CLINICAL STUDY
Outcomes of left atrial isomerism over a 28-year period at a single institution
Thomas Gilljam, MD, PhD*  ,
Brian W. McCrindle, MD, MPH, FACC* ,
Jeffrey F. Smallhorn, MBBS, FACC* ,
William G. Williams, MD, FRCS(C)* and
Robert M. Freedom, MD, FACC*
* Division of Cardiology, the Department of Pediatrics, the Hospital For Sick Children and the University of Toronto Faculty of Medicine, Toronto, Ontario, Canada
Division of Cardiovascular Surgery, the Department of Surgery, the Hospital For Sick Children and the University of Toronto Faculty of Medicine, Toronto, Ontario, Canada
Manuscript received June 17, 1999;
revised manuscript received April 3, 2000,
accepted April 11, 2000.
Reprint requests and correspondence: Dr. Robert M. Freedom, the Hospital For Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada
rfreedom{at}sickkids.on.ca
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.
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Abbreviations and Acronyms
| | AV | = atrioventricular | | BV | = biventricular | | LAI | = left atrial isomerism | | N | = normal | | SV | = single ventricle |
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