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J Thorac Cardiovasc Surg 1998;116:417-431
© 1998 Mosby, Inc.
Surgery for Congenital Heart Disease |
From the Division of Cardiothoracic Surgery, Department of Surgery,Deborah Heart and Lung Center, Browns Mills, NJ, The Department of Surgery,University of Alabama at Birmingham, The Division of Cardiac Surgery, Departmentof Surgery, Loma Linda University, Loma Linda, and the Congenital Heart SurgeonsSociety.
Presented at the Seventieth Scientific Sessions, American HeartAssociation, Orlando, Fla, Nov 11, 1997.
Received for publication May 14, 1998; revisions requested May 19,1998; revisions received May 26, 1998; accepted for publication May 26, 1998. Address for reprints: Marshall L. Jacobs, MD, Deborah Heart and LungCenter, 200 Trenton Rd, Browns Mills, NJ 08015
Objective: Controversy persists withregard to the treatment of patients with aortic atresia. Staged reconstructiveoperations and primary transplantation have been advocated as treatmentstrategies, but in many instances no treatment is undertaken. Amulti-institutional study was undertaken for the purpose of characterizing thischallenging patient group, comparing the prevalence and outcomes of the varioustreatment strategies, and identifying potential predictors of success or failurewith each. Methods and results: A total of 323neonates with aortic atresia were entered into a 21-institution prospective,nonrandomized study between January 1, 1994, and January 1, 1997. Threeprotocols were used, nonexclusively in many institutions: (1) stagedreconstructive surgery with initial palliation by a Norwood procedure andeventual Fontan operation, (2) heart transplantation as initial definitivetherapy, and (3) nonsurgical management. Analysis was based on initial protocolassignment: staged reconstructive surgery in 253 patients, heart transplantationin 49 patients, and nonsurgical management in 21 patients. For all patientsinitially entered into the 2 surgical treatment protocols, survival at 1, 3, 12,24, and 36 months after entry was 67%, 59%, 52%, 51%,and 50%, respectively. A multivariable analysis found incremental riskfactors for death at any time after entry to be lower birth weight (P = .04), associated noncardiac anomaly (P = .007), and entry into the nonsurgical protocol (P < .0001) or the staged reconstructive surgeryprotocol (P = .03). Four institutions hadhigher survival statistics; 2 used a heart transplantation protocol and 2 used astaged reconstructive surgery protocol. For the 113 patients treated at these 4institutions, survival at 1, 3, 12, 24, and 36 months after entry was 77%,70%, 64%, 62%, and 61%, respectively. Survival amongthe 4 institutions was similar (P = 0.1).Conclusions: Among patients with aortic atresia,other features of cardiac structure including aortic size, degree of leftventricular hypoplasia, and degree of mitral hypoplasia or atresia are notpredictive of survival from 2 surgical protocols. The highest survival wasachieved with either treatment strategy at institutions strongly committed tothe use of one or the other surgical management protocol. (J Thorac CardiovascSurg 1998;116:417-31)
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