Pregnancy as a Possible Trigger for Heritable Pulmonary Arterial Hypertension. (September 2016)

• Record Type: Journal Article
• Authors: Limoges, Maude; Langleben, David; Fox, Benjamin D.; Shear, Roberta; Wieczorek, Paul; Rudski, Lawrence G.; Hirsch, Andrew M.; Schlesinger, Robert D.; Lesenko, Lyda
• Is Part Of: Pulmonary circulation. Volume 6:Number 3(2016)
• Page Start: 381
• Page End: 383
• Publication Date: 2016-09
• Subjects: pulmonary arterial hypertension -- pregnancy -- right ventricle -- BMPR2; Pulmonary circulation -- Periodicals; Pulmonary circulation; Electronic journals -- Sciences; Periodicals; 616.24005
• DOI: 10.1086/686993
• Languages: English
• ISSNs: 2045-8932

Abstract

It is unclear whether pregnancy is a trigger or accelerant for idiopathic pulmonary arterial hypertension (PAH). Alternatively, its frequency close to the onset of symptoms and diagnosis in the idiopathic PAH population may represent a coincidence in a disease that predominates in young women. We describe a carrier of a BMPR2 gene mutation who had an uneventful first pregnancy but had aggressive PAH during her second pregnancy and now has ongoing heritable PAH. The possible role of pregnancy as a trigger in this vulnerable patient is discussed. Databases of patients with heritable PAH should be explored to see whether pregnancy is related to overt manifestation of the disease.