Pulmonary artery abnormalities in tetralogy of Fallot and relation to late physical performance.

Preoperative angiograms were reviewed in 86 (52%) of 165 patients who underwent intracardiac repair of tetralogy of Fallot in 1966-1976. The median age at repair was 7 years (range 9 months-55 years). Hypoplasia, stenosis or atresia of the pulmonary and aortopulmonary collateral arteries were present in 58 patients (67%). Hypoplasia of the main pulmonary artery was associated with hypoplasia of the pulmonary artery branches. In patients who received a transannular patch the pulmonary arteries were smaller than in those without patch. Three of five pulmonary artery branch stenoses were located at the site of a previous palliative shunt. The postrepair right ventricular/left ventricular systolic pressure ratio (PRV/LV) measured in the operating room was compared with a predicted ratio based on pulmonary artery dimensions. High PRV/LV was associated with poor prognosis. In survivors the postrepair PRV/LV had fallen significantly 13-26 years later. Work capacity and pulmonary function at follow-up were not influenced by presence of pulmonary artery abnormalities. We conclude that such abnormalities, although common in patients with tetralogy of Fallot, did not per se influence physical performance in long-term survivors.