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Viktor Hraska
Brian W. Duncan
John E. Mayer, Jr
Pedro J. del Nido
Richard A. Jonas
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J Thorac Cardiovasc Surg 2005;129:182-191
© 2005 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Long-term outcome of surgically treated patients with corrected transposition of the great arteries

Viktor Hraska, MDa, Brian W. Duncan, MDb, John E. Mayer, Jr, MDc, Michael Freed, MDd, Pedro J. del Nido, MDc, Richard A. Jonas, MDe,*

a Department of Pediatric Cardiac Surgery, University Hospital Hamburg–Eppendorf, Germany
b Department of Pediatric Cardiac Surgery, Children's Hospital at the Cleveland Clinic, Cleveland, Ohio
c Department of Cardiovascular Surgery, Children's Hospital, Boston, Mass
d Department of Cardiology, Children's Hospital, Boston, Mass
e Children's National Medical Center, Washington, DC

Received for publication December 2, 2003; accepted for publication February 26, 2004.

* Address for reprints: Richard A. Jonas, MD, Cardiac Surgery, Children's National Medical Center, 111 Michigan Ave NW, Washington, DC 20010 (E-mail: rjonas{at}cnmc.org).

OBJECTIVES: The purpose of the study was to examine long-term outcome after traditional surgical treatment of corrected transposition of the great arteries to provide a basis for comparison with new procedures, such as the double-switch or Senning-Rastelli procedures.

METHODS: Patient- and procedure-related variables in 123 patients with corrected transposition and 2 functional ventricles operated on between 1963 and 1996 were analyzed. Patients with intracardiac procedures underwent either a traditional 2-ventricle repair or a Fontan procedure.

RESULTS: The 1-, 5-, 10-, and 15-year survivals after the operation were 84%, 75%, 68%, and 61%, respectively. Patients requiring tricuspid valve replacement (27 patients) at any time during follow-up had a significantly worse outcome (P < .001; hazard ratio, 4.4), whereas the best outcome was seen in patients undergoing the Fontan procedure (17 patients, 0 deaths). Right ventricular end-diastolic pressure of greater than 17 mm Hg before the operation (P < .0001), complete heart block after the operation (P = .001), subvalvular pulmonary stenosis (P = .013), Ebstein malformation of the tricuspid valve (P = .025), and preoperative systemic (right) ventricular dysfunction (P = .041) were identified as risk factors for death at any time by means of univariate analysis. Ebstein malformation of the tricuspid valve (P = .036; hazard ratio, 1.5) was identified as a risk factor for death by multivariate analysis.

CONCLUSIONS: The long-term outcome of patients with corrected transposition after a classic surgical approach is unsatisfactory. The poorest outcome was seen in patients who required tricuspid valve replacement either at their initial operation or later during follow-up. Alternative surgical approaches, such as the double-switch, Senning-Rastelli, or Fontan procedures, are likely to have better long-term results, especially in the highest risk groups.




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