Patients who present with symptoms but do not have congestive heart failure may follow a much more stable course, and they frequently survive for many years. Sudden death occurs occasionally, but chronic congestive heart failure is the cause of death in most cases. Rupture or dissection of the pulmonary artery is the terminal event in some cases. Therapy Patients with primary pulmonary hypertension should be counseled to live within the limits of their disability and to avoid strenuous physical exercise. Actions or conditions leading to marked systemic vasodilatation, such as standing up after taking a very hot bath, should also be avoided.
Pregnancy is hazardous, and oral contraceptive agents are contraindicated.
Anticoagulation is recommended routinely by some authorities because microvascular thrombosis may play a major role in the progression of the disease. Vasodilators have been extensively used to lower pulmonary vascular resistance and pulmonary arterial pressure in patients with primary pulmonary hypertension. Virtually all drugs that have a vasodilator effect in the systemic arterial circulation lower pulmonary vascular resistance in at least a few patients with primary pulmonary hypertension. However, none of the vasodilators has been shown to have consistent efficacy, and there is no evidence that the natural history of the disease has been favorably affected by vasodilator therapy. The dose of a vasodilator is often limited by the fall in systemic arterial pressure that consistently occurs.