Older Adults With Congenital Heart Disease: A Coming Tide

Due to the success of diagnosis and treatment of congenital heart disease over the last 5 decades, adults with congenital heart disease (ACHD) are a growing population. CHD in adults is most commonly diagnosed and treated in childhood, although some patients may not be diagnosed until adulthood. The number of ACHD patients in the United States is not known, but estimates suggest that there are at least a million adults with CHD, with the population growing by 5% each year as children age into adulthood.^{1, 2} There is a growing but as yet incomplete understanding of the cardiovascular issues faced by adults with CHD, including arrhythmias, heart failure, sudden death, need for repeat surgery or catheter-based interventions, or premature mortality. However, because the population is relatively new and is mostly young adults, little is known about the consequences of CHD in older adulthood. The Scientific Statement from the American Heart Association entitled “Congenital Heart Disease in the Older Adult” is a very comprehensive review of the issues that are known or anticipated to face the adults with CHD over the age of 40 years.

Issues in older adults will often be consequences of the underlying CHD and its repairs. However, particularly in older adults at risk for acquired cardiovascular disease (CVD) or other diseases seen commonly as people age (diabetes, lung disease, malignancy), the interaction between CHD and concomitant diseases may be significant and may pose management difficulties. This statement articulates concerns regarding diagnosis and management in older adults with CHD, as well as articulating the important known or anticipated differences between CHD in the older adult and CHD seen in younger patients. There is also discussion of the applicability of current treatment paradigms and guidelines for adults with acquired cardiovascular disease, prevention of CVD, and management of concomitant diseases to the ACHD population, including guidelines that likely apply and those that should be applied with caution or not at all.

Amongst the topics covered, there are sections addressing CHD-related complications in the older adult that can affect all patients, regardless of underlying CHD diagnosis, such as arrhythmias or heart failure. There is also a section that articulates complications related to specific types of CHD and repairs, such as the failing Fontan or transposition with an atrial switch repair (Mustard/Senning). They also detail the acquired CVD and non-cardiac issues that may arise in older adults with CHD, with reference to the non-cardiac conditions that are more frequently encountered in certain ACHD patients. This includes hepatic dysfunction, particularly found in those with Fontan repairs and renal dysfunction, which is encountered more commonly in patients such as those with cyanosis.

There are relatively few data on which to base diagnosis, prognosis, and management decisions in ACHD patients, especially in older adults. However, the authors discuss data that are available, but offer expert opinions on diagnosis and management when data are lacking. The disease-specific sections discuss the commonly seen complications, and emphasize those issues that are common across the population of adults with CHD that will assuredly be more common in older adults, such as arrhythmias and heart failure.

Several issues are discussed that emphasize the potential limitations of applying existing guidelines to ACHD patients without taking their specific CHD-related issues into account. For example, many of the existing arrhythmia guidelines may apply to the care of the older adult with CHD, but it is worth noting that many of the risk scores for stroke prevention in atrial fibrillation (CHADS2, CHADS2VASC, or HASBLED) did not include CHD patients, a group thought to have a higher stroke risk related to atrial fibrillation. Thus, applying those risk scores to an ACHD patient may underestimate the stroke risk and miss an opportunity to lower risk with warfarin.

Similarly, hormone replacement therapy is used for relief of menopausal symptoms, but remains controversial in terms of the balance of risk in populations of women. Some women with CHD are at a higher thrombotic risk, such as those with Fontan physiology or Eisenmenger’s syndrome, therefore when deciding on the use of hormone replacement therapy, the CHD-related thrombotic risk should be incorporated into the decision making.

There are imaging techniques used frequently in evaluation of patients with acquired heart disease, in particular newer echocardiography modalities like strain and contrast echocardiography may have use in ACHD patients. There is also a detailed discussion of echocardiographic evaluation of specific CHD issues, like the systemic right ventricle or single ventricle, as well as the valuable role of MRI in CHD.

One consistent theme throughout the statement is the need for involved ACHD expertise in the care of these patients. In 2012, ACHD was recognized as a specialty of both pediatric and internal medicine cardiology, and the inaugural board exam will be given later this fall. ACGME criteria for ACHD fellowships were released in 2015, thus there will be a training pathway allowing interested cardiologists a clear path to develop ACHD expertise. Board certification and increasing numbers of ACGME fellowships should make it easier to identify ACHD cardiologists to help in the care of these patients.

In addition to the 2008 ACC/AHA Guidelines for the Care of Adults with Congenital Heart Disease,³ there are newly published recommendations from the Heart Rhythm Society/Pediatric and Congenital Electrophysiology Society on the management of arrhythmias in the ACHD population,⁴ and the revision of the 2008 ACC/AHA Guidelines for the Care of Adults with Congenital Heart Disease are anticipated this year. While those guidelines encompass issues germane to older adults with CHD, this scientific statement provides a needed comprehensive discussion of the issues affecting the older adult with CHD, a population that is growing and will assuredly require increasingly specialized care and medical resources.

Much progress has been made in treating CHD, with a marked improvement in survival of pediatric patients with CHD and increasing understanding of the sequelae faced by adults with CHD. However, older adults with CHD are a growing but less well understood group of patients. This statement is an excellent review of the known and anticipated issues facing older adults with CHD. Very accurately, the authors identify the importance of registries and larger studies to improve our understanding and thus care of older adults with CHD.