Adult congenital heart disease (ACHD) was the topic of the 47th Annual Louis F. Bishop Lecture presented Saturday by Carole A. Warnes, MD, FACC, of the Mayo Clinic.

Her lecture, “Adult Congenital Heart Disease: the Challenge of a Lifetime,” highlighted the challenges faced by surgeons, cardiologists and ACHD patients and the need for advanced education and training resources for all three of these groups.

More than one million U.S. adults have congenital heart disease (CHD), noted Warnes, and the population is growing. Cardiologists are seeing more ACHD patients in their practices, but often have very little training or education about the disease. ACHD patients are rarely referred to specialists in a timely manner.

One of the greatest challenges ACHD patients face, noted Warnes, is the fact that they believe they were “cured” in childhood – and many regular cardiologists may believe that too.

In the 1940s and 1950s, 25 percent of babies born with CHD would die as newborns, 60 percent would die in their first year and 15 percent would not survive past puberty. As the surgeries improved, along with anesthesia, intensive care, and noninvasive diagnosis, the mortality rate decreased, “and this is why we have so many adults with CHD today,” said Warnes.

While many ACHD patients believe that because they had successful surgeries as children, they are “fixed.” Warnes says that is rarely the case. “When a reoperation is needed, it comes as a huge surprise,” she said. “They may need a fourth or fifth operation when they thought they were cured by the first.” ACHD patients over the age of 40 face the same health issues that all people over 40 face – with added complications like arrhythmia, which, says Warnes, “skyrockets” in ACHD patients over 40. “General cardiologists may not realize that a patient’s arrhythmia comes from CHD,” she noted, which means the patient may not receive the appropriate treatment. Additional complications include endocarditis, pulmonary vascular disease and ventricular dysfunction.

The biggest challenge facing surgeons today is reoperation, which is often impeded by complications like progression of trivial lesions, degeneration of valve replacements and conduits and calcification of abnormal valves.

Many ACHD patients face the challenge of being “unaware, uninformed and uneducated,” said Warnes. “These patients are extraordinarily courageous – but they don’t have good advice or education so sometimes they just don’t know,” said Warnes. “They have to be their own advocates but they are not prepared.” In addition to dealing with complications from CHD, patients face huge insurance obstacles. Doctors have to spend a lot of time writing letters on behalf of their patients to insurance companies, workplaces, and a number of other entities.

The priority today, Warnes asserted, is to educate cardiologists and patients about the continuing challenges of ACHD. “We must bridge the gap between pediatric and ACHD physicians,” she explained. General cardiologists need to understand the complexities of CHD. “Cardiologists must work together to create an open exchange of information,” and commit to tracking and measuring results. She added that it needs to be easier for cardiologists to refer CHD patients.

Warnes closed by noting that many ACHD patients feel that their condition has been a positive force in their lives, giving them the gifts of resilience, compassion and empathy, a sense of what’s important, and a sense of the value of life.

Keywords: ACC Publications, ACC Annual Scientific Session

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