Thoracic society releases guidelines on pulmonary hypertension in sickle cell patients

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

Many patients with sickle cell disease are now surviving long enough to develop pulmonary hypertension, with an estimated prevalence of 6% to 11%, the society stated in a press release.

The guidelines appear in the March 15 American Journal of Respiratory and Critical Care Medicine.

Recommendations include the following:

• Mortality risk can be accurately determined noninvasively by measurement of the tricuspid regurgitant velocity (TRV) with Doppler echocardiography or by measurement of serum N-terminal pro-brain 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 can be considered for chronic transfusion therapy.
• Indefinite anticoagulant therapy rather than a limited duration of therapy should be used in patients with RHC-confirmed pulmonary hypertension, venous thromboembolism, and no additional risk factors for hemorrhage.
• 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.
• A trial of either a prostanoid or an endothelin receptor antagonist may be performed in select patents with RHC-confirmed marked elevation of pulmonary vascular resistance, normal pulmonary capillary wedge pressure, and related symptoms.
• 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.

For more on sickle cell disease, read ACP Internist's coverage from April 2013, view a video from ACP's multimedia collection, or explore ACP's Smart Medicine module on the condition.