Management of Pulmonary Arterial Hypertension

Pulmonary arterial hypertension, considered a death sentence just a generation ago, is a rare but serious disease that occurs when the walls of the lung’s arteries stiffen and narrow, raising blood pressure in these arteries. The lungs can be permanently damaged, and the heart must pump harder to push blood through the lung, leading to life-threatening heart failure in children and adults.

“Two decades ago, patients diagnosed were told they had one to two years to live,” says Erika Berman Rosenzweig, MD, associate professor of pediatrics (in medicine) and medical director of the Pulmonary Hypertension Comprehensive Care Center at Columbia.

“Now, with new advances, we have patients who have been managed at the center for more than 20 years and going strong,” Dr. Rosenzweig says. “This evolution has been amazing to be a part of and has enabled patients to live longer and stronger with pulmonary arterial hypertension.”

The Pulmonary Hypertension Comprehensive Care Center at Columbia is one of the first—and among the largest—in the world established to treat the disease. For the past 30 years, the center has been at the forefront of research, including major clinical trials, landmark genetic discoveries, and pioneering treatments such as mechanical support for patients who fail advanced medical therapy. 

“We have led the way in both pediatric and adult pulmonary arterial hypertension management and research by offering the most advanced medical therapies, often through clinical trials,” Dr. Rosenzweig says. “We have been involved in all of the pivotal drug trials that have led to FDA approval of the 14 medications available for the disease.”

The center has become known for providing the most advanced medical care for the sickest patients. Surgeries that would not have been considered previously are now possible.

The center has pioneered the use of ECMO—an external machine that oxygenates blood—in pulmonary hypertension patients as a bridge to a lung transplant or to help patients recover from a life-threatening crisis.

“Previously these patients were not offered ECMO because it was thought they could not be weaned from the support or survive long enough on ECMO while waiting for an organ,” says Matthew Bacchetta, MD, associate professor of surgery and the center’s surgical director.

“At Columbia, ECMO is being successfully used and, in many cases, physical condition improves and patients become better candidates for a transplant.”

Dr. Bacchetta is among only a few surgeons in the world who perform surgery for chronic thromboembolic pulmonary hypertension, which happens when thrombi build up in the pulmonary arteries. During the procedure, called pulmonary thromboendarterectomy, the blockages are surgically removed from the lungs. “It’s an attractive alternative to lung transplantation and is associated with better long-term survival, although the procedure isn’t well-known among most physicians,” says Dr. Bacchetta. 

“As the center sees patients surviving longer, we are now challenged with how to manage them through comorbid conditions,” says Dr. Rosenzweig. “We are proud that our team can literally change lives and improve outcomes on a daily basis for a disease that was recently considered universally fatal. To see patients get back out there and attend school, work, or travel and be an active member of their community gives us tremendous pride in the work we do.”

Jeff Ballinger

The Pulmonary Hypertension Comprehensive Care Center treats all forms of pulmonary hypertension, including hypertension caused by other conditions. For more information, contact the center at 212-305-4436.