Pulmonary hypertension epidemiology and demographics: Difference between revisions
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==Epidemiology== | ==Epidemiology== | ||
While previously considered a rare disease, the most recent evidence from a French registry suggests that the | *While previously considered a rare disease, the most recent evidence from a French registry suggests that the '''incidence''' of pulmonary arterial hypertension is 2-3 per million per year and the '''prevalence''' is about 15 per million. | ||
*Pulmonary Hypertension usually develops between the ages of 20 and 60, but it can occur at any age. | |||
'''Idiopathic pulmonary hypertension''' which is more prevalent in women, was considered the most common type of Pulmonary arterial hypertension in a French registry. | *'''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. | ||
'''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. | *'''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 | *PAH is also associated with <ref>ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension</ref>: | ||
#Congenital heart disease(30% of untreated) | |||
#Connective tissue diseases (12% of patients with scleroderma and upto 21% of patients with rheumatoid arthritis) | |||
#HIV (0.5%) | |||
#Portal hypertension(2-6%) | |||
#Sickle cell disease (20 to 40%). | |||
#Systemic lupus erythematosus (4 to 14%) | |||
#Hemoglobinopathies | |||
#Myeloproliferative disorders. | |||
#Drugs and toxins | |||
*Diet pills such as [[Fen-Phen]] produced an annual incidence of 25-50 per million per year. | |||
The most common cause of hospitilization is heart failure whereas, the most common cause of death is | *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 right ventricular failure and not chronic lower respiratory disease as was once thought. | |||
Revision as of 15:28, 12 September 2011
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assistant Editor(s)-in-Chief: Ralph Matar,
Epidemiology
- While previously considered a rare disease, the most recent evidence from a French registry suggests that the incidence of pulmonary arterial hypertension is 2-3 per million per year and the prevalence is about 15 per million.
- Pulmonary Hypertension usually develops between the ages of 20 and 60, but it can occur at any age.
- 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.
- 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 [1]:
- Congenital heart disease(30% of untreated)
- Connective tissue diseases (12% of patients with scleroderma and upto 21% of patients with rheumatoid arthritis)
- HIV (0.5%)
- Portal hypertension(2-6%)
- Sickle cell disease (20 to 40%).
- Systemic lupus erythematosus (4 to 14%)
- Hemoglobinopathies
- Myeloproliferative disorders.
- 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 whereas, the most common cause of death is right ventricular failure and not chronic lower respiratory disease as was once thought.
- ↑ ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension