EARLY CORRECTION OF COMPLETE ENDOCARDIAL CUSHION DEFECTS UTILIZING THE 2-PATCH TECHNIQUE - A 10-YEAR RETROSPECTIVE EXPERIENCE

The goal of this study was to review the short-term and long-term resu lts of aggressive corrective intervention in a consecutive series of p atients with atrioventricular canal defects, especially with respect t o minimizing progressive valvular insufficiency or pulmonary hypertens ion. A total of 46 consecutive patients with atrioventricular canal de fects had operative repair between 1981 and 1991, using a two-patch te chnique in all but 4 patients. The median age was 8.5 months, with 29 patients (63%) < 1 year old. Left-to-right shunting was severe in all cases (mean Qp/Qs = 2.9:1), with a mean systolic pulmonary artery pres sure of 63.6 mm Hg and a mean pulmonary vascular resistance of 4.03 Wo od units. Preoperatively, 35 patients (76.1%) had moderate to severe c ongestive heart failure. Hospital mortality was 6.5% (3 patients), and the systolic pulmonary artery pressure dropped significantly in all c ases, with a postrepair mean of 25.7 mm Hg. The 5 year actuarial survi val rate was 70.3%. Late echocardiographic studies graded mitral insuf ficiency as 0-2+ in 41 patients (95.2%) and 3-4+ in 2 patients (4.6%); 2 patients required reoperation, and 41 (95.2%) were New York heart A ssociation functional class I at follow-up. These data demonstrate exc ellent lat survival and functional results when complete atrioventricu lar canal correction is performed in infancy, despite significant preo perative pulmonary hypertension, valvular insufficiency, or symptoms. Prompt operative repair should be done for symptomatic patients and th ose with valvular incompetence; electrive repair is recommended before 1 year of age for most others.