Surgery for transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction: European Congenital Heart Surgeons Association multicentre study.

OBJECTIVES: Optimal surgical management for patients with transposition of the great arteries (TGA), ventricular septal defect (VSD) and left ventricular outflow obstruction (LVOTO) remains controversial. Although the Rastelli operation has been the most widely performed surgical procedure during the past decades, several studies have shown its suboptimal long-term prognosis. Other operations have been developed to improve results. This study was performed to compare the outcomes of the different surgical approaches for patients with TGA, VSD and LVOTO, as well as to determine risk factors for mortality and re-intervention. METHODS: Records from 146 patients undergoing surgery from 1980 to 2008 from eight European hospitals were reviewed. Median age at operation was 21.5 months (range 0.2-165.1 months), and median weight was 10.0 kg (range 2.0-41.0 kg). Surgical procedures involved were the Rastelli procedure (82), arterial (24) and atrial (5) switch operation with relief of LVOTO, Réparation à l'Etage ventriculaire (REV) procedure (7) and the Metras modification (24), as well as the Nikaidoh procedure (4). RESULTS: The overall survival was 88%, 88% and 58% at 1, 10 and 20 years, respectively. The overall event-free survival was 80%, 45% and 26% at 1, 10 and 20 years, respectively. The REV procedure and the Metras modification were found to have the best long-term results in both survival and event-free survival rates. Multivariate analysis revealed year of operation, non-commitment of the VSD and prolonged cardiopulmonary bypass (CPB) time as risk factors for mortality while age at surgery, year of operation and type of corrective surgery were risk factors for re-intervention. CONCLUSIONS: Different surgical approaches have been developed for patients with TGA, VSD and LVOTO. The REV procedure and the Metras modification were observed to have favourable long-term results in survival and event-free survival rates. Aortic translocation techniques such as the Nikaidoh procedure seem promising, but further studies will be needed to confirm this in the long term.

Left atrial isomerism: biventricular repair.

OBJECTIVE: Biventricular repair of hearts with left atrial isomerism often necessitates complex atrial and ventricular baffle procedures. We analysed our experience with an accent on baffle techniques. METHODS: From 1997 until 2008, 12 patients (four male) with left atrial isomerism received biventricular repair. Their median age at surgery was 9 (range: 1-24) months. Four patients had dextrocardia. Nine patients presented with left superior vena cava, three with absent right superior vena cava, five with unroofed coronary sinus and nine others with inferior vena cava interruption with (hemi)azygos continuation. Anomalous pulmonary venous drainage was present in three patients. Eight had a monoatrium. Atrioventricular septal defect (AVSD) occurred in six (complete AVSD in two), One patient with complete AVSD had right pulmonary agenesia with long segment tracheal stenosis. Multiple VSDs presented in one whereas three patients had double-outlet right ventricle (DORV) (one with borderline LV hypoplasia). Two had previous pulmonary artery banding. Complex intra-atrial baffle constructions were performed in seven patients. Complete AVSDs were corrected using two patches and all other AVSDs had one patch repair. Multiple VSDs were closed directly. DORV patients had intraventricular tunnel repair. RESULTS: No early mortality occurred. Median follow-up was 54 (range: 2-134) months. One patient with complete AVSD and pulmonary agenesia died late after tracheal repair. Four patients needed five re-operations (closure of residual ASD (one), relief of left (two) or right (two) ventricular outflow obstruction, pulmonary artery branch plasty (one)). There was no atrial baffle stenosis. Four received a pacemaker. All survivors are in NYHA class I. CONCLUSIONS: Survival and functional status of left isomerism patients after biventricular repair is good. Complex repairs with atrial or ventricular baffles are frequent. Arrhythmias were common and pose a concern late after repair.