Pulmonary arterial hypertension in women

Rev Mal Respir. 2010 Oct;27(8):e79-87. doi: 10.1016/j.rmr.2009.12.001. Epub 2010 May 13.


Introduction: Pulmonary arterial hypertension (PAH) is a rare condition characterized by sustained elevation in pulmonary arterial resistance leading to right heart failure.

Background: PAH afflicts predominantly women. Echocardiography is the initial investigation of choice for non-invasive detection of PAH but right-heart catheterization is necessary to confirm the diagnosis. Conventional treatment includes non-specific drugs (warfarin, diuretics, oxygen). The endothelin-1 receptor antagonist bosentan, the phosphodiesterase-5 inhibitor sildenafil, and prostanoids have been shown to improve symptoms, exercise capacity and haemodynamics. Intravenous prostacyclin is the first-line treatment for the most severely affected patients. Despite the most modern treatment, the overall mortality rate of pregnant women with severe PAH remains high. Therefore, pregnancy is contraindicated in women with PAH and an effective method of contraception is recommended in women of childbearing age. Therapeutic abortion should be offered, particularly when early deterioration occurs. If this option is not accepted, intravenous prostacyclin should be considered promptly.

Viewpoints and conclusion: Recent advances in the management of PAH have markedly improved prognosis and have resulted in more women of childbearing age considering pregnancy. A multidisciplinary approach should give new insights into cardiopulmonary, obstetric and anaesthetic management during pregnancy, delivery and the postpartum period.

Publication types

  • Review

MeSH terms

  • Abortion, Therapeutic
  • Adult
  • Anticoagulants / therapeutic use
  • Bone Morphogenetic Protein Receptors, Type II / genetics
  • Bosentan
  • Calcium Channel Blockers / therapeutic use
  • Contraception
  • Dyspnea / etiology
  • Endothelin A Receptor Antagonists
  • Female
  • Fetal Growth Retardation / etiology
  • Genetic Predisposition to Disease
  • Humans
  • Hypertension, Pulmonary* / classification
  • Hypertension, Pulmonary* / diagnostic imaging
  • Hypertension, Pulmonary* / drug therapy
  • Hypertension, Pulmonary* / epidemiology
  • Hypertension, Pulmonary* / genetics
  • Hypertension, Pulmonary* / physiopathology
  • Hypertension, Pulmonary* / therapy
  • Infant, Newborn
  • Male
  • Middle Aged
  • Phosphodiesterase Inhibitors / therapeutic use
  • Pregnancy
  • Pregnancy Complications, Cardiovascular / drug therapy
  • Pregnancy Complications, Cardiovascular / physiopathology
  • Pregnancy Complications, Cardiovascular / surgery
  • Preimplantation Diagnosis
  • Prognosis
  • Prostaglandins I / therapeutic use
  • Sex Distribution
  • Sulfonamides / therapeutic use
  • Ultrasonography


  • Anticoagulants
  • Calcium Channel Blockers
  • Endothelin A Receptor Antagonists
  • Phosphodiesterase Inhibitors
  • Prostaglandins I
  • Sulfonamides
  • BMPR2 protein, human
  • Bone Morphogenetic Protein Receptors, Type II
  • Bosentan