Pulmonary hypertension epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ralph Matar

Overview

Pulmonary arterial hypertension has been considered as a disease of young women. The mean age of patients in the U.S. registry was 36 years and the overall female-to-male ratio was 1.7:1.

Epidemiology and Demographics

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.

Association with Diseases

• PAH is also associated with:

1. Congenital heart disease(30% of untreated)
2. Connective tissue diseases(12% of patients with scleroderma and up to 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.