Effects of long-term sildenafil treatment for pulmonary hypertension in infants with chronic lung disease

J Pediatr. 2009 Mar;154(3):379-84, 384.e1-2. doi: 10.1016/j.jpeds.2008.09.021. Epub 2008 Oct 31.


Objective: 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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Blood Pressure / drug effects
  • Bronchopulmonary Dysplasia / complications*
  • Echocardiography
  • Female
  • Hernias, Diaphragmatic, Congenital*
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / etiology*
  • Infant
  • Infant, Newborn
  • Infant, Premature
  • Male
  • Phosphodiesterase Inhibitors / administration & dosage*
  • Phosphodiesterase Inhibitors / adverse effects
  • Piperazines / administration & dosage*
  • Piperazines / adverse effects
  • Pulmonary Artery / drug effects
  • Purines / administration & dosage
  • Purines / adverse effects
  • Retrospective Studies
  • Sildenafil Citrate
  • Sulfones / administration & dosage*
  • Sulfones / adverse effects
  • Treatment Outcome


  • Phosphodiesterase Inhibitors
  • Piperazines
  • Purines
  • Sulfones
  • Sildenafil Citrate