Jornal de Pediatria
Print version ISSN 0021-7557
OLIVEIRA, Edmundo Clarindo and AMARAL, Carlos Faria Santos. Sildenafil in the management of idiopathic pulmonary arterial hypertension in children and adolescents. J. Pediatr. (Rio J.) [online]. 2005, vol.81, n.5, pp. 390-394. ISSN 0021-7557. http://dx.doi.org/10.2223/JPED.1390.
OBJECTIVE: This study aims to provide data on the use of oral sildenafil in patients in New York Heart Association functional class III or IV with severe idiopathic pulmonary arterial hypertension unresponsive to conventional therapy. METHOD: In this series, six patients with idiopathic pulmonary arterial hypertension were prospectively treated with 2 to 8 mg of oral sildenafil in four to six doses a day. All pa-tients were submitted to physical examination, electrocardiogram and echocardiogram, chest computed tomography, ventilation and pulmonary perfusion scintigraphy, coagulation studies, and tests for collagen vascular disease, acquired immune deficiency syndrome and schisto-somiasis in order to rule out secondary causes of pulmonary arterial hypertension. All patients underwent cardiac catheterization for vasoreactivity tests using nitric oxide, O2 at 100% and oral nifedipine, and a 6-minute walking test was performed in those patients who were con-sidered able to exercise. RESULTS: All patients achieved a good therapeutic response, with improvement by at least one functional class, and presented an increase in systemic arterial oxygen saturation. Five patients showed a decrease in the pulmonary systolic pressure to systemic systolic pres-sure ratio and improvement in the six-minute walking test. No major side effects were ob-served at 4 to 36 months of follow-up. One patient had sudden death after sildenafil had been withdrawn by mistake. CONCLUSIONS: These data suggest that sildenafil may be useful in the manage-ment of idiopathic pulmonary arterial hypertension. Patients should be advised against the withdrawal of sidenafil without medical supervision.
Keywords : Pulmonary arterial hypertension; phosphodiesterase inhibitors.