ATS releases guidelines on the 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 with 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 , 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 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 for mortality and will ultimately be a collaborative effort including adult and pediatric pulmonologists, cardiologists, and hematologists."

add to favorites email to friend print save as pdf

Related Stories

Recommended for you

Global Ebola toll rises to 5,689: WHO

3 hours ago

The World Health Organization said Thursday that the global death toll from the Ebola virus had increased to 5,689 out of a total of 15,935 cases of infection, mainly in western Africa.

Ebola vaccine promising in first human trials

14 hours ago

Researchers say they're a step closer to developing an Ebola vaccine, with a Phase 1 trial showing promising results, but it will be months at the earliest before it can be used in the field.

At one month, US Ebola monitors finding no cases

17 hours ago

The U.S. program that requires weeks of monitoring for travelers from African countries with Ebola reaches the one-month mark Thursday. And so far, no cases of the disease have turned up.

User comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.