Pulmonary hypertension: An increasingly recognized complication of hereditary hemolytic anemias and HIV infection

Christopher F. Barnett, Priscilla Y. Hsue, Roberto F. Machado

Research output: Contribution to journalArticle

72 Scopus citations


Modern health care has greatly increased longevity for patients with congenital hemolytic anemias (such as sickle cell disease and thalassemia) and human immunodeficiency virus (HIV) infection. It is estimated that 10% of patients with hemoglobinopathies and 0.5% of patients with HIV infection develop moderate to severe pulmonary hypertension. Pulmonary hypertension is a relentlessly progressive disease leading to right heart failure and death. Worldwide, there are an estimated 30 million patients with sickle cell disease or thalassemia and 40 million patients with HIV disease. Considering the prevalence of pulmonary vascular disease in these populations, sickle cell disease and HIV disease may be the most common causes of pulmonary hypertension worldwide. In this review, the available data on epidemiology, hemodynamics, mechanisms, and therapeutic strategies for these diseases are summarized. Because therapy is likely to reduce morbidity and prolong survival, efforts to screen, diagnose, and treat these patients represent a global health opportunity.

Original languageEnglish (US)
Pages (from-to)324-331
Number of pages8
JournalJAMA - Journal of the American Medical Association
Issue number3
StatePublished - Jan 23 2008


ASJC Scopus subject areas

  • Medicine(all)

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