AHA: How to Care for Older Adults With Congenital Heart Disease

Marlene Busko

April 22, 2015

DALLAS, TX — A new scientific statement issued by the American Heart Association (AHA) spells out recommendations for healthcare providers treating adults over age 40 who have congenital heart disease[1].

Such patients are diverse and include those who had one or more surgeries in infancy or childhood, those diagnosed only as adults, and those with, for example, arrhythmia or heart failure superimposed on the congenital heart disease, lead author Dr Ami B Bhatt (Massachusetts General Hospital, Boston) told heartwire from Medscape.

The position statement, which was published online April 20, 2015 in Circulation, was needed because the population of adult patients with congenital heart disease is growing rapidly, she explained. This document is meant to complement the 2008 American College of Cardiology (ACC)/AHA guidelines for adult congenital heart disease.

The statement emphasizes the need to closely follow adult patients with congenital heart disease and manage their CVD risk factors. Perhaps surprisingly, "atherosclerotic cardiovascular disease risk factors—glucose intolerance, diabetes, hypertension, and a sedentary lifestyle—are all relatively significant in this population," which includes patients who may have been receiving cardiac care since birth, Bhatt noted.

Some patients may falsely believe that they have been "cured" by their childhood intervention and therefore don't need regular follow-up care, she said. They may return to see a physician only when they are in their 40s and have symptoms of heart failure or arrhythmia.

Patients with congenital heart disease may be hesitant to exercise, Bhatt added. However, "we [pediatric and adult congenital heart disease caregivers] are really trying to promote physical activity" now in people who have congenital heart disease; "we've gone from exercise restriction to prescription in a majority of patients," she said.

Moreover, adopting a healthy, proactive lifestyle can be challenging for older adults who may also have pervasive lifelong psychosocial issues, Bhatt noted. Thus, an exercise physiologist or social worker or psychologist who is involved in the care of a sedentary adult who has congenital heart disease can help encourage him or her to be active and live life to the fullest, she said.

"Over the lifetimes of these patients, there has been a significant evolution in cardiac surgery and noncardiac surgical interventions; however, there are few large clinical trials on which to base recommendations, and therefore, most recommendations are still based on expert consensus opinion," the statement authors write.

The statement has a broad scope, covering:

  • The evaluation and management of the patient with known congenital heart disease.

  • Recommendations for newly diagnosed congenital heart disease.

  • Heart failure, arrhythmias, pacemakers, and implantable cardioverter defibrillators (ICDs) in adults with congenital heart disease.

  • The impact of pulmonary disease, renal disease, and liver disease.

  • Cancer screening, gynecologic issues, sexual dysfunction, cognitive decline, psychosocial issues, and genetic screening and counseling.

  • Diagnostic testing, including imaging.

  • Residual lesions.

The statement recommends screening for pulmonary function and for sleep apnea. "Many of these patients may have dyspnea on exertion, and it's important not to underestimate pulmonary dysfunction in addition to potential cardiovascular sources of shortness of breath," according to Bhatt.

In addition, there is strong (class 1, level B) evidence for three recommendations related to liver function, she noted. All patients who had a Fontan palliative surgical procedure should have serial liver-function tests to detect progressive liver disease. All patients who had surgery for congenital heart disease before 1992 should be screened for hepatitis C. Last, clinicians need to be aware that adults with congenital heart disease, especially those who are cyanotic or those who had Fontan procedures, are more likely to develop gallstones and need a cholecystectomy.

There is also strong (class 1, level B) evidence that all adults with moderate to complex congenital heart disease should have routine renal-function tests.

Some patients with complex congenital heart disease in childhood may have stable disease as they get older, according to Bhatt. Other patients who may have had one surgery for a simple lesion may present with complications later on. "To manage these patients, it is important to not only . . . figure out the original anatomy but also think about their current physiology." A collaborative care approach with an adult congenital heart disease team and other healthcare professionals is often best, she said.

"We hope this statement serves as a foundation for the care of the older adult with [congenital heart disease] and that in the coming years, natural history studies through registries and carefully designed trials will inform future versions of this document," Bhatt and colleagues conclude. "It is encouraging for the field of [adult congenital heart disease] that the treatment of the older adult is an issue that is now upon us, and it is an exciting time, as our management strategies must now evolve along with this population of patients."

Bhatt has no relevant financial relationships. Disclosures for the coauthors are listed in the article.


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