Primary and Secondary Pulmonary Hypertension

Abstract

Interest in pulmonary hypertension (PH) has substantially increased over the last decade. This is a result of effective treatments and the ability to determine prognosis. Studies of the natural history of PH have indicated that the mixed venous oxygen saturation, the level of cardiac output and elevation of the mean right atrial pressure allow accurate prediction of survival.

The survival of patients without overt right heart failure is improved with anticoagulant treatment, with or without calcium channel oral vasodilators. Oral vasodilators are used if there is significant pulmonary vasodilation with prostacyclin at the time of diagnostic right heart catheter. In patients with advanced right heart failure, continuous infusion of prostacyclin, or its analogue Iloprost, improves quality of life and survival.

When medical treatment fails to control the progress of the disease, lung or heart lung transplantation provides an effective treatment. Survival from transplantation, however, is lower than with prostaglandin treatment.

New medical treatments, such as oral or inhaled prostaglandins, appear effective and recently inhaled nitric oxide has also been used. For patients with evidence of thromboembolic disease, proven at pulmonary angiography, surgical pulmonary end-arterectomy has been shown to reverse the pulmonary hypertension.

Further advances in treatment are likely to follow the recent localization of a gene for familial pulmonary hypertension to chromosome 2. Pulmonary hypertension, both secondary and primary, is no longer a diagnosis without hope.

Key Words:

primary pulmonary hypertension - secondary pulmonary hypertension - lung disease