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Guidelines for adults with congenital heart disease note changing landscape

Publish date: April 20, 2015

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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.


 

FROM CIRCULATION

References

• 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.

dbrunk@frontlinemedcom.com


On Twitter @dougbrunk

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