title: Experience with repair of complete atrioventricular canal.
abstract: Twenty-nine patients at out institution have undergone repair of complete atrioventricular canal since 1969. There were 3 operative and 5 late deaths, 4 of which were of infectious etiology. Age at operation ranged from 2 months to 12 years (mean, 50 months). Weight ranged from 3.6 kg to 30 kg (mean, 12 kg). Before repair, catheterization studies revealed pulmonary hypertension in all patients with unobstructed pulmonary arteries. Pulmonary to systemic flow ratio ranged from 1.5 to 10.6 (mean, 3.5). Pulmonary vascular resistance (PVR) ranged from 0.7 to 21.7 (mean, 5.3) Wood units/m2. At repair, 14 patients had Rastelli type A anatomy, 14 had type C, and 1 patient had a variant with crossing chordae and double-outlet right ventricle (DORV). Postoperative catheterization has been done in 16 patients 2 months to 8 years (mean, 30 months) after repair. One patient had residual ventricular shunting and later underwent successful repair. A 2-year-old patient had severe mitral regurgitation and died following mitral valve replacement. One patient required a permanent pacemaker. The 21 surviving patients have been followed from 7 months to 10 years 7 months and have excellent hemodynamic status. Long-term studies are needed to assess the ultimate effect on patients with high PVR.
mesh_terms: D006328:Cardiac Catheterization; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006325:Heart Atria; D006343:Heart Septal Defects / Q000401:mortality / Q000601:surgery*; D006352:Heart Ventricles; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D007223:Infant; D008297:Male; D011651:Pulmonary Artery / Q000503:physiopathology; D014655:Vascular Resistance