PULMONARY-ARTERY ABNORMALITIES IN TETRALOGY OF FALLOT AND RELATION TOLATE 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). Hypoplasi a, stenosis or atresia of the pulmonary and aortopulmonary collateral arteries were present in 58 patients (67%). Hypoplasia of the main pul monary artery was associated with hypoplasia of the pulmonary artery b ranches. In patients who received a transannular patch the pulmonary a rteries were smaller than in those without patch. Three of five pulmon ary artery branch stenoses were located at the site of a previous pall iative shunt. The postrepair right ventricular/left ventricular systol ic pressure ratio (P-RV/LV) measured in the operating room was compare d with a predicted ratio based on pulmonary artery dimensions. High P- RV/LV was associated with poor prognosis. In survivors the post-repair P-RV/LV had fallen significantly 13-26 years later. Work capacity and pulmonary function at follow-up were not influenced by presence of pu lmonary artery abnormalities. We conclude that such abnormalities, alt hough common in patients with tetralogy of Fallot, did not per se infl uence physical performance in long-term survivors.