TUESDAY, March 31 (HealthDay News) -- Although recent advances have led to increased recognition of new therapies for pulmonary hypertension, this complex and rare disorder has been inadequately explored, according to an Expert Consensus Document published online March 30 in the Journal of the American College of Cardiology to coincide with the American College of Cardiology's 58th Annual Scientific Sessions held March 29 to 31 in Orlando, Fla.

Vallerie V. McLaughlin, M.D., chair of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association, and colleagues reviewed the literature and released this first ACCF/AHA Clinical Expert Consensus Document on Pulmonary Hypertension.

The authors suggest that the prevalence of pulmonary arterial hypertension (PAH) is 15 per million, that the condition is more common in women, that it often results from a mutation in the bone morphogenic protein receptor-2, and is associated with congenital heart disease, connective tissue diseases, drugs and toxins, HIV, portal hypertension, hemoglobinopathies and myeloproliferative disorders.

"PAH is a rare disease with a high mortality," the authors write. "Given the complex nature of this disease, if at all possible, most patients should be managed at or in conjunction with a PAH Center of Excellence. Centers that specialize in PAH offer physicians with expertise and experience in this complicated disease, nursing staff specially trained to assist in the management of PAH patients and the complex therapies for this disease, and often clinical trials of investigational agents. Multidisciplinary programs enlist the expertise of both cardiologists and pulmonologists, and commonly rheumatologists, hepatologists, infectious disease specialists, hematologists, transplant physicians, psychologists and social workers."