Long-term response to calcium-channel blockers in non-idiopathic pulmonary arterial hypertension

Eur Heart J. 2010 Aug;31(15):1898-907. doi: 10.1093/eurheartj/ehq170. Epub 2010 Jun 11.


Aims: To assess the acute vasodilator response and long-term response to calcium-channel blockers (CCB) in pulmonary arterial hypertension (PAH) with associated conditions.

Methods and results: The response to acute vasodilator testing [>20% decrease in mean pulmonary artery pressure (mPAP) and total pulmonary resistance] was assessed in 663 consecutive PAH patients with connective tissue disease (CTD; n = 168), portal hypertension (PoPH; n = 153), anorexigen use (n = 127), human immunodeficiency virus infection (HIV; n = 124), congenital heart disease (CHD; n = 50), and pulmonary veno-occlusive disease or capillary haemangiomatosis (PVOD/PCH; n = 41). An acute vasodilator response was observed in 13.4% of PAH-anorexigen patients, 12.2% of PVOD/PCH, 10.1% of CTD, 1.6% of HIV, 1.3% of PoPH, and was absent in CHD. A long-term response to CCB (marked haemodynamic improvement at 3-4 months and New York Heart Association functional class I or II after 1 year) was reported in 9.4% of PAH-anorexigen patients but was rare in HIV, PoPH, CTD (1.6, 0.7, and 0.6%, respectively) and absent in PVOD/PCH. All patients with a long-term CCB response were alive after 5 years; two deaths not related to PAH occurred after this time. Recent criteria for acute response based on the fall in mPAP to <40 mmHg are more specific to detect long-term responders to CCB.

Conclusion: A long-term CCB response was reported in patients with PAH associated with anorexigen use, but was rare in patients with PoPH or HIV and absent in PVOD/PCH, CHD, and the vast majority of CTD. The prognosis of long-term responders was favourable and related to the underlying cause of PAH.

MeSH terms

  • Adult
  • Aged
  • Antihypertensive Agents / therapeutic use*
  • Appetite Depressants / adverse effects*
  • Calcium Channel Blockers / pharmacology*
  • Female
  • Humans
  • Hypertension, Pulmonary / chemically induced
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / physiopathology
  • Long-Term Care
  • Male
  • Middle Aged
  • Pulmonary Artery / physiopathology
  • Vascular Resistance / drug effects
  • Vasodilation / drug effects
  • Vasodilator Agents / pharmacology*
  • Young Adult


  • Antihypertensive Agents
  • Appetite Depressants
  • Calcium Channel Blockers
  • Vasodilator Agents