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:
"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."
|Contact: Nathaniel Dunford|
American Thoracic Society