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J Thorac Cardiovasc Surg 1994;107:1099-1113
© 1994 Mosby, Inc.


SURGERY FOR CONGENITAL HEART DISEASE

Outcomes in patients with interrupted aortic arch and ventricular septal defectA multiinstitutional study

R. A. Jonas, MD, Boston, Mass., J. M. Quaegebeur, MD, New York, N.Y., J. W. Kirklin, MD, E. H. Blackstone, MD, Birmingham, Ala., G. Daicoff, MD


St. Petersburg, Fla., and the Congenital Heart Surgeons Society

From the Department of Cardiac Surgery, The Children's Hospital, Boston, Mass., the Division of Cardiothoracic Surgery, College of Physician's and Surgeons of Columbia University, New York, N.Y., the Division of Cardiothoracic Surgery, the Department of Surgery, The University of Alabama at Birmingham Medical Center, Birmingham, Ala., and the Department of Cardiovascular and Thoracic Surgery, All Children's Hospital, St. Petersburg, Fla.

Address for reprints: John W. Kirklin, MD, University of Alabama at Birmingham, University Station, Birmingham, AL 35294.

Abstract

Among 183 neonates with interrupted aortic arch and ventricular septal defect entering a multiinstitutional study between 1987 and 1992, nine died before repair was accomplished. Among the remaining 174, survival at 1 month and 1, 3, and 4 years after repair was 73%, 65%, 63%, and 63%, respectively. The risk factors for death were low birth weight, younger age at repair, interrupted arch type B, outlet and trabecular ventricular septal defects, smaller size of the ventricular septal defect, and subaortic narrowing. Echocardiographically measured dimensions (expressed as Z-values) at all levels of the left heart–aorta complex were small. Two among thirty institutions were risk factors, and two others possibly were. Procedural risk factors for death after repair were (1) repair without concomitant procedures in patients with other important levels of obstruction in the left heart–aorta complex, (2) a Damus-Kaye-Stansel anastomosis, and (3) subaortic myotomy/myectomy in the face of subaortic narrowing. One-stage repair plus ascending aorta/arch augmentation had the highest predicted time-related survival in the 20% of patients with interrupted aortic arch and one or more coexisting levels of obstruction in the left heart–aorta complex, as did initial repair without or with aorta/arch augmentation in the 80% without these. (J THORAC CARDIOVASC SURG 1994;107:1099-113)




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