Multidisciplinary Management of Adult Congenital Heart Disease

The Philadelphia Adult Congenital Heart Center,a joint program of Penn Medicine and the Children’s Hospital of Philadelphia (CHOP),is designed to serve the unique healthcare needs of adults with congenital heart disease (ACHD).

Among less than a hundred such programs in the United States,the Center is comprised of specialists in cardiovascular surgery,anesthesiology,interventional catheterization,electrophysiology,non-invasive imaging and heart failure/ transplantation,genetics and reproductive services,collaborating to provide comprehensive inpatient and outpatient care.

In addition to surgery,the programs and services offered at the Center include the evaluation of patients for heart transplantation,the care of women requiring high-risk obstetrics and genetic research to develop better approaches to congenital heart disease.

Heart Transplantation — Between 2010 and 2011,nine patients with congenital heart disease underwent heart transplantation at the Hospital of the University of Pennsylvania,five of whom were combined heart-liver transplants for failing Fontan physiology.

High-Risk Obstetrics — The Maternal-Fetal Medicine group manages women with congenital anomalies,both repaired and unrepaired,ranging from severe aortic stenosis to tetralogy of Fallot to single ventricle physiology. The Center published the first report of successful pregnancies in two women with Hypoplastic Left Heart Syndrome in 2010.

Genetics — The Center collaborates with experts in genetics and dysmorphology at CHOP who specialize in DiGeorge Syndrome (at the “22q and You Center”),as well as channelopathies such as Long QT syndrome,and works closely with world-renowned geneticists in the field of Marfan syndrome and other connective tissue disorders at the Hospital of the University of Pennsylvania

About Adult Congenital Heart Disease

As a result of advances in cardiac surgery and medical therapies,it is estimated that there are now more than a million adults with congenital heart disease living in the United States,and that for the first time,adults with congenital heart disease outnumber children with the disorder. The spectrum of congenital heart disease varies widely from simple lesions (e.g.,isolated septal defects) to complex anomalies such as functional single ventricles. Adults with congenital heart disease require ongoing sub-specialty care and lifelong surveillance to ensure continued survival and quality of life.

Case Study

At age 22,Ms. M was referred to the Philadelphia Adult Congenital Heart Center for further evaluation and management of truncus arteriosus type A1 status post-complex right ventricular outflow tract (RVOT) reconstruction.

Surgical repair of common cyanotic congenital heart defects in infancy such as truncus arteriosus (and,more commonly,tetralogy of Fallot) often results in marked anatomic distortion of the pulmonary arteries and pulmonary valve insufficiency which can lead to right ventricular failure in adulthood (Figure 1).

During the previous year,Ms. M had developed increasing exercise intolerance and fatigue and dyspnea while walking between classes on campus. She was known to have an 80 mmHg gradient across the right ventricular outflow tract with free regurgitation;the right ventricular systolic pressures were approximately three-quarters systemic.

The Philadelphia Adult Congenital Heart Center is the only program in the region to offer a percutaneous option in select patients with congenital disease of the right ventricular outflow tract that would otherwise require surgery.

Given the complex anatomy of her RVOT reconstruction,Ms. M was offered a percutaneous pulmonary valve replacement. Subsequently,she received a Melody ^® transcatheter pulmonary valve during a two-hour,minimally invasive procedure performed by interventional cardiologists at the Philadelphia Adult Congenital Heart Center. Ms. M remained in the hospital overnight and went home the next day.

Six months after the Melody valve procedure,she returned to clinic reporting increased endurance and is now able to keep up with her friends at school. She has incorporated exercise back into her routine and walks over a mile on the treadmill daily. Repeat echocardiograms demonstrate a decrease in right ventricular size and no pulmonary insufficiency.

Melody® is a registered trademark of Medtronic,Inc.,
Minneapolis,MN.

Faculty Team

The Philadelphia Adult Congenital Heart Center team includes cardiovascular surgeons,cardiac anesthesiologists,cardiac specialists in catheterization,electrophysiology,non-invasive imaging,and heart failure/transplantation as well as genetics and reproductive services working together to provide comprehensive inpatient and outpatient care.

Medical Team

Yuli Kim,MD*†

Alex Davidson,MD†

Richard Donner,MD†

Martin St. John Sutton,MBBS*

Surgical Team

Stephanie Fuller,MD*†

Alberto Pochettino,MD*

Thomas L. Spray,MD†

Nurse Practioner

David Drajpuch,CRNP†

Patient Coordinators