(HealthDay News) — Evidence-based guidelines have been developed for adult patients with sickle cell disease (SCD) and pulmonary hypertension. The guidelines have been published in the March 15 issue of the American Journal of Respiratory and Critical Care Medicine.

Elizabeth S. Klings, MD, from Boston University, and colleagues produced evidence-based recommendations relating to management of adult patients with pulmonary hypertension in SCD.

According to the report, decision making should be guided by stratification of mortality risk. For patients with increased mortality risk (defined as tricuspid regurgitant velocity [TRV] ≥2.5 m/second; serum N-terminal pro-brain natriuretic peptide [NT-pro-BNP] ≥160 pg/mL; or right heart catheterization [RHC]-confirmed pulmonary hypertension), hydroxyurea was strongly recommended as first-line therapy, and chronic transfusions were weakly recommended as an alternative therapy. The authors strongly recommended against pulmonary arterial hypertension-specific therapy for all SCD patients with elevated TRV alone, elevated serum NT-pro-BNP alone, and for those with RHC-confirmed pulmonary hypertension with elevated pulmonary capillary wedge pressure and low pulmonary vascular resistance. There was a weak recommendation for prostanoid or endothelin receptor antagonist therapy, and a strong recommendation against phosphodeisterase-5 inhibitor therapy for select patients with SCD with RHC-confirmed pulmonary hypertension with increased pulmonary vascular resistance and normal pulmonary capillary wedge pressure.

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

Several authors disclosed financial ties to the pharmaceutical and biotechnology industries.

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