Primary pulmonary hypertension

This diagnosis is established when no evidence of a secondary cause can be established. The pulmonary artery wedge pressure should be normal ( TabieJ,). Although etiology cannot be attributed to any single disorder, the predominant target group, young women of childbearing age, is rather homogenous with a mostly fatal outcome. The pulmonary arteriopathy with plexiform lesions, pronounced intimal proliferation, and medial hypertrophy are not exclusive to primary pulmonary hypertension but can also be found in chronic thromboembolism. Evidence exists that a reduced fibrinolytic activity promotes thrombosis leading to vascular obstruction. Deposits of immune complexes, fibrin deposits, and lymphocytic infiltration are demonstrable. In the etiology of primary pulmonary hypertension, a familial predisposition has been demonstrated in some cases, as well as an association with certain drugs (e.g. oral contraceptives, sulfonamides, and antiarrhythmic drugs). Primary pulmonary hypertension has been associated with HIV infection in some patients. In pulmonary veno-occlusive disease the injury and structural changes are localized in the pulmonary veins and venules. The primary site of an increased vascular resistance is the postcapillary vessels, resulting from intimal alterations with organized and partly recanalized thrombi, medial hypertrophy, and arterialization, the hallmark of this etiology. Alterations in pulmonary arterial vasculature appear to be secondary to these features.

There are a number of therapeutic approaches to the management of primary pulmonary hypertension, but therapy remains largely symptomatic and no definite cure is possible. The course of the disease is determined by the successful relief of pulmonary hypertension. Right heart failure is the cause of death in the majority of the patients (63 per cent) and survival rate 5 years after diagnosis is 22 to 38 per cent ( Rubin eLaL 199.3).

Blood Pressure Health

Blood Pressure Health

Your heart pumps blood throughout your body using a network of tubing called arteries and capillaries which return the blood back to your heart via your veins. Blood pressure is the force of the blood pushing against the walls of your arteries as your heart beats.Learn more...

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