An Official American Thoracic Society Clinical Practice Guideline: Diagnosis, Risk Stratification, and Management of Pulmonary Hypertension of Sickle Cell Disease

• Published in: American journal of respiratory and critical care medicine Vol. 189; no. 6; pp. 727 - 740
• Main Authors: KLINGS, Elizabeth S, MACHADO, Roberto F, ROSENZWEIG, Erika B, SOOD, Namita, HSU, Lewis, WILSON, Kevin C, TELEN, Marilyn J, DECASTRO, Laura M, KRISHNAMURTI, Lakshmanan, STEINBERG, Martin H, BADESCH, David B, GLADWIN, Mark T, BARST, Robyn J, MORRIS, Claudia R, MUBARAK, Kamal K, GORDEUK, Victor R, KATO, Gregory J, ATAGA, Kenneth I, GIBBS, J. Simon, CASTRO, Oswaldo
• Format: Journal Article
• Language: English
• Published: New York, NY American Thoracic Society 15.03.2014
• Subjects: Adult; American Thoracic Society Documents; Anemia, Sickle Cell - complications; Anemia, Sickle Cell - drug therapy; Anemia, Sickle Cell - mortality; Anemias. Hemoglobinopathies; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Anticoagulants - therapeutic use; Antihypertensive Agents - therapeutic use; Antisickling Agents - therapeutic use; Biological and medical sciences; Cardiac Catheterization; Cardiology; Clinical medicine; Clinical practice guidelines; Decision making; Decision Support Techniques; Diseases of red blood cells; Echocardiography, Doppler; Erythrocyte Transfusion; Heart; Hematologic and hematopoietic diseases; Hemoglobin; Humans; Hydroxyurea - therapeutic use; Hypertension, Pulmonary - diagnosis; Hypertension, Pulmonary - etiology; Hypertension, Pulmonary - mortality; Hypertension, Pulmonary - therapy; Intensive care medicine; Internal medicine; Intubation; Medical sciences; Mortality; Pediatrics; Peptides; Phosphodiesterase 5 Inhibitors - therapeutic use; Pneumology; Pulmonary arteries; Pulmonary hypertension; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Respiratory system : syndromes and miscellaneous diseases; Risk Assessment; Risk factors; Severity of Illness Index; Sickle cell disease; Veins & arteries; United States; North America; America; United States > US; Intensive care; Sickle cell anemia; Prognosis; American; Cardiovascular disease; Hemopathy; Epidemiology; sickle cell disease; Hemolytic anemia; Dyspnea; Diagnosis; Stratification; Resuscitation; Hemolysis; Hemoglobinopathy; Lung disease; Respiratory disease; Mortality; Thorax; Pulmonary hypertension; Recommendation; Genetic disease; Professional practice; Society; Risk management
• ISSN: 1073-449X; 1535-4970
• DOI: 10.1164/rccm.201401-0065st

In adults with sickle cell disease (SCD), an increased tricuspid regurgitant velocity (TRV) measured by Doppler echocardiography, an increased serum N-terminal pro-brain natriuretic peptide (NT-pro-BNP) level, and pulmonary hypertension (PH) diagnosed by right heart catheterization (RHC) are independent risk factors for mortality. A multidisciplinary committee was formed by clinician-investigators experienced in the management of patients with PH and/or SCD. Clinically important questions were posed, related evidence was appraised, and questions were answered with evidence-based recommendations. Target audiences include all clinicians who take care of patients with SCD. Mortality risk stratification guides decision making. An increased risk for mortality is defined as a TRV equal to or greater than 2.5 m/second, an NT-pro-BNP level equal to or greater than 160 pg/ml, or RHC-confirmed PH. For patients identified as having increased mortality risk, we make a strong recommendation for hydroxyurea as first-line therapy and a weak recommendation for chronic transfusions as an alternative therapy. For all patients with SCD with elevated TRV alone or elevated NT-pro-BNP alone, and for patients with SCD with RHC-confirmed PH with elevated pulmonary artery wedge pressure and low pulmonary vascular resistance, we make a strong recommendation against PAH-specific therapy. However, for select patients with SCD with RHC-confirmed PH who have elevated pulmonary vascular resistance and normal pulmonary capillary wedge pressure, we make a weak recommendation for either prostacyclin agonist or endothelin receptor antagonist therapy and a strong recommendation against phosphodiesterase-5 inhibitor therapy. Evidence-based recommendations for the management of patients with SCD with increased mortality risk are provided, but will require frequent reassessment and updating.