Lifelong Care for Adults with Congenital Heart Disease

“Patient-Centered.” These two words encapsulate Boston Children’s Heart Center’s approach to care. When it comes to congenital cardiology, this means addressing a patient’s needs at every age and stage of life.A Continuum of CareIn the 1960s, when the Pediatric Cardiology Program at Boston Children’s Hospital was formally established, the field of congenital heart disease was largely focused on the infant and toddler years. As early interventions yielded higher success rates, patients lived longer. They blazed trails, faced new challenges and benefited from specialized care. A life-long continuum of care has since become integral to the Heart Center’s mission.

Many of our attending cardiologists follow their patients into adulthood.  We also accept new adult patients, who may be referred here by another physician or are personally seeking continued cardiac follow-up. Our specialized medical, surgical and interventional adult program is made up of surgeons and physicians who have expertise in caring for adults with congenital heart disease.

The Boston Adult Congenital Heart Service (BACH) is a joint collaboration with Brigham and Women’s Hospital. Founded in 1991 to address the varied set of challenges faced by adult congenital patients, BACH coordinates a diverse set of specialists from both institutions to care for adults with congenital heart disease (CHD). Open conversation and knowledge sharing are fundamental to BACH’s care model.

“Critical feedback between patient, family, adult congenital cardiologist, internists and pediatric cardiologists needs to occur in real time and on a continuing basis,” explains Michael Landzberg, MD, director of BACH. “What we learn from adult patients can inform care in pediatric practices and vice versa. This process of communication about practice and outcomes better informs and sets the stage for improved invention and innovation”

In the BACH group, cardiologists, physician assistants and nurses meet and follow the care of every patient, as a team. There is always someone available on call, and clinics are held at multiple locations weekly so that coming in for an appointment is convenient for adult patients with busy schedules. Everyone on the BACH team has a specific area of interest:

Dorothy Pearson, Caitlyn O’Brien Joyce, and Nancy Barker, PA-Cs, William Kerr, RN, and administrative associates Lauren Serge-Ryan and Samantha Buechner each add their own vital expertise and passion as integral members and leaders within BACH’s programming efforts.

Central to BACH’s mission is increasing awareness and education about the importance of lifelong follow-up for congenital heart disease. Today there are more adults living with congenital heart disease than children. However, it is estimated that 90-95 percent of adults with CHD do not receive care from adult CHD specialists; as many as 50 percent of these adults may not be seeing any cardiologist at all. Addressing this issue is becoming more and more important as the adult CHD patient population ages. Increasingly complex and diverse medical needs are emerging, and the potential for loss of quality and performance has become evident.

Our commitment to ensuring the highest quality congenital cardiology care for adult patients extends far beyond the walls of Boston Children’s. We serve as an industry leader in education, innovation and the practice of care. The BACH service was instrumental in partnering with patients and families in the creation of the Adult Congenital Heart Association (ACHA), which is now one of the most effective patient advocacy groups in America. BACH physicians sustain this connection as members and leaders of the ACHA’s medical advisory board.

“Part of what we do is work with adult and pediatric cardiologists, subspecialists, internists and primary care doctors around the region who seek guidance in treating young and older adults with congenital heart disease,” says Landzberg. “It is a personal affront to us if anyone in our region (and country) does not get adequate care for his or her congenital heart condition.”

Our Holistic Approach

Numerous body systems can be affected by a life with heart disease. Aortic health, arrhythmias, pulmonary health, and vascular health are common problem areas. Some patients also experience psychological issues and/ or diminished exercise ability. Adult congenital cardiologists must therefore not only consider the structural aspects of the heart, but also the physiologic implications of long-term cardiac disease. “Many of the anatomic or structural components are impacted upon from an early age, so we often focus on the residua and sequelae of the disease,” Landzberg explains.

Sometimes, however, the structure and function of an adult heart do need to be surgically corrected. For this reason, adult patients may receive a supplemental consultation from Sitaram Emani, MD, Surgical Director of the Adult Congenital Heart Program. In cases where further structural correction is beneficial, Emani has an edge: He is intimately familiar with the original procedure any adult patient with congenital heart disease has had, because he still performs these kinds of early interventions on infants and children. This knowledge is invaluable when repeat surgery is necessary. “Every patient is different,” Emani notes, “and we tailor our approach to the individual needs.”

Emani credits the department’s “culture of innovation” for advancing the success of new surgical techniques, particularly the “hybrid” procedures that are performed in conjunction with cardiologists in the catheterization laboratory. In cases where the heart has suffered so much damage that open-heart surgery is extremely risky, we have used a minimally invasive hybrid approach to replace the pulmonary and aortic valves.

Boston Children’s has made it a point to be appropriately equipped to treat adult patients with congenital cardiac disease. Adult beds on our inpatient floors are set back from the pediatric rooms, and we use a variety of technologies specific to adults in the operating room and the Cardiac Intensive Care Unit (CICU). Specialists in cardiac anesthesia, physicians managing patients in the CICU, and nurses focusing on improving the experience of adults with congenital heart disease who require inpatient care. But the Boston Children’s experience retains its essential quality for adults and children alike. Emani notes that many of his adult patients appreciate the extra attention paid to their individual concerns, questions and comfort levels. In his own words: “We treat our adults as tenderly as we treat our children.”