IDIOPATHIC PULMONARY ARTERY HYPERTENSION
SUMMARY Primary pulmonary artery hypertension is defined as a mean resting pulmonary artery pressure > 25 mmHg or a mean pulmonary artery pressure > 30 mmHg with exercise. The World Health Organizations definition is a pulmonary artery systolic pressure > 40 mmHg during echocardiography. Symptoms of pulmonary hypertension include shortness of breath on minimal exertion, fatigue, chest pain, dizzy spells and fainting. All patients with pulmonary artery hypertension must undergo diagnostic procedure, which means the right heart catheterization with vasodilators tests, followed by therapeutic support in the sense of anticoagulant therapy (warfarin) and oxygen. The final therapeutic choice is administration of the following drugs (or their combination): Calcium channel blockers, prostacyclines (epoprostenol, iloprost), antagonist of endothelin-1 receptors (Bosentan), phosphodiesterase type 5 inhibitors (sildenafil). There is a strong sentiment that identifying and treatment of disease at an earlier stage may be even more beneficial.