Philadelphia Adult Congenital Heart Program Reports Excellent Outcomes in Heart Transplants in ACHD Patients


A research team from the Philadelphia Adult Congenital Heart Program reported excellent outcomes in performing heart transplants in patients who had adult congenital heart disease (ACHD). Seventeen consecutive adult heart transplant patients, including seven in the highest-risk category, had a 100 percent survival rate. The researchers, members of a joint program between The Children’s Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, presented their findings were recently at the American College of Cardiology 65th Annual Scientific Session in Chicago.

Philadelphia Adult Congenital Heart Program

“The Philadelphia Adult Congenital Heart Program is multidisciplinary. All patients with complex CHD being evaluated for transplant automatically see both a cardiologist and a cardiac surgeon,” said Yuli Kim, MD, medical director of the Program. “Most of the patients originate from the ACHD Program, which is the destination at the Hospital of the University of Pennsylvania (HUP) for adult patients who transition from a CHOP pediatric cardiologist, and need adult care that might necessitate a heart transplant.”

“We are a stepping stone and a bridge for some of these adults at CHOP, for example from the Single Ventricle Survivorship Program to the Adult Congenital Heart Program team to transplant,” said Dr. Kim. Patients might also have a heart rhythm problem or be a sudden cardiac arrest survivor who transition to the Adult Congenital Heart Program and then to transplant,” added Dr. Kim.

About the study

The research team examined data from 17 consecutive patients with ACHD who underwent heart transplantation between March 2010 and July 2015. Patients were evaluated by a multidisciplinary team of adult and pediatric subspecialists, including experts in heart failure and transplant, ACHD cardiology, cardiac surgery, and in some cases, hepatology and pulmonary medicine.

The transplanted patients ranged in age from 23 to 57. Of the 17 patients, eight underwent a heart and liver transplant, one underwent a heart and lung transplant, and none of the patients required post-operative mechanical support. Of the eight heart and liver transplants, seven were in patients with single ventricles — as opposed to two ventricles in normal hearts — who previously underwent a Fontan, a pediatric surgical procedure used to reconfigure the heart’s circulation to maximize the efficiency of the single ventricle without overworking it. These patients are among the highest risk transplants undertaken. Researchers found that with an average follow-up of 35 months, as of September 2015, there was 100 percent survival at both 30 days and one year.

While there is little data to inform best practices in treating patients with ACHD, the researchers conclude that their integrated, team-based approach presents a promising start in treating patients with this unique physiology and anatomy.

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