Kaplan-Meier survival was estimated, and independent factors associated with time-related death were identified. RESULTS: There were 163 patients (63% women), and extracardiac anomalies were noted in 36%, including biliary atresia in 10%. Cardiac defects included interrupted inferior caval vein in 92%, anomalous pulmonary veins in 56%, atrioventricular 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. Of 71 patients with hearts suitable for biventricular repair, 62 (87%) had surgery, with survival of 80% at one year, 71% at five years, 66% at 10 years and 63% after 15 years. Of 70 patients with unbalanced cardiac defects suitable for single-ventricle palliation, 47 (67%) had surgery, with survival 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 included congenital atrioventricular block, aortic coarctation, single ventricle, biliary atresia and other gastrointestinal malformations. CONCLUSIONS: Both cardiac and noncardiac anomalies contribute to a high mortality with LAI. Cardiac transplantation may need to be a considered a primary option for selected high-risk patients.
OBJECTIVES: We determined long-term outcomes in a large cohort with left atrial isomerism (LAI). BACKGROUND:Left atrial isomerism is associated with a complex spectrum of cardiac and noncardiac anomalies that may impact on outcomes. METHODS: The records of all patients with LAI, born between 1970 and 1998, and treated at one center were reviewed. Kaplan-Meier survival was estimated, and independent factors associated with time-related death were identified. RESULTS: There were 163 patients (63% women), and extracardiac anomalies were noted in 36%, including biliary atresia in 10%. Cardiac defects included interrupted inferior caval vein in 92%, anomalous pulmonary veins in 56%, atrioventricular 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. Of 71 patients with hearts suitable for biventricular repair, 62 (87%) had surgery, with survival of 80% at one year, 71% at five years, 66% at 10 years and 63% after 15 years. Of 70 patients with unbalanced cardiac defects suitable for single-ventricle palliation, 47 (67%) had surgery, with survival 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 included congenital atrioventricular block, aortic coarctation, single ventricle, biliary atresia and other gastrointestinal malformations. CONCLUSIONS: Both cardiac and noncardiac anomalies contribute to a high mortality with LAI. Cardiac transplantation may need to be a considered a primary option for selected high-risk patients.
Authors: Matthew Swisher; Richard Jonas; Xin Tian; Elaine S Lee; Cecilia W Lo; Linda Leatherbury Journal: J Thorac Cardiovasc Surg Date: 2010-09-29 Impact factor: 5.209
Authors: Y Maeno; W Himeno; A Saito; S Hiraishi; O Hirose; M Ikuma; N Inamura; M Kawataki; A Mizukami; M Ota; H Shiraishi; G Satomi; H Kato Journal: Heart Date: 2005-08 Impact factor: 5.994
Authors: Maria C Escobar-Diaz; Kevin Friedman; Yishay Salem; Gerald R Marx; Brian T Kalish; Terra Lafranchi; Rahul H Rathod; Sitaram Emani; Tal Geva; Wayne Tworetzky Journal: Am J Cardiol Date: 2014-06-06 Impact factor: 2.778