Macitentan Administration for Pulmonary Hypertension Due to β-thalassemia with Multiple Organ Failure

Intern Med. 2024 Jun 1;63(11):1585-1590. doi: 10.2169/internalmedicine.2307-23. Epub 2023 Nov 13.

Abstract

A 51-year-old Thai woman diagnosed with β-thalassemia underwent regular blood transfusion and iron-chelating therapy. However, after voluntarily discontinuing treatment, the patient developed progressive dyspnea and was diagnosed with pulmonary hypertension following right heart catheterization. Despite resuming blood transfusions, her condition did not improve. Because the patient had a history of multiple organ failure, curative treatment for β-thalassemia was not feasible, and macitentan was administered. Despite experiencing hypotension as an adverse event, her condition remained stable during macitentan treatment. Thus, macitentan may be well tolerated in patients with pulmonary hypertension caused by β-thalassemia with multiple organ dysfunction.

Keywords: macitentan; multiple organ failure; pulmonary hypertension; β-thalassemia.

Publication types

  • Case Reports

MeSH terms

  • Female
  • Humans
  • Hypertension, Pulmonary* / diagnosis
  • Hypertension, Pulmonary* / drug therapy
  • Hypertension, Pulmonary* / etiology
  • Middle Aged
  • Multiple Organ Failure* / diagnosis
  • Multiple Organ Failure* / etiology
  • Pyrimidines* / administration & dosage
  • Pyrimidines* / adverse effects
  • Pyrimidines* / therapeutic use
  • Sulfonamides* / administration & dosage
  • Sulfonamides* / adverse effects
  • Sulfonamides* / therapeutic use
  • Treatment Outcome
  • beta-Thalassemia* / complications
  • beta-Thalassemia* / drug therapy

Substances

  • macitentan
  • Pyrimidines
  • Sulfonamides