Primary Pulmonary Hypertension
What is primary pulmonary hypertension?
Primary pulmonary hypertension is a progressive disorder characterised by abnormally high blood pressure (hypertension) that affects arteries in the lungs (pulmonary arteries). Hypertension is caused by the narrowing (obstruction) of very small arteries throughout the lungs. "Primary" means that the condition is not caused by other diseases of the heart or lungs.
Shortness of breath (dyspnea) and fatigue are the most common symptoms of primary pulmonary hypertension. People with this disorder may experience additional symptoms, particularly as the condition worsens. Other symptoms include dizziness, fainting spells, swelling (oedema) of the ankles or legs, chest pain, and a racing pulse.
How common is primary pulmonary hypertension?
In the United States, an estimated 300 new cases of primary pulmonary hypertension are diagnosed each year. This disorder is twice as common in females as in males.
What genes are related to primary pulmonary hypertension?
Mutations in the BMPR2 gene cause primary pulmonary hypertension.
The BMPR2 gene plays a role in regulating cell number in certain tissues. Researchers suggest that a mutation in this gene promotes cell division or prevents cell death, resulting in an overgrowth of cells. Cell overgrowth in small arteries can narrow the diameter of the arteries and increase resistance to blood flow. When arteries are constricted, blood pressure increases to overcome the increased resistance. Additionally, the heart is strained, as it has to work harder than usual to pump blood to the lungs.
Other genes that have not yet been identified may also cause primary pulmonary hypertension. In addition, other genes may contribute to the development of disease in people with a known mutation in the BMPR2 gene.
How do people inherit primary pulmonary hypertension?
This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. In many cases, however, people with an altered BMPR2 gene never develop symptoms of primary pulmonary hypertension.
Inherited cases of this disorder are known as familial pulmonary arterial hypertension. As the altered gene is passed down from one generation to the next, the disorder generally begins earlier in life. This phenomenon is called anticipation.
Most cases of primary pulmonary hypertension, however, occur in individuals with no known family history of the disorder. These cases are known as idiopathic pulmonary arterial hypertension. Some idiopathic cases are due to mutations in the BMPR2 gene, but in most cases a gene mutation has not yet been identified.
What other names do people use for primary pulmonary hypertension?
- Ayerza Syndrome
- familial primary pulmonary hypertension (FPPH)
- Idiopathic pulmonary hypertension
- PPH
- PPHT - Primary pulmonary hypertension
- pulmonary arterial hypertension
- sporadic primary pulmonary hypertension
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