How Mass General Is Advancing the Care of Adult Congenital Heart Patients
In This Article
- More and more pediatric patients with complex congenital heart disease are surviving into adulthood
- Mass General's Adult Congenital Heart Disease (ACHD) Program specializes in caring for this growing population
- Through its Pulmonary Valve Program, the ACHD Program is leveraging both transcatheter and surgical approaches to offer pulmonary valve replacement to a rising numbers of patients
Historically, pediatric patients with complex congenital heart disease rarely survived childhood. Today, more and more of these individuals are not only living longer but thriving as adults.
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The Adult Congenital Heart Disease (ACHD) Program at Massachusetts General Hospital offers personalized, lifelong care for adult congenital heart patients, who constitute the fastest-growing population in need of cardiac interventions today. This comprehensive, high-volume practice is led by cardiologist Christopher Learn, MD, medical director, and cardiac surgeon Jordan Bloom, MD, MPH, surgical director.
Congenital heart conditions such as pulmonary stenosis or tetralogy of Fallot often lead to pulmonary valve problems starting in early adulthood. Through its Pulmonary Valve Program, the ACHD Program is leveraging both transcatheter and surgical approaches to offer pulmonary valve replacement (PVR) to rising numbers of these patients.
As part of the Pulmonary Valve Program, Dr. Bloom and interventional cardiologist Ada Stefanescu Schmidt, MD, MSC, have a joint clinic (co-located or sequential appointments) to evaluate patients, offer personalized approaches, and help them arrive at a decision on treatment. Necessary imaging tests can be done as part of the same visit; most commonly, a high-quality and low-radiation cardiac CT angiography focused on the right ventricle and pulmonary anatomy is obtained to support planning for either intervention.
According to Dr. Bloom, patients who need PVR are generally young, have already had one or more operations, and may need additional interventions in the future. He and his colleagues take a collaborative, multidisciplinary approach to reach a consensus on what strategy makes sense for each patient: transcatheter or surgical PVR.
"There are clear data showing that surgical PVR is more durable, but transcatheter PVR is a much easier thing for the patient to go through," Dr. Bloom says. "Taking their personal preferences into strong consideration, we create a bespoke strategy for the patient and present them with our recommendation."
Collaboration Within a Multidisciplinary Team
A hallmark of the Pulmonary Valve Program — and the ACHD Program as a whole — is close collaboration among a wide range of adult specialists, including:
- Cardiologists
- Interventional cardiologists
- Cardiac surgeons
- Cardiac anesthesiologists
- Electrophysiologists
- Radiologists and sonographers who specialize in congenital heart imaging
- Maternal-fetal medicine specialists
"There is an important movement to have adult congenital heart patients cared for in adult centers because you have the infrastructure of adult specialists, imagers, and equipment around you," says ACHD cardiologist Doreen DeFaria Yeh, MD. "When patients hit their early twenties and need to choose who their adult doctors will be, they benefit from being in an adult hospital with adult expertise and specialists who can help them think about their heart health for the next 70 years of their lives. This includes considerations around optimizing prevention strategies for future issues like heart failure."
Members of the ACHD Program's care team meet virtually each week to carefully review patient cases. Each patient's primary cardiologist is invited to participate in the conversation, along with other specialities as appropriate.
A common reason to need pulmonary valve intervention is significant pulmonary valve regurgitation, which can cause dilation and dysfunction of the right ventricle, reduce exercise capacity, and cause fatigue. The size and function of the right side of the heart is followed by imaging such as echocardiography, CT, and cardiac MRI. These tests can be done locally and reviewed by expert imagers at Mass General, such as ACHD cardiologist Sihong Huang, MD, and experienced cardiac radiologists. However, since certain dedicated protocols are sometimes preferred, these tests can alternatively be done at one of the many Mass General Brigham imaging locations.
The ACHD Program regularly collaborates with Mass General's Cardiovascular Performance Program to conduct tests to gauge a patient's exercise capacity and energy level following a procedure. Program faculty also work closely with recreational and professional athletes to support them in reaching their exercise goals safely in the context of their heart condition.
Since many of their patients are of childbearing age, the ACHD team often must determine the proper timing for an intervention—either before or after a pregnancy. Colleagues in Mass General Obstetrics and Maternal-Fetal Medicine are always part of these discussions. "The care of the pregnant patient and family planning are important factors as we think about the timing of procedures," Dr. DeFaria says.
Caring for adult congenital heart patients longitudinally is a point of emphasis for the ACHD Program. While its patients skew younger, they are likely to develop cardiology problems like high cholesterol, high blood pressure, diabetes, heart attacks, and heart failure in the future.
"It's critical for this population to get lifelong adult cardiology care, and that's what we specialize in at Mass General Brigham Cardiology," Dr. DeFaria says.
Driving Advances in Transcatheter Pulmonary Valve Replacement
Mass General's ACHD Program is one of the few in the country with two adult interventional congenital cardiologists: Dr. Stefanescu Schmidt and Ignacio Inglessis-Azuaje, MD.
"In addition to innovative congenital and structural heart disease techniques, Dr. Inglessis-Azuaje and I are very knowledgeable about how the tools and techniques for treating coronary or peripheral artery disease can be used to treat congenital heart disease," Dr. Stefanescu Schmidt says. "As adult congenital heart disease specialists with busy clinical practices, we both have a good perspective on the lifelong trajectory of ACHD patients, how to take care of them in the clinic, and when interventional tools are most helpful."
With the FDA approval of self-expanding transcatheter pulmonary valves in 2021, Mass General has been able to offer this minimally invasive option to many more patients, Dr. Stefanescu Schmidt notes.
"After a transcatheter pulmonary valve replacement, most patients already feel they are breathing better by the evening of the procedure, leave the hospital the next day, get back to work within a few days, and gradually return to exercise and activity," Dr. Stefanescu Schmidt says. "It's gratifying to see there are both immediate and long-term benefits in terms of the heart getting healthier over time."
The ACHD Program participates in the COMPASSION S3 trial, which investigates the use of the SAPIEN 3 Ultra transcatheter heart valve in the pulmonary position for patients with congenital heart disease, and in other investigational device trials for valves and devices only available as part of clinical studies.
Dr. Stefanescu Schmidt is working on multiple other fronts to improve the care of transcatheter PVR patients. She was lead author of 2024 papers on balloon-expandable valves in JACC: Cardiovascular Interventions and the Canadian Journal of Cardiology and is part of the two large national registries of transcatheter PVR. This year, she was an invited speaker at the American College of Cardiology, the Society for Cardiovascular Angiography and Interventions, CSI:Frankfurt, and the annual conference of the International Society for Adult Congenital Heart Disease. She is also scheduled to speak at the World Congress of Pediatric Cardiology and Cardiac Surgery in December.
A High-Volume Center for Surgical Pulmonary Valve Replacement
Dr. Bloom regularly collaborates with Dr. Stefanescu Schmidt in seeing patients as well as educating providers nationally about PVR options. The hallmark of his approach to surgical PVR is the use of acellular human pulmonary homografts rather than prosthetic valves made from porcine or bovine tissue.
"Introducing all-human tissue is the best way to avoid infection and optimize flowthrough because there's no prosthetic material," he says. "The failure rate for homografts is about 1% per year, which is far more durable than any prosthetic valve, whether it's transcatheter or open. And since we generally oversize these homografts because they can shrink over time, they make a nice home for Dr. Stefanescu Schmidt to put a transcatheter valve inside later should the patient ever develop stenosis or regurgitation."
Homografts are more difficult to implant than prosthetic valves, Dr. Bloom notes. As the sole surgeon at Mass General performing surgical PVR, he has developed the extensive experience needed to achieve elite status in homograft implantation. Dr. Bloom has implanted nearly 200 homografts in the last three years, placing the ACHD Program among a small group of high-volume programs nationally. For the isolated PVR cohort, the program had zero mortality and an average length of stay of five days.
Dr. Bloom looks forward to seeing how the PVR field continues to evolve and incorporating new practices into the ACHD Program.
"Dr. Stefanescu Schmidt and her team have saved many patients from open-heart operations, but transcatheter PVR is still pretty young compared to surgical PVR," he says. "We're hoping that some of the newer valves available for transcatheter PVR will have better durability and lower infection rates. This is an active space, and it's exciting to be in it."