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The multidisciplinary care offered at ��ɫtv Langone for pulmonary hypertension (PH) has been formalized and expanded with the creation of the new Pulmonary Hypertension Program. Under the leadership of noted pulmonologist Roxana Sulica, MD, associate professor of medicine, who joined ��ɫtv Langone in July 2017 as the program’s director, the program will offer comprehensive diagnostic and therapeutic services to patients with all forms of PH.

Dr. Sulica and her team will collaborate with the Interstitial Lung Disease Program directed by Rany Condos, MD, clinical professor of medicine and director of the Adult Cystic Fibrosis Program; the Heart Failure Advanced Care Center, led by Alex Reyentovich, MD, associate professor of medicine and medical director of the Ventricular Assist Device Program; and with Luis F. Angel, MD, professor of medicine and cardiothoracic surgery, and medical director of the new Lung Transplant Program, part of the Transplant Institute, which launched in early 2018.

The new ��ɫtv Langone program is one of the country’s largest treating this rare, complex progressive disorder characterized by abnormally high blood pressure in the arteries of the lungs and leading to right ventricular failure and death. “We are currently caring for over 400 patients with this condition,” says Dr. Sulica, a fellowship-trained expert in the condition who previously directed the pulmonary hypertension programs at Mount Sinai Hospital and Mount Sinai Beth Israel in New York. She notes that these patients come from New York City’s five boroughs and beyond—Long Island, upstate New York, New Jersey, Pennsylvania, and Connecticut—seeking the highly specialized care the center offers.

“Untreated, the life expectancy of an individual with PH is less than three years, but with proper care, they can expect to live significantly longer,” says Dr. Sulica. “Therefore, it is critical that the procedures involved—such as a right heart catheterization for accurate disease diagnosis—be performed with precision by experienced specialists.”

Zachary N. Kon, MD, assistant professor of cardiothoracic surgery, will lead a new program to treat chronic thromboembolic pulmonary hypertension. The recommended treatment, pulmonary thromboendarterectomy, is currently performed by only a small number of institutions across the country.

Highly Specialized Medication Helps to Treat Pulmonary Hypertension

Epoprostenol, the first medication approved by the Food and Drug Administration for treatment of PH, is only available as a continuous intravenous infusion. Patients carry a battery-operated pump that infuses the drug; because of its short half-life, any interruption in therapy from a pump malfunction or human error can be life-threatening. “If the system stops, the patient can die within 30 minutes,” Dr. Sulica says. “For this reason, all our patients have 24/7 coverage and can reach me by phone at any time.”

Over the past 15 years, additional drugs have been approved for the treatment of PH, with a total of 14 agents—including pills, inhaled agents, and subcutaneous injections—now available. Epoprostenol, however, remains the only drug to show survival benefit in a three-month controlled trial, and the optimal choice for the very sick patients. Tailoring the appropriate drug regimen for each patient requires both expertise and long-standing experience, and studies from Europe have demonstrated better outcomes for patients treated in specialized centers from the moment of diagnosis as opposed to patients who had therapy initiated in non-expert centers.

“We are hopeful that three new classes of drugs on the horizon for PH may be even more effective,” says Dr. Sulica. These drugs may also offer alternative administration routes, such as an implantable pump filled monthly or biweekly in the clinic, eliminating the need for patients to replace a cassette or syringe of medication every day or every other day. Dr. Sulica, who has been principal investigator in numerous multicenter PH therapeutic trials and registries for more than a decade, is preparing to enroll patients in multiple trials of these new agents, beginning in early 2018.