Pulmonary arterial hypertension in women.
BACKGROUND
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.


CONCLUSIONS
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.
Service de pneumologie et soins intensifs, hôpital européen Georges-Pompidou, Assistance publique-Hôpitaux de Paris, faculté de médecine, université Paris-Descartes, 20, rue Leblanc, 75015 Paris, France. olivier.sanchez@egp.aphp.fr
O
O
Sanchez
E
E
Marié
U
U
Lerolle
D
D
Wermert
D
D
Israël-Biet
G
G
Meyer
D000032:Abortion, Therapeutic; D000328:Adult; D000925:Anticoagulants / Q000627:therapeutic use; D052006:Bone Morphogenetic Protein Receptors, Type II / Q000235:genetics; D000077300:Bosentan; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D003267:Contraception; D004417:Dyspnea / Q000209:etiology; D065130:Endothelin A Receptor Antagonists; D005260:Female; D005317:Fetal Growth Retardation / Q000209:etiology; D020022:Genetic Predisposition to Disease; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics / Q000503:physiopathology / Q000628:therapy; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000188:drug therapy / Q000503:physiopathology / Q000601:surgery; D019836:Preimplantation Diagnosis; D011379:Prognosis; D044062:Prostaglandins I / Q000627:therapeutic use; D017678:Sex Distribution; D013449:Sulfonamides / Q000627:therapeutic use; D014463:Ultrasonography
D000925:Anticoagulants; D002121:Calcium Channel Blockers; D065130:Endothelin A Receptor Antagonists; D010726:Phosphodiesterase Inhibitors; D044062:Prostaglandins I; D013449:Sulfonamides; C498218:BMPR2 protein, human; D052006:Bone Morphogenetic Protein Receptors, Type II; D000077300:Bosentan