Pulmonary hypertension associated with sickle cell disease
- Elizabeth S Klings, MD
Elizabeth S Klings, MD
- Associate Professor of Medicine
- Boston University School of Medicine
- Harrison W Farber, MD
Harrison W Farber, MD
- Professor of Medicine
- Boston University School of Medicine
- Section Editors
- Stanley L Schrier, MD
Stanley L Schrier, MD
- Editor-in-Chief — Hematology
- Section Editor — Myeloproliferative Disorders; Red Cell Disorders
- Professor of Medicine
- Stanford University School of Medicine
- Jess Mandel, MD
Jess Mandel, MD
- Section Editor — Pulmonary Vascular Disease
- Professor of Medicine
- University of California, San Diego
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 "Classification and prognosis 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 "Classification and prognosis pulmonary hypertension in adults", section on 'Classification'.)
●Group 1 – PAH
●Group 2 – PH due to left heart disease
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- CLINICAL PRESENTATION
- SCREENING AND RISK STRATIFICATION
- EVALUATION FOR PH
- Supportive care and treatment of comorbidities
- SCD-specific treatments
- Long-term anticoagulation
- Pulmonary vasodilator therapy
- SUMMARY AND RECOMMENDATIONS