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J Am Coll Cardiol. 2000 Sep;36(3):908-16.

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

Author information

1
Department of Pediatrics, Hospital for Sick Children and the University of Toronto Faculty of Medicine, Ontario, Canada.

Abstract

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.

PMID:
10987619
[Indexed for MEDLINE]
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