Abstract

Background: Congenitally corrected transposition of the great arteries (ccTGA) is characterized by atrioventricular and ventriculoarterial discordance. The indications, timing, and type of repair vary according to the morphology of the heart, the clinical status of the patient, and the patient’s age. Optimal surgical management of patients with ccTGA remains controversial. Methods: The medical records of all patients with ccTGA who underwent definitive repairs between 1977 and 2013 were retrospectively reviewed. The surgical procedures comprised a physiologic repair in 45 patients, anatomic repair (double switch operation) in 14 patients, and a Fontan-type repair in 12 patients. Results: The Kaplan-Meier survival at 20 years was similar (75.7% in physiologic repair vs. 83.3% in anatomic repair). However, patients with significant TR showed a worse survival rate than those of the other groups (20 year survival without TVR 96.3%, but only 58.6% with TVR). The freedom from reoperation at 10 years was 78.3% in physiologic repair vs. 66.7% in anatomic repair. The freedom from pacemaker implantation at 10 years was 93.0% in physiologic repair vs. 64.3% in anatomic repair. 14 patients underwent a Fontan-type repair without mortality or heart block. Conclusions: No differences were observed between long-term survival rates of patients who underwent physiologic versus anatomic repair. The poorest outcome was seen in patients who required tricuspid valve replacement. Performing an anatomic repair should be considered for patients with significant TR. The best outcome was seen in the patients undergoing the Fontan procedure. However, various complications late after Fontan procedure remain a concern.

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