Pulmonary hypertension associated with thalassemia syndromes

Dustin R. Fraidenburg, Roberto F. Machado

Research output: Contribution to journalArticle

21 Scopus citations

Abstract

Chronic hemolytic anemia has increasingly been identified as an important risk factor for the development of pulmonary hypertension (PH). Within the thalassemia syndromes, there are multiple mechanisms, both distinct and overlapping, by which PH develops and that differ among β-thalassemia major or intermedia patients. PH in β-thalassemia major correlates with the severity of hemolysis, yet in patients whose disease is well treated with chronic transfusion therapy, the development of PH can be related to cardiac dysfunction and the subsequent toxic effects of iron overload rather than hemolysis. β-Thalassemia intermedia, on the other hand, has a higher incidence of PH owing to the low level of hemolysis that exists over years without the requirement for frequent transfusions, while splenectomy is shown to play an important role in both types. Standard therapies such as chronic transfusion have been shown to mitigate PH, and appropriate chelation therapy can avoid the toxic effects of iron overload, yet is not indicated in many patients. Limited evidence exists for the use of pulmonary vasodilators or other therapies, such as l-carnitine, to treat PH associated with thalassemia. Here, we review the most recent findings regarding the pathogenic mechanisms, epidemiology, presentation, diagnosis, and treatment of PH in thalassemia syndromes.

Original languageEnglish (US)
Pages (from-to)127-139
Number of pages13
JournalAnnals of the New York Academy of Sciences
Volume1368
Issue number1
DOIs
StatePublished - Mar 1 2016
Externally publishedYes

Keywords

  • Chronic hemolytic anemia
  • Pulmonary hypertension
  • Thalassemia

ASJC Scopus subject areas

  • Neuroscience(all)
  • Biochemistry, Genetics and Molecular Biology(all)
  • History and Philosophy of Science

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