SURGICAL-MANAGEMENT OF ATRIO VENTRICULAR SEPTAL-DEFECTS WITH NORMAL KARYOTYPE

Objective: Atrio ventricular septal defects (AVSD) with normal caryoty pe represent in average 25% of AVSD. They constitute a more complex gr oup of patients characterized by frequent left sided heart obstructive lesions, raising the problem of the appropriate indications between b iventricular and univentricular procedures. Methods: Sixty-nine consec utive patients, who had AVSD with normal caryotype underwent surgery. According to the anatomical complexity there were 22 intermediate AVSD , 36 complete AVSD and II complex AVSD. Associated lesions were presen t in 68% of the patients including left sided heart anomalies in 57%. According to the size of the left ventricle (LV) evaluated on the LV/R V end diastolic diameter ratio calculated at 2D echocardiogram: right ventricular (RV) dominance was found in 29%; with border line LV in 13 patients and truly hypoplastic LV in 7 patients. Biventricular repair was always favored in case of border line LV and precluded when the L V/RV ratio was less than 0.33. Results: There were 57 biventricular re pairs with 10 years actuarial survival of 70 +/- 8% and respectively, 100% in the complex AVSD, 86% in the intermediate AVSD and 51% in the complete AVSD (P = 0.006). The risk factors for biventricular repair w ere the association to a subaortic stenosis (P = 0.01) and the severit y of the mitral valve lesions (P = 0.03) that led to 38% reoperation. There were 12 univentricular palliation with 10 years survival of 66 /- 14%. The risk factor for univentricular palliation was the associat ion to a severe pre-operative mitral regurgitation (P = 0.005). Conclu sions: biventricular repair should be precluded in patients presenting with subaortic stenosis. Severe mitral valve anomalies lead to elevat ed mortality and morbidity with frequent reoperations. Univentricular repair might have larger indications and cardiac transplantation might be considered in patients with truly hypoplastic LV presenting with s evere pre-operative AV valve regurgitation. (C) 1997 Elsevier Science B.V.