Pulmonary hypertension in hemolytic disorders

Pulmonary vascular disease: The global perspective

Roberto Machado, Mark T. Gladwin

Research output: Contribution to journalReview article

45 Citations (Scopus)

Abstract

The inherited hemoglobin disorders sickle cell disease and thalassemia are the most common monogenetic disorders worldwide. Pulmonary hypertension is one of the leading causes of morbidity and mortality in adult patients with sickle cell disease and thalassemia, and hemolytic disorders are potentially among the most common causes of pulmonary hypertension. The pathogenesis of pulmonary hypertension in hemolytic disorders is likely multifactorial, including hemolysis, impaired nitric oxide (NO) bioavailability, chronic hypoxemia, chronic thromboembolic disease, chronic liver disease, and asplenia. In contrast to patients with traditional forms of pulmonary arterial hypertension, patients with hemolytic disorders have a mild-to-moderate degree of elevation in mean pulmonary pressures, with mild elevations in pulmonary vascular resistance. The hemodynamic etiology of pulmonary hypertension in these patients is multifactorial and includes pulmonary arterial hypertension, pulmonary venous hypertension, and pulmonary hypertension secondary to a hyperdynamic state. Currently, there are limited data on the effects of any specific treatment modality for pulmonary hypertension in patients with hemolytic disorders. It is likely that maximization of treatment of the primary hemoglobinopathy in all patients and treatment with selective pulmonary vasodilators and antiproliferative agents in patients with pulmonary arterial hypertension would be beneficial. However, there is still a major need for large multinational trials of novel therapies for this patient population.

Original languageEnglish (US)
JournalChest
Volume137
Issue number6 SUPPL.
DOIs
StatePublished - Jun 1 2010
Externally publishedYes

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Vascular Diseases
Pulmonary Hypertension
Lung Diseases
Thalassemia
Sickle Cell Anemia
Chronic Disease
Hemoglobinopathies
Lung
Therapeutics
Hemolysis
Vasodilator Agents
Vascular Resistance
Biological Availability
Liver Diseases
Nitric Oxide
Hemoglobins
Hemodynamics
Morbidity
Pressure
Mortality

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine

Cite this

Pulmonary hypertension in hemolytic disorders : Pulmonary vascular disease: The global perspective. / Machado, Roberto; Gladwin, Mark T.

In: Chest, Vol. 137, No. 6 SUPPL., 01.06.2010.

Research output: Contribution to journalReview article

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