Primary pulmonary hypertension

Primary pulmonary hypertension includes increased pulmonary artery pressure and right ventricular myocardial hypertrophy, not associated with congenital or acquired cardiac and vascular pathology. Primary pulmonary hypertension refers to diseases with an autosomal dominant type of inheritance. Family forms of this pathology are described. Primary pulmonary hypertension is a rare disease.

There are many assumptions about the formation of primary pulmonary hypertension. The cause of it may be a congenital defect of the pulmonary vessels, in which the blood circulation in the lungs remains as the fetus in the intrauterine period. It is suggested that a direct cause of increased pressure in the pulmonary artery is a change in the structure of the walls of the pulmonary arterioles with a predominance of connective tissue in them. In all likelihood, the development of the disease is associated with a genetically conditioned defect in muscle fibers. It is also indicated that at the heart of primary pulmonary hypertension is a violation of the nervous system, which leads to the formation of spasm of the blood vessels of the lungs and subsequent structural changes in them.

Primary pulmonary hypertension is more common in young women and girls. The early stages of the disease usually do not reveal, because the increase in pressure in the pulmonary artery by 2 times, and sometimes more, is accompanied only by a moderate decrease in tolerance to the load and does not give a pronounced clinical manifestations. The earliest symptoms of the disease are shortness of breath with moderate physical stress, pain in the heart, palpitation, attacks of suffocation. Cyanotic staining of the skin appears in later stages of the disease. As a rule, there is a thickening of the nail phalanges of the fingers( "drum sticks").In the terminal phase of the disease, the liver is enlarged and painful.

For correct recognition of this disease it is necessary to conduct X-ray and ECG, where the characteristic signs are revealed. The diagnosis of primary pulmonary hypertension is finally confirmed by probing the cavities of the right heart and pulmonary artery, in which other causes that lead to secondary pulmonary hypertension and cyanosis are excluded, and precise figures of pressure in the pulmonary artery are established.

There are currently no effective methods of treatment.

The outlook is unfavorable. Lethal outcome comes from progressive heart failure.