What are the AHA/ACC guidelines on tetralogy of Fallot (TOF) in adults?

Updated: Nov 13, 2018
  • Author: Shabir Bhimji, MD, PhD; Chief Editor: Yasmine S Ali, MD, FACC, FACP, MSCI  more...
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The American Heart Association/American College of Cardiology (AHA/ACC) released updates to their 2008 guideline for the management of adults with congenital heart disease (CHD) in August 2018. [43, 43] Their recommendations for tetralogy of Fallot (TOF) are outlined below.

Management and care of patients with tetralogy of Fallot (repaired or unrepaired) should involve a cardiology with expertise in CHDs. Interventions involving congenital heart lesions (cardiac surgery, catheter-based interventional cardiac procedures, electrophysiologic procedures) in adults with CHD should be performed by those with expertise in adult CHD procedures as well as that of a cardiologist specializing in adult CHD.

The AHA/ACC antomic/physiologic (AP) classification categorizes repaired tetralogy of Fallot to be of moderate complexity.

Experts in imaging with ultrasonography, echocardiography, and cardiac magnetic resonance imaging (CMRI) are preferred for cardiac imaging in patients with tetralogy of Fallot. 

Use a standard 12-lead electrocardiogram (ECG) in adults with CHD with serial assessment based on the specific CHD AP classification or when symptoms develop or worsen. Use ambulatory ECG monitoring in patients with CHD who are at risk for tachyarrhythmia, bradyarrhythmia or heart block, of when symptoms of a potential arrhythmic etiology develop.

Use ECG to measure QRS duration in patients following repair of tetralogy of Fallot and as part of the evaluation for cardiac resynchronization therapy (CRT).

CMRI, cardiac computed tomography (CCT) scanning, transesophageal echocardiography (TEE), and/or cardiac catherization may be superior to transthoracic echocardiography (TTE) in the assessment of right ventricular (RV) size and function in repaired tetralogy of Fallot, systemic RVs, and other conditions associated with RV volume and pressure overload.

Tetralogy of Fallot among other cardiac findings is commonly associated with DiGeorge (velocardiofacial syndrome) and Down syndromes.

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