What is now considered “conventional therapy” for patients with primary pulmonary hypertension?

Conventional therapy includes supplemental oxygen as needed to maintain an oxygen saturation of at least 91 %, diuretics if the patient has clinically significant edema or ascites, oral vasodilators (usually calcium channel antagonists), anticoagulation in the absence of contraindications, and occasionally digitalis.

How are oral vasodilators used in the treatment of primary pulmonary hypertension?

Many oral vasodilators have been tried in the treatment of primary pulmonary hypertension. Most recently, interest has focused on the calcium channel blockers nifedipine and diltiazem. A recent study suggests that approximately one-quarter of patients with primary pulmonary hypertension have a favorable response to calcium channel blockers. Patients who respond are thought to have a better prognosis. However, continued follow-up remains important, because patients who demonstrate favorable initial response later deteriorate.

The response to vasodilator therapy in an individual patient is difficult to predict. There is significant risk of adverse reactions, particularly systemic hypotension, since most of the drugs utilized are not selective pulmonary vasodilators. For these reasons, a trial of a short-acting intravenous vasodilator should be done in the cardiac catheterization laboratory or intensive care unit.

Why is anticoagulation recommended for patients with primary pulmonary hypertension?

Although no prospective randomized studies have not been done, retrospective data suggest that anticoagulation decreases mortality.

Is heart-lung transplantation the transplant procedure of choice in patients with severe pulmonary hypertension?

In most cases, it is no longer thought necessary to transplant the heart in patients with severe pulmonary hypertension. When its afterload is reduced by the transplantation of one or both lungs, the right ventricle is able to recover function. Transplantation of the heart, therefore, is often unnecessary and adds the potential for cardiac rejection and accelerated coronary vascular disease. Occasionally, heart-lung transplantation is still needed in the patient with an underlying congenital heart defect that cannot be repaired at the time of transplantation.

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