Macitentan treatment of portopulmonary hypertension with hepatopulmonary syndrome: a case report and literature review

ESC Heart Fail. 2023 Aug;10(4):2718-2721. doi: 10.1002/ehf2.14431. Epub 2023 Jun 14.

Abstract

Pulmonary arterial hypertension-targeted therapies in portopulmonary hypertension (PoPH) are scarce, let alone for patients with chronic liver failure (CLF) and hepatopulmonary syndrome (HPS). A 48-year male was admitted to the hospital because of cirrhosis for 18 years, systemic oedema, and chest distress after exercise for 1 week. He was diagnosed with CLF, PoPH, and HPS. After 7 weeks of macitentan treatment, the patient's activity tolerance, pulmonary artery systolic pressure, arterial partial pressure of oxygen (PaO2 ), cTNI, and NT-proBNP changes indicated gradual recovery, without hepatic safety concerns. This case indicated that administering macitentan in patients diagnosed as PoPH (with CLF and HPS) may be efficient and safe enough in a clinical setting.

Keywords: Hepatopulmonary syndrome; Liver failure; Macitentan; Portopulmonary hypertension.

Publication types

  • Review
  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Hepatopulmonary Syndrome* / complications
  • Hepatopulmonary Syndrome* / diagnosis
  • Hepatopulmonary Syndrome* / drug therapy
  • Humans
  • Hypertension, Portal* / complications
  • Hypertension, Portal* / diagnosis
  • Hypertension, Portal* / drug therapy
  • Hypertension, Pulmonary* / complications
  • Hypertension, Pulmonary* / drug therapy
  • Male
  • Pulmonary Arterial Hypertension*

Substances

  • macitentan