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American Thoracic Society Releases Guidelines for the Diagnosis and Management of Pulmonary Hypertension of Sickle Cell Disease

The  American Thoracic Society has developed clinical practice guidelines to help  clinicians identify and manage patients with sickle cell disease who are at 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 percent,” said Elizabeth  S. Klings, MD, associate professor of medicine  at the Boston University School of Medicine and chair of the committee  that produced the guidelines. “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 appear in the March 15th issue of the American Thoracic  Society’s American Journal of  Respiratory and Critical Care Medicine.
Guideline  recommendations include:

  • Mortality  risk can be accurately determined noninvasively by measurement of the 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 treatment  can be considered for  chronic  transfusion therapy.
  • In  patients with RHC-confirmed pulmonary hypertension, venous thromboembolism, and  no additional risk factors for hemorrhage, indefinite anticoagulant therapy  rather 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.”