Sildenafil for pulmonary hypertension secondary to congenital heart diseases.

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.