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Fifty-eight patients with ventricular septal defect (VSD) associated with severe pulmonary hypertension (Pp/Ps>_0.90) were repaired between 1971 and 1992. Their preoperative Pp/Ps, Rp/Rs and Rp were 0.98± 0.06, 2.37± 1.20 and 4.81 ± 3.06 units • m2, respectively. Late results were analyzed in 56 operative survivors. The age at the time of operation ranged from 2 months to 32 years (average 4.1 years) and the postoperative follow-up period ranged from 1 month to 20 years (average 5.5 years). Eighty-two percent of the patients were in New York Heart Association functional class I, 15% were in class II and 3% in class III. The postoperative Pp/Ps and Rp/Rs significantly decreased to 0.41 ± 0.13 (p < 0.001) and 0.25 ±0.16 (p <0.001), respectively. There were significant differences in Rp/Rs and Rp between the patients operated on before (Group 1) and after 2 years of age (Group 2). Rp/Rs and Rp in Group 1 were 0.17± 0.06 and 2.52±0.65 units • m2, whereas 0.31± 0.19 (p <0.05) and 4.26± 1.88 units. m2 (p <0.05) in Group 2, respectively. One patient died 14 months after VSD closure due to respiratory failure. It is concluded that a patient with VSD associated with severe but reversible pulmonary hypertension should be surgically corrected before 2 years of age. ventricular septal defect; pulmonary hypertension; pulmonary vascular disease; oxygen inhalation test Recent advances in operative techniques, and in pre-and postoperative patient management have allowed safe surgical closure of ventricular septal defect (VSD) with a low mortality rate. However, progress of pulmonary vascular disease (PVD) and late death due to PVD after correction of heart defects have been reported in patients with severe pulmonary hypertension (Allen et al. 1974;Friedli et al. 1974;Weidman and DuShane 1974;Moller et al. 1991). Therefore, this study was conducted to analyze the late results with respect to hemodynamics, physical activities, quality of school or social life and late mortality after correction of VSD with pulmonary hypertension equal to or greater than 0.9 of peak pulmonary to systemic arterial pressure ratio (Pp/Ps).

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