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. 2010 Sep 28:5:74.
doi: 10.1186/1749-8090-5-74.

Long term follow up after surgery in congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation

Ad J J C Bogers  1 , Stuart J Head, Peter L de Jong, Maarten Witsenburg, Arie Pieter Kappetein
Affiliations

Long term follow up after surgery in congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation

Ad J J C Bogers et al. J Cardiothorac Surg. .

Abstract

Aim of the study: To investigate the long-term outcome of surgical treatment for congenitally corrected transposition of the great arteries (CCTGA), in patients with biventricular repair with the right ventricle as systemic ventricle.

Methods: A total of 32 patients with CCTGA were operated between January 1972 and October 2008. These operations comprised 18 patients with a repair with a normal left ventricular outflow tract, 11 patients with a Rastelli repair of the left ventricle to the pulmonary artery and 3 patients with a cardiac transplantation.

Results: Excluding the cardiac transplantation patients, mean age at operation was 16 years (sd 15 years, range 1 week - 49 years). Median follow-up was 12 years (sd 10 years, range 7 days - 32 years). Survival obtained from Kaplan-Meier analysis at 20 years after surgery was 63% (CI 53-73%). For the non-Rastelli group these data at 20 years were 62% (CI 48-76%) and for the Rastelli group 67% (CI 51-83%). Freedom of reoperation at 20 years was 32% (CI 19-45%) in the overall group. In the non-Rastelli group the data at 20 years were 47% (CI 11-83%) and for the Rastelli group 21% (CI 0-54%) after almost 19 years.

Conclusions: Long term follow up confirms that surgery in CCTGA with the right ventricle as systemic ventricle has a suboptimal survival and limited freedom of reoperation. Death occurred mostly as a result of cardiac failure.

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Figures

Figure 1
Figure 1
Kaplan-Meier survival after surgery. A) Overall survival. B) Survival split by non-Rastelli and Rastelli surgery. Between brackets the number of patients at risk.
Figure 2
Figure 2
Freedom of reoperation after primary surgery. A). Overall freedom of reoperation. B) Freedom of reoperation split by non-Rastelli and Rastelli surgery. Between brackets the number of patients at risk.
See this image and copyright information in PMC

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