Primary pulmonary hypertension

August 12, 2017 17:51 | Genetic Diseases

to primary pulmonary hypertension include increased pulmonary artery pressure and right ventricular hypertrophy infarction not related to congenital or acquired heart disease and blood vessels.Primary pulmonary hypertension refers to a disease with autosomal dominant inheritance.Described familial forms of the disease.Primary pulmonary hypertension - a rare disease.

There are many assumptions about the formation of primary pulmonary hypertension.The reason it can be a birth defect pulmonary vessels in which the blood circulation in the lungs is retained for itself the type of the fetus in utero.It is believed that the immediate cause of increased pulmonary artery pressure is the change in pulmonary arterioles wall structure with predominance of connective tissue therein.In all likelihood, the disease is associated with the genetically determined defect in the muscle fibers.Also points out that the basis of primary pulmonary hypertension is a violation of the nervous system, which leads to the formation

of the lungs spasm of blood vessels and subsequent structural changes in them.

Primary pulmonary hypertension is more common in young women and girls.The early stage of the disease is usually not detected as an increase in pressure in the pulmonary artery in 2 times, and sometimes more, accompanied by only a modest decrease in tolerance to stress and gives marked clinical manifestations.The earliest symptoms are shortness of breath with moderate physical exertion, pain in the heart, palpitations, asthma attacks.A bluish coloration of the skin appears in the later stages of the disease.As a rule, there is thickening of the nail phalanges ( "drumsticks").In the terminal phase of the disease revealed an increase in pain and liver.

for proper recognition of the disease is necessary to conduct X-ray examination and an electrocardiogram, which revealed the characteristic features.The diagnosis of primary pulmonary hypertension definitively confirmed by probing the right cavities of the heart and pulmonary artery, where the exclusion of other causes that may lead to secondary pulmonary hypertension and cyanosis, and set the exact figures of pressure in the pulmonary artery.

of effective therapies currently exist.

Prognosis is poor.The lethal outcome occurs due to progressive heart failure.