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Guidelines target pulmonary hypertension in patients with sickle cell disease
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The American Thoracic Society recently formulated clinical practice guidelines to better identify and manage patients with sickle cell disease who are at increased risk for death from pulmonary hypertension.
The guidelines, published in the American Journal of Respiratory and Critical Care Medicine, elucidate the preferred approaches to assessing and managing mortality risk.
“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%,” Elizabeth S. Klings, MD, associate professor of medicine at the Boston University School of Medicine and chair of the committee that produced the guidelines, said in a press release. “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 offer both noninvasive and invasive approaches for determining mortality risk of patients with sickle cell disease.
Noninvasive options include measurement of tricuspid regurgitation velocity using Doppler echocardiography, as well as measurement of blood levels of N-terminal pro-brain natriuretic peptide (NT-proBNP).
Risk also can be assessed invasively through direct hemodynamic measurements using right heart catheterization (RHC).
According to the guidelines, a sickle cell disease patient with TRV ≥2.5 m/second, an NT-proBNP level ≥160 pg/mL or RHC-confirmed pulmonary hypertension should be considered at increased risk for pulmonary hypertension-related mortality.
Those determined to be at elevated risk should undergo treatment with hydroxyurea, according to the guidelines. Those who fail to respond or those who are not eligible for hydroxyurea therapy may be candidates for chronic transfusion therapy.
For patients with RHC-diagnosed pulmonary hypertension, venous thromboembolism and no further risk factors for hemorrhage, ongoing anticoagulant therapy is indicated, as opposed to limited-duration therapy. Those with high TRV alone or elevated NT-pro-BNP alone should not be placed on a regimen of targeted pulmonary arterial hypertension medications, including prostanoids, endothelin receptor antagonists or phosphodiesterase-5 inhibitors.
Additionally, most patients with RHC-diagnosed pulmonary hypertension should not receive these targeted therapies, the American Thoracic Society noted.
Exceptions would include certain patients with RHC-confirmed marked elevation of pulmonary vascular resistance, normal pulmonary capillary wedge pressure and associated symptoms. For these patients, a trial regimen of either a prostanoid or an endothelin receptor antagonist might be indicated. However, these patients should not receive phosphodiesterase-5 inhibitors as first-line treatment, according to the guidelines.
“As our understanding of sickle cell disease develops, so will our ability to detect disease earlier and to tailor treatment approaches,” Klings said. “Most of our current recommendations are limited by a lack of large-scale clinical trials in this population. 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.”
Disclosure: The researchers report no relevant financial disclosures.
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