Sildenafil for pulmonary hypertension secondary to congenital heart diseases.

ABSTRACT

OBJECTIVE: The purpose of this study was to evaluate the effects of oral Sildenafil for treatment of pulmonary hypertension secondary to congenital heart diseases. METHODS & RESULTS: Twelve patients with un-repaired congenital heart diseases resulting in significant pulmonary hypertension were enrolled in a prospective trial of oral Sildenafil treatment for 12 weeks. The outcomes measured were change in the systemic oxygen saturations, 6-minute walk test distance, and New York Heart Association class. The systemic blood pressure and visual symptoms were monitored for evaluating the tolerability of Sildenafil. The mean age was 25.7+/-14.4 years. The mean pulmonary vascular resistance before treatment was 17.4 Wood units. After 12 weeks of sildenafil, an increase in oxygen saturations (87.8+/-5.3%-90.25+/-4%, p=0.04) with an associated increase of one New York Heart Association class (p=0.009) was noted. A non-statistically significant increase in the 6-minute walk test distance (310.33+/-42-333.75+/-54.6 meters, p=0.47) was also noted. Sildenafil was well-tolerated with no significant change in systemic blood pressure or visual side-effects. CONCLUSION: Oral Sildenafil is well-tolerated and produces some improvement in the oxygenation and functional status of patients with pulmonary hypertension secondary to congenital heart diseases.