Download Guidelines for Management of Congenital Heart Disease in Adults

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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