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Pulmonary hypertension associated with sickle cell disease

Elizabeth S Klings, MD
Harrison W Farber, MD
Section Editors
Stanley L Schrier, MD
Jess Mandel, MD
Deputy Editors
Helen Hollingsworth, MD
Jennifer S Tirnauer, MD


Sickle cell disease (SCD) encompasses a group of hemoglobinopathies characterized by amino acid substitutions in the beta globin chain. The most frequently occurring form of SCD is caused by homozygous presence of hemoglobin S (HbSS).

Pulmonary hypertension (PH) is a relatively frequent and severe complication of SCD and an independent risk factor for mortality [1-3]. The prevalence, pathogenesis, screening, and treatment of PH associated with SCD are discussed here. A discussion of other aspects of SCD and overviews of the pulmonary complications of SCD and of pulmonary hypertension are provided separately. (See "Overview of the clinical manifestations of sickle cell disease" and "Overview of the management and prognosis of sickle cell disease" and "Overview of the pulmonary complications of sickle cell disease" and "Overview of pulmonary hypertension in adults".)


The World Health Organization (WHO) classifies patients with pulmonary hypertension into five groups based upon etiology (table 1). Patients in the first group are considered to have pulmonary arterial hypertension (group 1 PAH), while patients in the remaining four groups are considered to have pulmonary hypertension (groups 2, 3, 4, and 5). When all five groups are discussed collectively, the term PH is used. SCD is placed in group 5, as there are some patients with hemodynamics consistent with PAH, while others have features of PH related to left-sided heart disease or thromboembolic disease. (See "Overview of pulmonary hypertension in adults", section on 'Classification'.)

Group 1 – PAH

Group 2 – PH due to left heart disease


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Literature review current through: Feb 2017. | This topic last updated: Fri Jul 15 00:00:00 GMT+00:00 2016.
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