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AHA issues recommendations for older adults with congenital heart disease
The increasing population and improved longevity of adults with congenital heart disease prompted the American Heart Association to release a scientific statement for health care providers who treat patients older than 40 years with congenital heart disease.
The statement encourages physical activity, declares that sexual activity is reasonable for most adults with congenital heart disease, and alerts clinicians that these patients may be at elevated risk for HF, valvular heart disease, pulmonary hypertension and arrhythmia disorders.
Patients living longer
“People born with congenital heart disease are living longer and fuller lives than ever before, and there are now more adults than children with congenital heart disease,” statement author Ami Bhatt, MD, director of the adult congenital heart disease program at Massachusetts General Hospital, said in a press release. “These patients often have a sense that their heart has been ‘fixed’ and they don’t need follow-up. However, in adulthood they can develop complications from the underlying disease or the heart repairs that kept them alive as children, therefore, these adults need lifelong cardiac care.”
Because these patients may have abnormal myocardial substrate, coronary physiology and/or coronary anatomy, “primary prevention for acquired CAD with a thorough assessment for and approach to risk factor management is recommended in this population,” according to the statement.
Patients with moderate to complex congenital heart disease are at risk for HF, so “clinicians should emphasize early referral to an [adult congenital heart disease] center to initiate a plan for [HF] prevention or treatment,” the authors wrote.
The group made many recommendations on diagnosis and treatment of arrhythmias, including that patients older than 40 years with many congenital heart conditions should receive routine ECGs and be monitored periodically for dysrhythmias, and that anticoagulation with warfarin should be initiated in any adult older than 40 years with congenital heart disease and sustained atrial fibrillation, regardless of the presence of traditional risk factors.
Certain adults with congenital heart disease also should be monitored closely for pulmonary arterial hypertension, and treatment with pulmonary arterial vasodilator drugs can be useful, Bhatt and colleagues wrote.
Patients with a history of previous palliation with the Fontan procedure should receive serial evaluation of liver function, and adults with moderate to complex congenital heart disease should undergo routine assessment of renal function.
Women with congenital heart disease struggling with menopausal symptoms may benefit from vaginal estrogen therapy if they have vaginal dryness, and thrombotic risks should be assessed before initiation of hormone therapy, according to the statement.
Sexual activity usually reasonable
In other recommendations, sexual activity is reasonable in this population for patients who do not have decompensated or advanced HF, severe and/or highly symptomatic valvular disease or uncontrolled arrhythmias.
Use of erectile dysfunction drugs in men with congenital heart disease is safe in those for whom sexual activity is reasonable, as long as they are not also taking nitrates, the authors wrote.
All patients in this population should be encouraged to attain an ideal body weight, eat healthy and engage in regular physical activity.
Foundation for care
“We hope this statement serves as a foundation for the care of the older adult with [congenital heart disease],” Bhatt and colleagues wrote. “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.” – by Erik Swain
Disclosure: Bhatt reports no relevant financial disclosures. Other members of the writing group report financial ties with Abbott Vascular, Actelion Pharmaceuticals, Biotronik, Gilead, Novartis and Siemens Healthcare.
Perspective
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In my opinion, the most important point raised in this document is the importance of utilizing adult congenital heart disease specialists in the care of patients with congenital heart disease, particularly those with more complex disease or those who are newly diagnosed and need clinical intervention. We finally have data that support that patients seen in adult congenital heart disease clinics have improved clinical outcomes, and so these statements are now “evidence-based.”
The recommendations with the most potential to change clinical practice are those that meld the problems related specifically to the congenital heart disease lesion and the non–congenital-heart-disease-related health concerns. We finally have a document that addresses what adult congenital heart disease experts know — that these patients have many different organ systems affected by their disease, not just the heart. We have to pay attention to many things, including their CV risks, as many will develop the same problems that plague most of our non–congenital-heart-disease patients — CAD, peripheral vascular disease and cerebrovascular disease, albeit a decade or two earlier. This is why it is specified in this document that “older” adults are those that are over the age of 40 years. While at first glance that may seem young — and as someone who is 46 years of age, almost offensive — it is important to recognize that these patients are often high-risk patients who ideally need to have their risks appropriately addressed at a younger age than most other patients.
There are still many research gaps. Many recommendations have a level of evidence of C. This means that the statement is mainly based on consensus opinion of the document writers and not based on randomized clinical trials; often, not even basic clinical studies have been done in these areas. That’s what makes practicing adult congenital heart disease medicine so much of a challenge and also so exciting. There are also areas the guideline writers chose not to address in this document, such as pregnancy. As these patients get into adulthood, and even late adulthood, these types of questions surface. Can I safely have a child? What is the risk to my baby if I get pregnant? What is the risk of my child having congenital heart disease? These are all questions that each of us practicing in the field need to be able to answer. They await further documents for a consensus opinion.
This is a well-written document. I am excited by how many studies are now included in the writing of the document. Unlike the last set of guidelines, much of this literature is very new. The field of adult congenital heart disease research is very much alive and vibrant. It is a great time to practice in this field and also to participate in research related to this field.