Information on Idiopathic Pulmonary Hypertension

Idiopathic pulmonary hypertension also called is Pulmonary hypertension Pulmonary hypertension is an abnormal altitude of the pressure in the pulmonary flow caused by the constriction of the blood vessels that supply blood to the lungs. Pulmonary hypertension can be a severe disease with a markedly decreased exercise tolerance and heart failure. Frequency in children as well as adults is not known. Conceivably, more patients have the disease than previously suspected. IPAH occurs at a female-to-male ratio ranging from 2-9:1, depending on the treatment center sampled.

The worldwide incidence of Idiopathic pulmonary hypertension approximates that observed in the United States. Pumonary hypertension may be caused by a genetic defect, confident diet medications, or medical conditions such as lung or heart valve disease or history of a blood clot in the lung. Various triggers, such as high altitude, hypoxemia, toxins, sympathetic tone, and autoimmune disorders, can cause pulmonary vasoconstriction in susceptible individuals. Symptoms may develop very gradually. Shortness of breath and dizziness are symptoms of pulmonary hypertension.

Treatment involves of Idiopathic pulmonary hypertension diuretics, blood thinners, calcium channel blockers, and using supplemental oxygen to increase blood oxygen levels. Medicines used to treat pulmonary hypertension include ambrisentan (Letairis), bosentan, calcium channel blockers, diuretics and prostacyclin  Prostacyclin (prostaglandin I2) is commonly considered the most effective treatment for PAH. Treprostinil (Remodulin) can be given intravenously or subcutaneously, but the subcutaneous form can be very painful. If treatment with medications fails, suitable candidates may be helped by lung or heart-lung transplant.

High dose calcium channel blockers are useful in only 5% of IPAH patients who are vasoreactive by Swan-Ganz catheter. Use digoxin therapy to improve right ventricular function in patients with right ventricular failure. Use diuretics to manage peripheral edema. The use of loop diuretics requires potassium supplementation and close monitoring of serum potassium. Potassium-sparing diuretics may have a role in ameliorating the sometimes-intractable hypokalemia observed with daily diuretic use. Patients taking warfarin must limit their intake of vitamin K–containing foods, such as green leafy and coliform vegetables.