Pulmonary hypertension epidemiology and demographics

Epidemiology

While previously considered a rare disease, the most recent evidence from a French registry suggests that the prevalence of Pulmonary arterial hypertension is about 15 per million.

Idiopathic pulmonary hypertensionwhich is more prevalent in women, was considered the most common type of PAH in a French registry.

Familial PAH often results from a mutation in the bone morphogenic protein receptor-2 (BMPR2) and is inherited as an autosomal dominant disease with incomplete penetrance and anticipation.

PAH is also associated with congenital heart disease(30% of untreated), connective tissue diseases like systemic sclerosis(12% of patients), drugs and toxins, HIV, portal hypertension(2-6%), hemoglobinopathies, and myeloproliferative disorders.

Mortality rate is 5.2-5.4 per 100000 and was more common in African-Americans and women.

The most common cause of hospitilization is heart failure whereas, the most common cause of death is pulmonary hypertension itself and not chronic lower respiratory disease as was once thought.