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lüll Effects of long-term sildenafil treatment for pulmonary hypertension in infants with chronic lung disease Mourani PM; Sontag MK; Ivy DD; Abman SHJ Pediatr 2009[Mar]; 154 (3): 379-84, 384.e1-2OBJECTIVE: To determine the clinical course and outcomes of infants with chronic lung disease (CLD) and pulmonary hypertension (PH) who received prolonged sildenafil therapy. STUDY DESIGN: We conducted a retrospective review of 25 patients <2 years of age with CLD in whom sildenafil was initiated for the treatment of PH while they were hospitalized from January 2004 to October 2007. Hemodynamic improvement was defined by a 20% decrease in the ratio of pulmonary to systemic systolic arterial pressure or improvement in the degree of ventricular septal flattening with serial echocardiograms. RESULTS: Chronic sildenafil therapy (dose range, 1.5-8.0 mg/kg/d) was initiated at a median of 171 days of age (range, 14-673 days of age) for a median duration of 241 days (range, 28-950 days). Twenty-two patients (88%) achieved hemodynamic improvement after a median treatment duration of 40 days (range, 6-600 days). Eleven of the 13 patients with interval estimates of systolic pulmonary artery pressure with echocardiogram showed clinically significant reductions in PH. Five patients (20%) died during the follow-up period. Adverse events leading to cessation or interruption of therapy occurred in 2 patients, 1 for recurrent erections, and the other had the medication held briefly because of intestinal pneumatosis. CONCLUSION: These data suggest that chronic sildenafil therapy is well-tolerated, safe, and effective for infants with PH and CLD.|*Hernias, Diaphragmatic, Congenital[MESH]|Blood Pressure/drug effects[MESH]|Bronchopulmonary Dysplasia/*complications[MESH]|Echocardiography[MESH]|Female[MESH]|Humans[MESH]|Hypertension, Pulmonary/diagnosis/*drug therapy/*etiology[MESH]|Infant[MESH]|Infant, Newborn[MESH]|Infant, Premature[MESH]|Male[MESH]|Phosphodiesterase Inhibitors/*administration & dosage/adverse effects[MESH]|Piperazines/*administration & dosage/adverse effects[MESH]|Pulmonary Artery/drug effects[MESH]|Purines/administration & dosage/adverse effects[MESH]|Retrospective Studies[MESH]|Sildenafil Citrate[MESH]|Sulfones/*administration & dosage/adverse effects[MESH]|Treatment Outcome[MESH] |