Pulmonary Hypertension Program

Advances in Medical Therapy for Pulmonary Hypertension

Allegheny's program makes available all of the latest medication-based therapies rapidly being developed, even as the understanding of the disease's pathophysiology expands, including all oral, inhaled, subcutaneous and intravenous therapies. Patients also have access to the latest in combination therapies, which attempt to target multiple pathophysiologic pathways — an emerging new treatment strategy. Multiple clinical trials are underway at Allegheny, providing patients access to the novel options.

Historically, the only oral medications available were calcium channel blockers, which were effective in up to 10 percent of patients. Within the past few years bosentan, a non-selective endothelin receptor antagonist, and more recently, sildenafil, a phosphodiesterase-5 inhibitor, have been approved by the FDA for chronic oral therapy. In July 2007, a new selective endothelin receptor antagonist called ambrisentan was FDA approved.

Prostanoids remain a mainstay for advanced disease, and several new delivery routes are now available. Intravenous epoprostenol is the standard of care for the advanced, severely symptomatic patient, although it is a complex medication with limiting side effects. Trepostinil is approved both for subcutaneous and intravenous delivery. Iloprost, an inhaled prostanoid, has recently been approved and requires specialized nebulizer treatments six to nine times each day. Combination therapy in an attempt to target multiple pathophysiologic pathways is emerging as a new strategy for PH treatment.

Lung and heart-lung transplantation remain the only curative therapies and are recommended for select patients with progressive symptoms despite optimal medical therapy. Balloon atrial septostomy is available for select patients with refractory right ventricular failure. Some patients with thrombo-embolic disease may be candidates for pulmonary thrombo-endarterectomy.