Operative results in patients with pseudotruncus arteriosus.
Total repair was carried out in 18 patients with pseudotruncus arteriosus. Age at the time of operation ranged from 1 to 25 years and averaged 9 years. Operative methods were transannular patch reconstruction for the relief of pulmonary atresia in 4 patients (no deaths) and reconstruction with a valved external conduit from the right ventricle to the pulmonary arteries in 14 patients (5 deaths). The overall operative mortality was 28%. An average cross-sectional area of the right and left pulmonary arteries (PA area) was calculated using angiograms and compared with the cross-sectional area of the normal right pulmonary artery (N-rPA area) in all patients. The PA area ranged from 0.06 to 2.60 cm2 and averaged 1.02 cm2; the PA area/N-rPA area ratio ranged from 0.03 to 1.05, with an average of 0.54. Three patients who died of hypoplastic right and left pulmonary arteries had a PA area/N-rPA area ratio less than 0.20. These patients had a right-to-left ventricular peak systolic pressure ratio (pRV/LV) greater than 1.0 after total repair. Fifteen patients had a PA area/N-rPA area ratio greater than 0.20, and 13 survived corrective procedures. One patient died of lung edema, which was due to failure to ligate the large bronchial collateral artery properly, and the other died of pulmonary vascular obstructive disease. Our results show that a PA area/N-rPA area ratio greater than 0.20 is a necessity in performing total repair of pseudotruncus arteriosus.
D000293:Adolescent; D000328:Adult; D001794:Blood Pressure; D002648:Child; D002675:Child, Preschool; D006352:Heart Ventricles / Q000601:surgery; D006801:Humans; D007223:Infant; D008722:Methods; D011651:Pulmonary Artery / Q000473:pathology / Q000503:physiopathology; D011652:Pulmonary Circulation; D011664:Pulmonary Valve / Q000601:surgery; D013771:Tetralogy of Fallot / Q000503:physiopathology / Q000601:surgery*