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Updated: Jun 25, 2025

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
[Heritable pulmonary arterial hypertension]
Christina A Eichstaedt1,2, Memoona Shaukat3,4, Ekkehard Grünig3
1Zentrum für Pulmonale Hypertonie, Thoraxklinik am Universitätsklinikum Heidelberg, Röntgenstraße 1, 69126, Heidelberg, Deutschland. christina.eichstaedt@med.uni-heidelberg.de.
Abstract:
Heritable pulmonary arterial hypertension (PAH) can be triggered by at least 18 genes. The most frequently altered gene is the bone morphogenetic protein receptor 2 (BMPR2). Further genes from the same pathway are also well known PAH-causing genes. Genetic testing can aid to confirm differential diagnoses such as a pulmonary veno-occlusive disease. It also enables the testing of healthy family members. In addition to the PAH patient population particularly served by genetic testing, this article touches on the mode of inheritance and provides insights into the first treatments soon on the market that rebalance the BMPR2 signaling pathway.
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