Pulmonary hypertension epidemiology and demographics: Difference between revisions
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==Incidence== | ==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. | 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== | ==Prevalence== | ||
The '''prevalence''' is about 15 per million. | The '''[[prevalence]]''' is about 15 per million. | ||
==Age== | ==Age== | ||
| Line 18: | Line 18: | ||
==Genetics== | ==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. | *'''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== | ==Incidence of Associated Diseases== | ||
*PAH is also associated with <ref>ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension</ref>: | *PAH is also associated with <ref>ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension</ref>: | ||
#Congenital heart disease(30% of untreated) | #[[Congenital heart disease]](30% of untreated) | ||
#Connective tissue diseases (12% of patients with scleroderma and upto 21% of patients with rheumatoid arthritis) | #[[Connective tissue diseases]](12% of patients with scleroderma and upto 21% of patients with rheumatoid arthritis) | ||
#HIV (0.5%) | #HIV (0.5%) | ||
#Portal hypertension(2-6%) | #[[Portal hypertension]](2-6%) | ||
#Sickle cell disease (20 to 40%). | #[[Sickle cell disease]](20 to 40%). | ||
#Systemic lupus erythematosus (4 to 14%) | #[[Systemic lupus erythematosus]](4 to 14%) | ||
#Hemoglobinopathies | #[[Hemoglobinopathies]] | ||
#Myeloproliferative disorders. | #[[Myeloproliferative disorders]]. | ||
#Drugs and toxins | #Drugs and toxins | ||
| Line 36: | Line 36: | ||
*Mortality rate is 5.2-5.4 per 100000 and was more common in African-Americans and women. | *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 hospitilization is [[heart failure]] | ||
*The most common cause of death is right ventricular failure and not chronic lower [[respiratory disease]] as was once thought. | |||
==References== | ==References== | ||
Revision as of 19:01, 22 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
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)
- 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
- The most common cause of death is right ventricular failure and not chronic lower respiratory disease as was once thought.
References
- ↑ ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension