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.”

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.”

  The use of hormone replacement therapy by women with ACHD must consider the risk for thromboembolic disease as well as the severity of menopausal symptoms. “For example, women with Fontan surgery have a high risk of venous thromboembolism and should avoid HRT, whereas women with [tetralogy of Fallot] repair and good RV function have a low risk and could probably receive HRT for symptoms,” the authors wrote.

The statement also includes recommendations for clinicians treating ACHD patients regarding screening for and management of concomitant lung, kidney, or liver disease. For example, it recommends serial evaluation of liver function for all patients with a history of previous palliation with the Fontan procedure and routine assessment of renal function for all adults with moderate-to-complex CHD.

The information provided in the AHA statement is based on scientific research and combined clinical experience from longitudinal care, Dr. Bhatt said in the interview. “The authors engaged in a truly multidisciplinary effort as pediatric and adult cardiologists, cardiac subspecialists, radiologists, and surgeons worked together to create a document to assist caregivers in meeting the needs of this challenging and growing population,” she said. “Importantly, by sharing the clinical trajectory of the older adult with CHD, the authors hope this statement and future versions will inform pediatric and young adult care and research as we strive to together improve lifelong care in congenital heart disease.”

Five of the coauthors disclosed relevant financial relationships. Dr. Michael C. Earing has received honoraria from Actelion Pharmaceuticals. Dr. Elyse Foster has received a research grant from Abbott Vascular and is a consultant or advisory board member for Gilead. Dr. Brian B. Ghoshhajra is a consultant or advisory board member for Siemens Healthcare. Dr. Seema Mital is a consultant or advisory board member for Novartis. Dr. Zian H. Tseng has received honoraria from Biotronik. The remaining authors reported having no relevant financial disclosures. On Twitter @dougbrunk