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ATS releases guidelines on the management of pulmonary hypertension of sickle cell disease
Date:3/14/2014

The American Thoracic Society has developed clinical practice guidelines to help clinicians identify and manage patients with sickle cell disease who are at increased risk for mortality from pulmonary hypertension.

"With the development of new treatments, many patients with sickle cell disease are now surviving long enough to develop pulmonary hypertension, with an estimated prevalence of 6 to 11 percent," said Elizabeth S. Klings, MD, associate professor of medicine at the Boston University School of Medicine and chair of the committee that produced the guidelines. "Although pulmonary hypertension and elevated tricuspid jet velocity (TRV, an indicator of pulmonary hypertension measured by echocardiography) are both associated with an increased mortality risk, there is currently no standardized approach for identifying and managing these patients."

The guidelines appear in the March 15th issue of the American Thoracic Society's American Journal of Respiratory and Critical Care Medicine.

Guideline recommendations include:

  • Mortality risk can be accurately determined noninvasively by measurement of the TRV with Doppler echocardiography or by measurement of serum N-terminal probrain natriuretic peptide (NT-pro-BNP) levels and can be determined invasively by direct hemodynamic measurements via right heart catheterization (RHC).

  • An increased risk for mortality is defined as a TRV ≥ 2.5 m/second, an NT-pro-BNP level ≥ 160 pg/ml, or RHC-confirmed pulmonary hypertension.

  • Patients found to have an increased mortality risk should be treated with hydroxyurea. Patients who do not respond to or are not candidates for hydroxyurea treatment can be considered for chronic transfusion therapy.

  • In patients with RHC-confirmed pulmonary hypertension, venous thromboembolism, and no additional risk factors for hemorrhage, indefinite anticoagulant therapy rather
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Contact: Nathaniel Dunford
ndunford@thoracic.org
American Thoracic Society
Source:Eurekalert

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