Clinical Outcomes after Anatomic Repair Including Hemi-Mustard Operation in Patients with Congenitally Corrected Transposition of the Great Arteries

BACKGROUND AND OBJECTIVES: The aims of this study were to determine the early and late outcomes of anatomic repair of congenitally corrected transposition of the great arteries (ccTGA) and to evaluate effectiveness of the hemi-Mustard procedure. SUBJECTS AND METHODS: We conducted a retrospective, single-center study of patients who underwent anatomic repair for ccTGA between July 1996 and December 2013. Sixteen patients were included in the study. The median age at the time of the operation was 3.5 years (range: 0.5-29.7), and the median body weight was 13.3 kg (range: 5.8-54). The median follow-up duration was 7.7 years (range: 0.2-17.4). RESULTS: Atrial switch was achieved using the Mustard procedure in 12 patients (hemi-Mustard procedure in 11) or the Senning procedure in four patients. The ventriculoarterial procedure was performed using the Rastelli procedure in 11 patients and arterial switch in five patients. Six patients underwent tricuspid valvuloplasty. The survival rate was 93.8±6.1%. The rate of freedom from reoperation at 5 years was 92.3±7.4% in the Rastelli group. All patients except one were New York Heart Association class I. All patients except one had mild tricuspid regurgitation. CONCLUSION: Anatomic repair can be performed with a low risk of in-hospital mortality. The hemi-Mustard strategy for selected patients is one solution for reducing early mortality and morbidity, and long-term complications such as venous pathway stenosis or sinus node dysfunction.

An Adult Case of Congenitally Corrected Transposition of the Great Arteries Associated with Paroxysmal Atrial Fibrillation and Heart Failure

Congenitally corrected transposition of the great arteries is a rate congenital heart disease. In this discase, there is discordance between both the atria and ventricles and the ventricles and great vessels. The anatomic left ventricle lies on the rightside and is connected to the pulmonic trunk, whereas the anatomic right ventricles lies on the left side and functions as the systemic ventricle. Most patients have associated another cardiac anomalies and conduction disturbance.Less commonly, ventricular extrasystoles, paroxismal supraventricular tachycardia, WPW preexitaion and atrial fibrillation may be obserced. We report an adult case of congenitally corrected transposition of great arteries associated with paroxysmal atrial fibrillation and heart failure.