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

than a limited duration of therapy should be used.

  • Patients with elevated TRV alone or elevated NT-pro-BNP alone should not be treated with targeted pulmonary arterial hypertension therapies, including prostanoid, endothelin receptor antagonist, and phosphodiesterase-5 inhibitor therapy

  • Most patients with RHC-confirmed pulmonary hypertension should not be treated with targeted therapy

  • In select patents with RHC-confirmed marked elevation of pulmonary vascular resistance, normal pulmonary capillary wedge pressure, and related symptoms, a trial of either a prostanoid or an endothelin receptor antagonist may be performed.

  • Patients with RHC-confirmed marked elevation of pulmonary vascular resistance, normal pulmonary capillary wedge pressure, and related symptoms should not receive phosphodiesterase-5 inhibitor therapy as first-line treatment.

    "As our understanding of sickle cell disease develops, so will our ability to detect disease earlier and to tailor treatment approaches. We need to continue our research efforts into this disease and its management to understand what the optimal treatment regimen for these patients is. Most of our current recommendations are limited by a lack of large-scale clinical trials in this population." said Dr. Klings. "Management of patients with sickle cell disease with an increased risk for mortality and pulmonary hypertension will ultimately be a collaborative effort including adult and pediatric pulmonologists, cardiologists, and hematologists."


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  • Contact: Nathaniel Dunford
    ndunford@thoracic.org
    American Thoracic Society
    Source:Eurekalert

    Page: 1 2

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