THE MANAGEMENT OF TETRALOGY OF FALLOT WITH PULMONARY ATRESIA AND DIMINUTIVE PULMONARY-ARTERIES

Since September 1991, 14 consecutive patients with tetralogy of Fallot , pulmonary atresia, and diminutive pulmonary arteries have undergone staged repair, All patients had multiple aortopulmonary collateral art eries and the ductus arteriosus was absent in 11. Mean sizes of the ri ght and left pulmonary arteries were 2.2 +/- 0.7 mm and 1.9 +/- 0.8 mm , respectively (range 0.5 to 3.0 mm). Eight patients (57%) have subseq uently received complete repair. Age at initial procedure (shunt, righ t ventricle-pulmonary artery conduit, or direct aorta-pulmonary artery anastomosis) in this group aas 5.3 +/- 6.8 months. The number of oper ative procedures to achieve complete repair was 2.9 +/- 0.8 per patien t (range 2 to 4), Intraoperative postrepair peak right ventricle-left ventricle pressure ratio was 0.57 +/- 0.17, Six of 8 patients (75%) re quired additional interventional procedures (mean 1.5 +/- 1.2 per pati ent) for angioplasty of peripheral pulmonary artery stenoses, coil emb olization of aortopulmonary collateral arteries, or intraoperative ins ertion of intravascular pulmonary artery stents, Mean follow-up from c omplete repair was 8.7 +/- 8.3 months (range 0.5 to 23.8 months) and i s complete. There was one in-hospital death at 45 days, and one late c ardiac death at 20.3 months, Six patients had initial palliative opera tions (unifocalization, right ventricle-pulmonary artery conduit, dire ct aorta-pulmonary artery anastomosis, or transannular outflow patch) but have not undergone complete repair. Age at initial procedure in th is group was 27.9 +/- 56.9 months (range 0.27 to 155 months), and mean follow-up from initial procedure was 10.9 +/- 11.2 months (range 0 to 31.4 months). The operative mortality rate was 33% (2 of 6 patients). There was one late noncardiac death at 5.3 months, Three patients are awaiting further intervention or repair, This experience suggests tha t complete repair is feasible even in patients with extremely diminuti ve pulmonary arteries (less than or equal to 3.0 mm). Pulmonary artery growth is facilitated by early (3 to 6 month) establishment of centra l pulmonary artery how by right ventricle-pulmonary artery conduit (pu lmonary arteries >1.5 mm) or by direct ascending aorta-pulmonary arter y anastomosis (pulmonary arteries <1.5 mm), Subsequent interventional catheterization and operative procedures as required for pulmonary art ery stenoses and coil embolization of collateral arteries allow contin ued recruitment of central pulmonary arteries and may obviate or minim ize the need for unifocalization procedures.