What is the role of transplantation and septostomy for the treatment of idiopathic pulmonary arterial hypertension (IPAH)?

Updated: Jul 08, 2020
  • Author: Ronald J Oudiz, MD, FACP, FACC, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Answer

A single- or double-lung transplant is indicated for patients who do not respond to medical therapy. Simultaneous cardiac transplantation may not be necessary even with severe right ventricular dysfunction; however, this depends on the transplant institution. Interestingly, IPAH is not thought to recur after transplant.

Go to Pediatric Lung Transplantation for more complete information on this topic.

Atrial septostomy is a palliative procedure that may afford some benefit to patients whose condition is deteriorating. This procedure works by allowing interatrial right-to-left shunting to occur, thus delivering more overall oxygen content to the respiring tissues, albeit with a lower overall saturation.


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