Guidelines for Management of Congenital Heart Disease in ...



Guidelines for Management of Congenital Heart Disease in Adults

Experts provide recommendations for systemic and policy improvement as well as specific guidance for clinicians.

Background and Purpose: In part as a result of advances in cardiac surgery, an estimated 85% of children with congenital heart disease now survive into adulthood. In 2000, about 800,000 such individuals received care in adult healthcare systems, some of which may lack the physicians, advanced-practice nurses, and social workers sufficiently experienced in the unique problems of these patients. These guidelines were created to provide disease-specific recommendations for the care of adults with CHD (ACHD).

Key Points:

1. A need exists to strengthen transition clinics for adolescents and young adults and to recover patients with CHD lost to follow-up after leaving pediatric supervisory care.

2. Specialists in adult cardiovascular medicine require enhanced education about the pathophysiology and management of ACHD, and regional ACHD centers of excellence are needed to serve the surrounding medical community.

3. Regional ACHD centers should include experts with specific interest in and knowledge of congenital cardiology, cardiac surgery, cardiac anesthesia, echocardiography and other imaging modalities, catheterization, nursing, data management, and other subspecialties (e.g., electrophysiology, obstetrics, and pulmonary hypertension).

4. Specific types of CHD are classified as being of simple, moderate, or great complexity, with specific recommendations for the clinician training levels and patient access required to manage each condition.

5. The authors emphasize the recognition of the complex psychosocial issues encountered by individual patients and their families and the need to address these issues in patient care.

6. Prophylaxis against infective endocarditis (IE) is considered reasonable only in patients who are at the highest risk for adverse outcomes, including those with prosthetic valves, previous IE, and unrepaired cyanotic CHD.

7. A thorough evaluation of patients with ACHD should be undertaken before anticipated noncardiac surgery; this evaluation should include ECG, chest x-ray, transthoracic echocardiography, and blood work. High-risk patients should be managed at a specialized ACHD center.

8. Cardiac arrhythmias, particularly in patients with intracardiac repairs, are a major source of illness and death in ACHD. Even usually straightforward procedures, such as permanent pacemaker implantation, can be challenging because of unusual anatomy in these patients.

9. Specific counseling before pregnancy is important to ensure that the risks to both mother and baby, as well as those of transmitting CHD to offspring, are fully understood.

10. Finally, the authors provide specific disease management recommendations. Examples include closure of secundum atrial septal defect for right atrial and ventricular enlargement with or without symptoms, annual follow-up of all patients with tetralogy of Fallot, and lifelong follow-up of all patients with aortic valve disease.

Comment: These guidelines are useful to clinicians both with and without expertise in caring for adults with congenital heart disease. The guidelines help the nonspecialist to understand the unique issues of transition of care from pediatric centers to adult primary care physicians and cardiologists; and they provide the specialist with concrete, disease-specific recommendations for diagnosis, therapy, and follow-up. As an adult cardiologist with a special interest in procedural interventions for structural and valvular heart disease, I have placed this document on my desktop where I can consult it quickly.

— Howard C. Herrmann, MD

Published in Journal Watch Cardiology December 24, 2008

Citation(s):

Warnes CA et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease): Developed in collaboration with the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol 2008 Dec 2; 52:e1.

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