Guidelines for adults with congenital heart disease note changing landscape

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This patient population continues to grow

Diagnosis and treatment of congenital heart disease has improved dramatically over the last 5 decades, such that there is a growing population of adults with CHD. By some estimates, there are over a million adults with CHD and the population is growing by 5% per year. Despite the significant improvement in outcomes in children with CHD, there are significant sequelae of underlying CHD and necessary repairs that affect adults with CHD (ACHD). Common problems include arrhythmias, heart failure, sudden death, premature mortality, and complications related to other affected organs, such as hepatic or renal dysfunction. While understanding of these issues in ACHD patients as a whole is increasing steadily, most ACHD patients are young adults, thus the understanding of how CHD will impact older adults is less clear. Many of the issues related to CHD would be expected to progressively worsen through the lifespan, such that arrhythmias and heart failure may be even more prevalent in older adults, yet due to the underlying CHD may not respond to treatment in the ways expected in other adults with acquired cardiovascular disease. Additionally, the impact of comorbid diseases commonly encountered in older adults on underlying CHD will add a layer of complexity to both the CHD and the other comorbid diseases.

Dr. Karen Stout

The scientific statement from Dr. Bhatt and her colleagues is a comprehensive, detailed discussion of the issues anticipated in older adults with CHD. The statement reviews the breadth of issues in older adults with CHD, beginning with CHD-related complications that occur regardless of the specific type of CHD and followed by a discussion on issues of specific types of CHD, such as transposition of the great arteries and shunt lesions. They discuss both unoperated and operated CHD in the older adult. An important part of the document is the sections reviewing the acquired cardiovascular risks and diseases in the ACHD patient and the noncardiac issues that are important in older adults with CHD.

Throughout the document, there is an overarching theme that ACHD cardiology expertise is needed in the care of these patients. There also is a call to arms that more data are needed to better care for these patients, and that we must develop registries and larger clinical trials to improve outcomes for these patients.

Dr. Karen K. Stout is a cardiologist and professor of medicine at the University of Washington, Seattle.




New recommendations from the American Heart Association focus on the treatment of people older than age 40 years with congenital heart disease (CHD), a population that was believed to number about 850,000 in the year 2000 and is estimated to increase 5% each year.

“This improved longevity is leading to increased use of the medical system for both routine and episodic care, and caregivers need to be prepared to diagnose, follow-up, and treat the older adult with congenital heart disease,” authors led by Dr. Ami B. Bhatt wrote in a scientific statement published online April 20, 2015 in Circulation. “The predictable natural progression of CHD entities and sequelae of previous interventions must now be treated in the setting of late complications, acquired cardiac disease, multiorgan effects of lifelong processes, and the unrelenting process of aging. Despite the advances in this field, death rates in the population from 20 to [more than] 70 years of age may be twice to 7 times higher for the [adults] with CHD population than for their peers.”

Dr. Ami B. Bhatt

Dr. Ami B. Bhatt

Intended as a complement to the 2008 American College of Cardiology/AHA guidelines for ACHD (Circulation 2008;118:e714-833), the new recommendations cover the diagnosis and management of CHD in adults over the age of 40 years to summarize what is currently known “and to outline areas in which additional knowledge is critical to their care.” The scientific statement is limited to structural CHD, including coronary artery anomalies and aortopathy associated with bicuspid aortic valve disease (Circulation 2015 April 20; doi:10.1161/CIR.0000000000000204).

Working on behalf of the American Heart Association Council on Clinical Cardiology, Dr. Bhatt, who directs the adult congenital heart disease program at Massachusetts General Hospital, Boston, and her coauthors emphasized that the exposure to cardiovascular risk factors among ACHD patients is “no less problematic than with the non-CHD population. The ACHD individual may have abnormal myocardial substrate, abnormal cardiovascular physiology, abnormal anatomy, or any combination of the 3. The adverse impact of superimposed cardiovascular risk factors may well be amplified in this group, who also may already be at risk for systemic ventricular dysfunction, rhythm disturbances, and heart failure.”

In an interview, Dr. Bhatt noted that the ACHD population is distinct from both the pediatric and young adult populations with CHD and has many interactions with the health care system outside of adult congenital cardiac visits. “Therefore, this statement is written to serve as a reference for the many caregivers who will increasingly come across this population in their practice,” she said. “This includes general adult and pediatric cardiologists, electrophysiologists, interventionalists (percutaneous and surgical), cardiac imagers, as well as primary care physicians, hospitalists, and emergency medicine colleagues who need to understand and easily reference the issues and clinical challenges pertinent to this segment of the CHD population.”

The statement addresses diagnosis and management of late presentation of native disease, evolving long-term complications in disease diagnosed and/or intervened upon in childhood, and the additional burden of multiorgan dysfunction and acquired cardiovascular disease with age. Special attention is given to noncardiac involvement, including hepatic and renal disease screening and management, issues of aging including cognitive decline and sexual dysfunction, and challenging populations including those with coronary artery anomalies or superimposed pulmonary hypertension. The statement includes thorough discussions of diagnostic imaging, arrhythmia management, and surgical options in the older adult.

Among the issues addressed in the statement:

Patient medical records, especially cardiac catheterization reports, should be obtained from primary sources. “This allow[s] comprehensive evaluation of these patients,” the authors wrote. They also emphasized the importance of multidisciplinary care when needed, in a medical center where other illnesses can be managed in a setting that also is knowledgeable about CHD.

Psychosocial screening should be part of routine care of ACHD patients. This includes a team approach involving physicians, advanced practice nurses, physician assistants, psychologists, and social workers.

Physical activity is encouraged. Sedentary lifestyle is a risk factor for many older adults with CHD. Cardiopulmonary exercise testing can be used in ACHD patients to help physicians create an individualized exercise plan. Research demonstrates that a structured regimen can improve exercise tolerance in this population.

Sexual activity is reasonable for most ACHD patients. Exceptions include those who have decompensated or advanced heart failure, severe and/or significantly symptomatic valvular disease, or uncontrolled arrhythmias. Counseling must be provided by health care providers and “is useful to assist in resumption of sexual activity [especially] after an acute cardiac event, new cardiovascular disease diagnosis, or [implantable cardioverter defibrillator] implantation.”

Many men with ACHD can take erectile dysfunction drugs as long as they are not taking nitrates and as long as their condition does not preclude sexual activity. However, “the effectiveness of phosphodiesterase type 5 inhibitors has not been established in the presence of severe ventricular outflow tract obstruction.”

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