Abstract
Aim of the studyTo 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.MethodsA 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.ResultsExcluding 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.ConclusionsLong 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.
Highlights
Corrected transposition of the great arteries (CCTGA) is a rare cardiac anomaly with an incidence of less than 1% of patients with congenital heart disease [1]
The right atrium is connected to the morphologically left ventricle, which connects to the pulmonary artery and the left atrium is connected to the morphologically right ventricle, which connects to the aorta, resulting in atrio-ventricular discordance and ventriculo-arterial discordance, or double discordance [2]
The prognosis of patients with corrected transposition of the great arteries (CCTGA) is variable with some patients showing satisfactory long-term survival
Summary
Corrected transposition of the great arteries (CCTGA) is a rare cardiac anomaly with an incidence of less than 1% of patients with congenital heart disease [1]. In 90% of these patients associated anomalies are present as well, with ventricular septal defect as the most common, followed by pulmonary stenosis and atrial septal defect [1,3,4,5]. The prognosis of patients with CCTGA is variable with some patients showing satisfactory long-term survival [1,5,6,7]. Both deteriorating right ventricular function on the long-term, as well as associated anomalies have an adverse effect on outcome. For instance a ventricular septal defect, pulmonary stenosis or arrhythmia have been found to limit the prognosis [6,8]
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