Pulmonary hypertension epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] 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.