(right) and a normal one
Credit: Betty Pace
The American Thoracic Society has developed clinical practice guidelines to help clinicians identify and manage patients with sickle cell disease who are at an 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%,” said guideline author Elizabeth S. Klings, MD, of the Boston University School of Medicine in Massachusetts.
“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.”
So Dr Klings and her colleagues decided to offer some guidance. Their recommendations for managing these patients appear in the American Journal of Respiratory and Critical Care Medicine.
The guideline authors point out that clinicians can evaluate patient mortality noninvasively by measuring the TRV with Doppler echocardiography or by measuring serum N-terminal pro–brain natriuretic peptide (NT-pro-BNP) levels.
An invasive method is taking 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 who meet these criteria should receive hydroxyurea. Patients who do not respond to or are not candidates for hydroxyurea treatment can be considered for chronic transfusion therapy.
For patients with RHC-confirmed pulmonary hypertension, venous thromboembolism, and no additional risk factors for hemorrhage, the guidelines recommend indefinite anticoagulant therapy rather than a limited duration of therapy.
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 receive targeted therapy.
For select patents with RHC-confirmed marked elevation of pulmonary vascular resistance, normal pulmonary capillary wedge pressure, and related symptoms, the guidelines suggest a trial of either a prostanoid or an endothelin receptor antagonist.
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.
“Most of our current recommendations are limited by a lack of large-scale clinical trials in this population,” Dr Klings noted. “We need to continue our research efforts into this disease and its management to understand what the optimal treatment regimen for these patients is.”
“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.”
