PULMONARY-HYPERTENSION IN PATIENTS WITH COMPLETE TRANSPOSITION OF THEGREAT-ARTERIES - MIDTERM RESULTS AFTER SURGERY

Postoperative results of surgical repair of complete transposition of the great arteries (TGA) with pulmonary hypertension (PH) in 19 patien ts in whom mean pulmonary arterial pressure was >50 mmHg or the pulmon ary/systemic arterial pressure ratio was >0.8 were examined, TGA with intact ventricular septum was diagnosed in 10 patients and TGA with ve ntricular septal defect in 9. At the time of corrective surgery (arter ial switch 10, atrial switch 9), patients ranged in age from 7 months to 14 years (mean 2.4 years), Seventeen patients (89%) survived with N ew York Heart Association functional class I, Seven patients in whom c alculated pulmonary vascular resistance was 10-20 U . m(2) after surge ry survived with subsequent regression of PH. Residual PH was diagnose d in 36% of survivors, Two patients in whom pulmonary vascular resista nce was 26 and 36 U . m(2), respectively, died after surgery, In patie nts with TGA, severe PH was not necessarily fatal and was potentially reversible after successful surgery.