Primary pulmonary hypertension

Primary pulmonary hypertension is defined as an intrinsic, idiopathic, obstructive disease of the small pulmonary arteries and arterioles. The aetiology is unknown. However pregnancy, oral contraceptives and familial occurrence are noteworthy .Primary pulmonary hypertension is more common in females. The mean patient age is 20-30 years. Death commonly occurs within 5 years of the onset of symptoms. Clinically dysponea, weakness, fatigue, exercise included syncope and chest pain in an otherwise healthy, young, acyanotic woman with no heart disease or cardiac murmurs suggest primary pulmonary hypertension. . Sudden death is common and physical signs are the same as that for pulmonary hypertension. Some managements of this disease are 1.Restrict physical activities, 2.Careful use of diuretics in patients with right ventricular failure and functional tricuspid regurgitation; 3.Calcium channel blockers may alleviate pulmonary vaso-constriction and prolong life in about 20% cases. Unfortunately, there is no way to predict which patient will respond to oral vasodilators; 4. Epoprostenol is a potent short acting vasodilator. Continuous intravenous infusion of this agent has been shown to prolong life, 5.Endothelin receptor and antagonists and selective phosphodiesterase-5 inhibitors are under trial; 6. Maintaining an International Normalized Ratio is recommended and ultimate answer is lung transplantation.