Tetralogy of Fallot (TOF) in Adults Guidelines

Updated: Nov 13, 2018
  • Author: Shabir Bhimji, MD, PhD; Chief Editor: Yasmine S Ali, MD, FACC, FACP, MSCI  more...
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Guidelines

Guidelines Summary

2018 AHA/ACC guidelines

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.

Diagnostic recommendations

CMRI is useful for quantification of ventricular size and function, pulmonary valve function, pulmonary artery (PA) anatomy, and left heart abnormalities in patients with repaired tetralogy of Fallot.

Obtain coronary artery compression testing before performing right ventricle-to-PA conduit stenting or transcatheter valve placement in repaired tetralogy of Fallot.

Programmed ventricular stimulation can be useful for risk stratification of adults with tetralogy of Fallot and additional risk factors for sudden cardiac death (SCD).

In patients with repaired tetralogy of Fallot, cardiac catheterization with angiography, if indicated, is reasonable to assess hemodynamics when adequate data cannot be obtained noninvasively in the setting of an arrhythmia, heart failure, unexplained ventricular dysfunction, suspected pulmonary hypertension, or cyanosis.

Therapeutic recommendations

Pulmonary valve replacement (surgical or percutaneous) for symptomatic relief is recommended for patients with repaired tetralolgy of Fallot and moderate or greater pulmonary regurgitation (PR) with otherwise unexplained cardiovascular symptoms.

Pulmonary valve replacement (surgical or percutaneous) is reasonable for preservation of ventricular size and function in asymptomatic patients with repaired tetralogy of Fallot and ventricular enlargement or dysfunction and moderate or greater PR.

Primary prevention with implantable cardioverter-defibrillator (ICD) therapy is reasonable in adults with tetralogy of Fallot and multiple risk factors for SCD.

Surgical pulmonary valve replacement may be reasonable for adults with repaired tetralogy of Fallot and moderate or greater PR with other lesions that require surgical interventions.

Consider pulmonary valve replacement, in addition to arrhythmia management, for adults with repaired tetralogy of Fallot and moderate or greater PR and ventricular tachyarrhythmia.

RV-to-PA conduit

Diagnostic recommendations

Coronary artery compression testing with simultaneous coronary angiography and high-pressure balloon dilation in the conduit is indicated before RV-to-PA conduit stenting or transcatheter valve placement.

In patients with stented RV-to-PA conduits and worsening pulmonary stenosis (PS) or PR, evaluate for conduit complications, including fluoroscopy to evaluate for stent fracture and blood cultures to assess for infective endocarditis.

In adults with RV-to-PA conduit and arrhythmia, congestive heart failure, unexplained ventricular dysfunction, or cyanosis, cardiac catheterization is reasonable to assess the hemodynamics. 

Therapeutic recommendations

RV-to-PA conduit intervention is reasonable for adults with RV-to-PA conduit and moderate or greater PR or moderate or greater stenosis with reduced functional capacity or arrhythmia.

RV-to-PA conduit intervention may be reasonable for asymptomatic adults with RV-to-PA conduit and severe stenosis or severe regurgitation with reduced RV ejection fraction or RV dilatation.