Pulmonary hypertension epidemiology and demographics

Assistant Editor(s)-in-Chief: Ralph Matar,

Incidence
While previously considered a rare disease, the most recent evidence from a French registry suggests that the incidence of new cases of pulmonary arterial hypertension is 2-3 cases per million per year.

Prevalence
The prevalence is about 15 per million.

Age

 * Pulmonary Hypertension usually develops between the ages of 20 and 60, but it can occur at any age.

Gender

 * Idiopathic pulmonary hypertension(IPAH) which is more prevalent in women(3x more common), was considered the most common type of Pulmonary arterial hypertension in a French registry.

Genetics

 * 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.

Incidence of Associated Diseases

 * PAH is also associated with :
 * 1) Congenital heart disease(30% of untreated)
 * 2) Connective tissue diseases(12% of patients with scleroderma and upto 21% of patients with rheumatoid arthritis)
 * 3) HIV (0.5%)
 * 4) Portal hypertension(2-6%)
 * 5) Sickle cell disease(20 to 40%).
 * 6) Systemic lupus erythematosus(4 to 14%)
 * 7) Hemoglobinopathies
 * 8) Myeloproliferative disorders.
 * 9) Drugs and toxins


 * Diet pills such as Fen-Phen produced an annual incidence of 25-50 per million per year.


 * 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


 * The most common cause of death is right ventricular failure and not chronic lower respiratory disease as was once thought.