Adult Congenital Heart Disease Comes of Age

At a Glance

  • It is expected that by the year 2020 more adults than children will need open-heart procedures to correct congenital heart defects.
  • Cardiologists, interventional cardiologists, cardiac surgeons, and cardiac imaging specialists at NewYork-Presbyterian have come together to establish major programs in adult congenital heart disease with a focus on transitioning children seamlessly to adult care.

“Ten years ago, nobody would touch these patients. We’re benefitting now from advances in ventricle assist devices, heart transplant, intraoperative care, and ICU postoperative care. Better ways of managing patients in surgery allow us to operate on highly complex, highly difficult cases with a good success rate.”

— Emile Bacha, MD

Adult aged patients with congenital heart disease make up a highly varied, extremely complex, and rapidly growing population. Before the development of novel surgical interventions in the 1960s, ‘70s, and ‘80s, many of these patients did not survive through infancy. Today, the number of adult congenital heart disease patients exceeds the number of pediatric aged patients with congenital heart disease, and continues to grow.

Each year, the pediatric cardiac surgeons at the Congenital Heart Center (CHC) of NewYork-Presbyterian, perform more than 700 cardiac operations, including some 25 heart transplants, 175 newborn heart repairs, and 100 adult congenital heart repairs. Under the direction of Emile A. Bacha, MD, Chief, Division of Cardiac, Thoracic, and Vascular Surgery at NewYork-Presbyterian/Columbia, and Director, Congenital and Pediatric Cardiac Surgery at NewYork-Presbyterian, CHC has been performing pediatric heart surgeries since the early 1970s.

The Congenital Heart Center is one of the first pediatric cardiology and cardiac surgery centers in the country, and one of the nation’s major pediatric transplant centers. Today CHC has a roster of highly skilled and experienced cardiovascular surgeons, including national leaders specializing in complex neonatal surgery, hybrid minimally invasive surgery, and transplant/assist devices.

In particular, Dr. Bacha cites close collaboration with cardiology colleagues as integral to caring for both pediatric and adult patients with congenital heart disease.

“Our survival rates for children with CHD are about 98 percent,” says Dr. Bacha, who is known for developing novel techniques to perform hybrid surgical-interventional catheter-based procedures for many complex congenital heart defects, including hypoplastic left heart syndrome. “Patients are now thriving and growing to adulthood.”

Adult congenital heart disease patients continue to need advanced care throughout their lives, including:

  • imaging studies of their complex anatomy
  • catheter-based interventions for amelioration of residual defects and rhythm disturbances
  • additional surgery for repair or replacement of valves that may no longer be functioning normally
  • repair of residual holes in the heart
  • replacement of artificial blood vessels implanted decades earlier that may become obstructed

Adult congenital heart disease patients are very complicated,” says Dr. Bacha. “They’ve often had two, three, and four surgeries in the past — sometimes five and six. Ten years ago, nobody would touch these patients. We’re benefitting now from advances in ventricle assist devices, heart transplant, intraoperative care, and ICU postoperative care. Better ways of managing patients in surgery allow us to operate on highly complex, highly difficult cases with a good success rate.”

Adult Centers Focused on Congenital Heart Disease

“There are about 35 or more abnormalities of the heart that can occur during fetal development,” says Marlon S. Rosenbaum, MD, Director of the Schneeweiss Adult Congenital Heart Center (SACHC) at NewYork-Presbyterian/Columbia. Dr. Rosenbaum came to Columbia in 1987 to create one of the first adult congenital heart disease programs in the country.

“Every congenital heart disease patient is different,” he says. “For example, someone born in the 1970s with the aorta and pulmonary artery attached to the wrong chambers would have had a baffle constructed within the upper chamber of the heart, which creates its own problems later in adulthood. Someone with the same anatomy born in 1990 would have had the two arteries returned to the appropriate chambers, a procedure that creates an entirely different set of potential issues.”

The research arm of SACHC, which is under the direction of Matthew J. Lewis, MD, is involved in several studies aimed at addressing those unique issues. Some projects underway include multicenter studies of adults with a failing Fontan repair and patients with a systemic right ventricle. Other research studies on the effect of pacing on Fontan outcome, outcome of patients who underwent pulmonary stenosis repair, and outcome of catheter ablation in patients with adult congenital heart disease are ongoing.

The Cornell Center for Adult Congenital Heart Disease directed by Harsimran S. Singh, MD, is a collective effort among adult and pediatric specialists, offering a comprehensive approach that includes imaging experts, cardiologists, interventional cardiologists, and cardiac surgeons.

“We follow adult patients with a wide spectrum of congenital heart problems,” says Dr. Singh. “These include cardiac defects often first discovered in adulthood, such as atrial septal defects and bicuspid aortic valve disease. We also see more complex birth anomalies that may have already required multiple surgeries in childhood, such as tetralogy of Fallot, transposition of the great arteries, coarctation of the aorta, and single ventricle anatomy or Fontan circulation.”

Surgery across the Lifespan

“Congenital heart disease accounts for almost 1 percent of all malformations in babies,” notes David M. Kalfa, MD, PhD, a pediatric cardiac surgeon at NewYork-Presbyterian/Columbia. “In recent years we have had major successes in the surgical, medical, and ICU treatments of these children, and their survival has increased impressively. Now as adults, however, many require reoperations.”

Certain congenital heart defects, such as hypoplastic left heart syndrome, require multiple surgeries in children to correct a number of heart structures that do not fully develop.

“The Norwood procedure is a three-stage heart surgery in which we create a ‘new’ aorta and connect it to the right ventricle and also place a tube from either the aorta or the right ventricle to the vessels supplying the lungs. This is a complex operation and probably has one of the highest risks of mortality and morbidity. We have been particularly successful with this surgery,” says Paul J. Chai, MD, Director of Pediatric Heart Transplantation and Mechanical Assist Device Services, and Director of Congenital Heart Surgery at NewYork-Presbyterian/Weill Cornell. “These outcomes reflect the skill of our medical and surgical teams, as well as the excellence of our postoperative care.”

Dr. Chai notes, because these patients have already undergone four or five cardiac surgeries in the childhood, by the time they require additional surgery as an adult, they have considerable amounts of scar tissue that also presents complications.

“You have to be very versatile in pediatric congenital heart surgery to be able to deal with all of these different types of patients as adults with their unique anatomy and physiology,” he says.

Role of Interventional Cardiology

While medical and surgical therapies for congenital heart disease have made significant strides in recent years, at the same time the field of interventional cardiology has also experienced rapid growth.

Robert J. Sommer, MD, Director of Invasive Adult Congenital Heart Disease at NewYork-Presbyterian/Columbia is a pediatric cardiologist who began his medical career taking care of children. Over the last 15 years, Dr. Sommer found himself transitioning to adult cardiology specifically for the management of the congenital heart disease population of patients.

“Interventions have become available for closing holes, opening up valves, and fixing obstructions in large blood vessels that were congenitally malformed,” says Dr. Sommer.

A major focus of Dr. Sommer’s expertise is treating patients who have patent foramen ovale (PFO), which has been associated with various disease processes. These include paradoxical emboli causing cryptogenic strokes or other systemic arterial occlusion events, systemic hypoxemia from right-to-left shunt, decompression sickness in divers, and migraine headaches. The presenting symptom is a neurological event, either stroke or transient ischemic attack.

“PFO is a critical component of the fetal circulation,” explains Dr. Sommer. “In most babies, this flap in the heart wall closes after birth. However, there is incomplete closure in about 25 percent of babies. The vast majority of these patients will grow up and never find out that they have a PFO. But in rare cases, these patients may present with a stroke if a clot crosses from the right side of the heart to the left side, through the PFO, and reaches the brain. Additionally, there’s a significant percentage of patients with PFOs who have migraine headaches.”

At NewYork-Presbyterian/Weill Cornell, both Dr. Singh and Geoffrey W. Bergman, MB, BS, perform structural interventions for adults with uncorrected congenital heart defects, including PFO and atrial septal defects. The main population of patients considered for PFO closure is those with cryptogenic stroke, particularly younger patients.

“Potential candidates range from teenage years up to about 60,” says Dr. Bergman. “Beyond age 60, more common causes of stroke become more predominant. However, if the workup for stroke results in no other obvious cause, then PFO must be considered.”

Dr. Rosenbaum and Dr. Singh note the importance of having an infrastructure in place to care for congenital heart disease patients in adulthood.

“For adult patients with complex congenital heart disease, adult and pediatric cardiac specialists work together to streamline the process of transition from pediatric to adult care,” says Dr. Singh. “The idea is to empower patients to achieve knowledge about their medical diagnosis and independence in their self-care. It is important for patients to understand that their pediatric doctor is not abandoning them, but rather is working together with the adult specialist as the spectrum of medical disease shifts with age. Even when they are 30 years old, there will be times when the pediatric cardiologist should be involved in clinical decision making.”

“You cannot practice adult congenital heart disease in a vacuum,” says Dr. Rosenbaum. “Patients are referred from other medical centers with multiple abnormalities that require correction. There are very few centers in the United States that can offer the level of multidisciplinary expertise in adult congenital heart disease that can be found at NewYork-Presbyterian.”

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