Cardiovascular Institute

AGH offers PH patients the most advanced medication-based treatments, including all oral, inhaled, subcutaneous and intravenous therapies. Patients also have access to the latest combination therapies, which attempt to target multiple pathophysiologic pathways — an emerging new treatment strategy. Multiple clinical trials are underway at the Cardiovascular Institute, giving patients access to new, promising 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; sildenafil and tadalafil, phosphodiesterase-5 inhibitors; and ambrisentan, a new selective endothelin receptor antagonist, have been approved by the FDA for chronic oral therapy.

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 for subcutaneous, intravenous and inhalational delivery. Iloprost, another inhaled prostanoid, is also approved and requires specialized nebulizer treatments six to nine times each day. Combination therapy, an attempt to target multiple pathophysiologic pathways, is emerging as a new strategy for PH treatment and remains a focus of our center's treatment plan.

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.