Current and future treatment of pulmonary hypertension

Abstract

Pulmonary hypertension is a disorder characterized by an increase in mean pulmonary arterial pressure (mPAP > 25 mmHg), which is responsible for the transport of blood from the heart to the lungs. Increased pressure leads to decreased flow of blood through the lungs and decreased oxygen deliverance throughout the body. The disorder causes right ventricular hypertrophy and can quickly lead to death, especially with the severe forms of pulmonary hypertension. Symptoms include fatigue, shortness of breath, dizziness and peripheral edema in the lower extremities. Symptoms are usually delayed in appearance and progress slowly, which leads to a late diagnosis and often a poor prognosis. Despite large advances in the last 10 years, there is still about a 15% annual mortality for diagnosed patients. Despite the number of medications available, there are still no cures for this fatal disease. Current therapies include endothelin receptor antagonists, prostacyclin agonists and cGMP-specific 3',5'-cyclic phosphodiesterase (PDE5) inhibitors or combinations. Recent strategies have shown promise in animal models to prevent the onset of pulmonary hypertension when it is induced. However, few of them show a sustained benefit in clinical trials. Strategies for the cure of this debilitating disease should be the focus of future research.