TETRALOGY OF FALLOT WITH PULMONARY ATRESIA - REHABILITATION OF DIMINUTIVE PULMONARY-ARTERIES

Background. Patients with tetralogy of Fallot, pulmonary atresia, and diminutive pulmonary arteries are a high-risk group for whom there is no consensus on the correct approach to medical management. The purpos e of this report is to review a 14-year experience in the treatment of these patients comparing management schemes. Methods and Results. Bet ween January 1978 and August 1988, 91 patients with tetralogy of Fallo t and pulmonary atresia had an adequate evaluation of their pulmonary artery anatomy before any surgical management. Forty-eight of these pa tients had diminutive pulmonary arteries (38 to 104 mm2/m2) supplied b y aortopulmonary collaterals and were managed in four different fashio ns. Of the 9 patients repaired primarily, 7 died early and the two sur vivors had poor hemodynamic outcome. Of the 9 patients conservatively managed with no intervention before 5 years of age, 4 died and only 1 had a satisfactory hemodynamic result after repair. Of 10 shunted pati ents, 3 died and 3 had satisfactory repairs. Since 1984, we have rehab ilitated pulmonary arteries with (1) right ventricle to pulmonary arte ry surgical graft, (2) balloon dilation of residual pulmonary artery s tenoses and embolization of collaterals, and (3) surgical closure of v entricular septal defect and repair of remaining obstructions. Of 20 p atients so managed, 7 died after various stages, but 10 of 20 had comp lete repairs. All repaired patients with subsystemic right ventricular pressures had at least one successful pulmonary artery dilation. Conc lusions. A combined catheter-surgery approach begun at an early age in patients with tetralogy of Fallot and pulmonary atresia with diminuti ve pulmonary arteries appears to enhance the chances of satisfactory c omplete repair.