Impact of sildenafil therapy on pulmonary arterial hypertension in adults with congenital heart disease

Cardiovasc Ther. 2010 Dec;28(6):350-5. doi: 10.1111/j.1755-5922.2010.00213.x.

Abstract

Background: It has been demonstrated that sildenafil is effective in patients with pulmonary arterial hypertension (PAH). However, the impact of sildenafil on PAH in adults with congenital heart disease (CHD) has been less investigated.

Objective: In this prospective, open-label, uncontrolled and multicenter study, 60 patients with PAH related to CHD received oral sildenafil (75 mg/day) for 12 weeks. The enrolled patients underwent six-minute walk test (SMWT) and cardiac catheterization at the beginning and the end of the 12 weeks. The primary end point was the changes in exercise capacity assessed by SMWT; the secondary end point included assessment of functional class, evaluation of cardiopulmonary hemodynamics, and clinical worsening (defined as death, transplantation, and rehospitalization for PAH). Drug safety and tolerability were also examined.

Results: Oral sidenafil significantly increased SMWT distances (422.94 ± 76.95 m vs. 371.99 ± 78.73 m, P < 0.0001). There was also remarkable improvement in Borg dyspnea score (2.1 ± 1.32 vs. 2.57 ± 1.42, P = 0.0307). Moreover, significant improvements in World Healthy Organization (WHO) functional class and cardiopulmonary hemodynamics were also discovered (mean pulmonary artery pressure, P = 0.0002; cardiac index, P < 0.0001; pulmonary vascular resistance, P < 0.0001). Side effects in this study were mild and consistent with reported studies. None of the enrolled patients experienced significant clinical worsening.

Conclusions: This study confirmed and extended previous studies. It suggested that oral sildenafil was safe and effective for the treatment of adult patients with CHD-related PAH.

Publication types

  • Clinical Trial
  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Administration, Oral
  • Adolescent
  • Adult
  • Antihypertensive Agents / administration & dosage
  • Antihypertensive Agents / adverse effects
  • Antihypertensive Agents / therapeutic use*
  • Blood Pressure / drug effects*
  • Cardiac Catheterization
  • China
  • Drug Administration Schedule
  • Dyspnea / drug therapy
  • Dyspnea / etiology
  • Dyspnea / physiopathology
  • Exercise Test
  • Exercise Tolerance / drug effects
  • Familial Primary Pulmonary Hypertension
  • Female
  • Heart Defects, Congenital / complications*
  • Heart Defects, Congenital / physiopathology
  • Humans
  • Hypertension, Pulmonary / drug therapy
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / physiopathology
  • Male
  • Piperazines / administration & dosage
  • Piperazines / adverse effects
  • Piperazines / therapeutic use*
  • Prospective Studies
  • Purines / administration & dosage
  • Purines / adverse effects
  • Purines / therapeutic use
  • Sildenafil Citrate
  • Sulfones / administration & dosage
  • Sulfones / adverse effects
  • Sulfones / therapeutic use*
  • Time Factors
  • Treatment Outcome
  • Vascular Resistance / drug effects
  • Vasodilator Agents / administration & dosage
  • Vasodilator Agents / adverse effects
  • Vasodilator Agents / therapeutic use*
  • Young Adult

Substances

  • Antihypertensive Agents
  • Piperazines
  • Purines
  • Sulfones
  • Vasodilator Agents
  • Sildenafil Citrate