An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease.

Abstract

BACKGROUND:

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.

METHODS:

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.

RESULTS:

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.

CONCLUSIONS:

Evidence-based recommendations for the management of patients with SCD with increased mortality risk are provided, but will require frequent reassessment and updating.

• Authors: Elizabeth S Klings, Roberto F Machado, Robyn J Barst, Claudia R Morris, Kamal K Mubarak, Victor R Gordeuk, Gregory J Kato, Kenneth I Ataga, J Simon Gibbs, Oswaldo Castro, Erika B Rosenzweig, Namita Sood, Lewis Hsu, Kevin C Wilson, Marilyn J Telen, Laura M Decastro, Lakshmanan Krishnamurti, Martin H Steinberg, David B Badesch, Mark T Gladwin, American Thoracic Society Ad Hoc Committee on Pulmonary Hypertension of Sickle Cell Disease
• Journal: American journal of respiratory and critical care medicine (Am J Respir Crit Care Med) Vol. 189 Issue 6 Pg. 727-40 (Mar 15 2014) ISSN: 1535-4970 [Electronic] United States
• PMID: 24628312 (Publication Type: Journal Article, Practice Guideline)

Chemical References

• Anticoagulants
• Antihypertensive Agents
• Antisickling Agents
• Phosphodiesterase 5 Inhibitors
• Hydroxyurea

Topics

• Adult
• Anemia, Sickle Cell (complications, drug therapy, mortality)
• Anticoagulants (therapeutic use)
• Antihypertensive Agents (therapeutic use)
• Antisickling Agents (therapeutic use)
• Cardiac Catheterization
• Decision Support Techniques
• Echocardiography, Doppler
• Erythrocyte Transfusion
• Humans
• Hydroxyurea (therapeutic use)
• Hypertension, Pulmonary (diagnosis, etiology, mortality, therapy)
• Phosphodiesterase 5 Inhibitors (therapeutic use)
• Risk Assessment
• Severity of Illness Index