294: Outcome of patients with “infantile partial atrioventricular septal defects” necessitating surgical repair within the first year of life

Abstract

Precise features of infants with partial and transitional atrioventricular septal (PAVC) defect requiring surgical repair remain undetermined, as well as their outcome. We retrospectively analyzed this patient population. Cases with single ventricle palliation were excluded. Since January 1 st , 2000, 11 infants (< 1 year of age) underwent surgical repair of PAVC in our institution, at a median age and weight of 7 (2 to 11) months and 6.2 (3.4 to 7.8) Kg, respectively. All patients had refractory congestive heart failure before surgery. Six patients were prenatally diagnosed and 2 had Down syndrome. Three patients had a small left atrioventricular valve (LAVV) with a Z-score diameter < − 2. One infant had and associated aortic coarctation necessitating concomitant repair. Other associated cardiac anomalies included persistent left superior vena cava and interrupted inferior vena cava. All the patients survived after surgical repair, with a median stay of 2 (1 to 21) days in the intensive care unit. One patient with a parachute LAVV was reoperated 1 month after surgery for LAVV insufficiency. After a median follow-up of 4,56 (2,6 to 12,2) years, all the patients are in NYHA class I, with no medication. Echocardiography shows moderate LAVV insufficiency in 2 cases. Surgical repair of PAVC in the first year of life is feasible with excellent mid-term outcome, even in cases with a small LAVV. Patients with “parachute like” LAVV may experience significant regurgitation necessitating reoperation.