An official american thoracic society clinical practice guideline: Diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease

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

Research output: Contribution to journalArticle

113 Scopus citations

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 clinicianinvestigators 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 evidencebased recommendations. Target audiences include all clinicians who take care of patients with SCD. Results: Mortality risk stratification guides decision making. An increased risk formortality is defined as a TRVequal to or greater than 2.5m/second, anNT-pro-BNPlevel 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 aweak 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 capillarywedge 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.

Original languageEnglish (US)
Pages (from-to)727-740
Number of pages14
JournalAmerican journal of respiratory and critical care medicine
Volume189
Issue number6
DOIs
StatePublished - Mar 15 2014
Externally publishedYes

Keywords

  • Dyspnea
  • Hemolysis
  • Mortality
  • Pulmonary hypertension
  • Sickle cell disease

ASJC Scopus subject areas

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

Fingerprint Dive into the research topics of 'An official american thoracic society clinical practice guideline: Diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease'. Together they form a unique fingerprint.

  • Cite this

    Klings, E. S., Machado, R. F., Barst, R. J., Morris, C. R., Mubarak, K. K., Gordeuk, V. R., Kato, G. J., Ataga, K. I., Gibbs, J. S., Castro, O., Rosenzweig, E. B., Sood, N., Hsu, L., Wilson, K. C., Telen, M. J., DeCastro, L. M., Krishnamurti, L., Steinberg, M. H., Badesch, D. B., & Gladwin, M. T. (2014). An official american thoracic society clinical practice guideline: Diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. American journal of respiratory and critical care medicine, 189(6), 727-740. https://doi.org/10.1164/rccm.201401-0065ST