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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editors-In-Chief: Priyamvada Singh, MBBS [2]; Cafer Zorkun, M.D., Ph.D. [3]; Keri Shafer, M.D. [4]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [5]
Overview
Recommendations[1]
Major Recommendations
The American College of Cardiology/American Heart Association (ACC/AHA) classification of the recommendations for patient evaluation and treatment (classes I-III) and the levels of evidence (A-C) are defined at the end of the "Major Recommendations" field.
Recommendations for Evaluation and Follow-Up of the Repaired Patient
Class I
- Patients with repaired tetralogy of Fallot should have at least annual follow-up with a cardiologist who has expertise in adult congenital heart disease (ACHD). (Level of Evidence: C)
- Patients with tetralogy of Fallot should have echocardiographic examinations and/or magnetic resonance imagings (MRIs) performed by staff with expertise in ACHD. (Level of Evidence: C)
- Screening for heritable causes of their condition (e.g., 22q11 deletion) should be offered to all patients with tetralogy of Fallot. (Level of Evidence: C)
- Before pregnancy or if a genetic syndrome is identified, consultation with a geneticist should be arranged for patients with tetralogy of Fallot. (Level of Evidence: B)
- Patients with unrepaired or palliated forms of tetralogy should have a formal evaluation at an ACHD center regarding suitability for repair. (Level of Evidence: B)
Recommendations for Imaging
Class 1
- Comprehensive echocardiographic imaging should be performed in a regional ACHD center to evaluate the anatomy and hemodynamics in patients with repaired tetralogy of Fallot. (Level of Evidence: B)
Recommendations for Diagnostic and Interventional Catheterization for Adults With Tetralogy of Fallot
Class I
- Catheterization of adults with tetralogy of Fallot should be performed in regional centers with expertise in ACHD. (Level of Evidence: C)
- Coronary artery delineation should be performed before any intervention for the right ventricular outflow tract (RVOT). (Level of Evidence: C)
Class IIb
- In adults with repaired tetralogy of Fallot, catheterization may be considered to better define potentially treatable causes of otherwise unexplained left ventricle (LV) or right ventricle (RV) dysfunction, fluid retention, chest pain, or cyanosis. In these circumstances, transcatheter interventions may include:
- Elimination of residual shunts or aortopulmonary collateral vessels. (Level of Evidence: C)
- Dilation (with or without stent implantation) of RVOT obstruction. (Level of Evidence: B)
- Elimination of additional muscular or patch margin ventricular septal defect (VSD). (Level of Evidence: C)
- Elimination of residual atrial septal defect (ASD). (Level of Evidence: B)
Recommendations for Surgery for Adults With Previous Repair of Tetralogy of Fallot
Class I
- Surgeons with training and expertise in congenital heart disease (CHD) should perform operations in adults with previous repair of tetralogy of Fallot. (Level of Evidence: C)
- Pulmonary valve replacement is indicated for severe pulmonary regurgitation and symptoms or decreased exercise tolerance. (Level of Evidence: B)
- Coronary artery anatomy, specifically the possibility of an anomalous anterior descending coronary artery across the RVOT, should be ascertained before operative intervention. (Level of Evidence: C)
Class IIa
- Pulmonary valve replacement is reasonable in adults with previous tetralogy of Fallot, severe pulmonary regurgitation, and any of the following:
- Moderate to severe right ventricular (RV) dysfunction. (Level of Evidence: B)
- Moderate to severe RV enlargement. (Level of Evidence: B)
- Development of symptomatic or sustained atrial and/or ventricular arrhythmias. (Level of Evidence: C)
- Moderate to severe tricuspid regurgitation (TR). (Level of Evidence: C)
- Collaboration between ACHD surgeons and ACHD interventional cardiologists, which may include preoperative stenting, intraoperative stenting, or intraoperative patch angioplasty, is reasonable to determine the most feasible treatment for pulmonary artery stenosis. (Level of Evidence: C)
- Surgery is reasonable in adults with prior repair of tetralogy of Fallot and residual RVOT obstruction (valvular or subvalvular) and any of the following indications:
- Residual RVOT obstruction (valvular or subvalvular) with peak instantaneous echocardiography gradient greater than 50 mm Hg. (Level of Evidence: C)
- Residual RVOT obstruction (valvular or subvalvular) with RV/LV pressure ratio greater than 0.7. (Level of Evidence: C)
- Residual RVOT obstruction (valvular or subvalvular) with progressive and/or severe dilatation of the right ventricle with dysfunction. (Level of Evidence: C)
- Residual ventricular septal defect (VSD) with a left-to-right shunt greater than 1.5:1. (Level of Evidence: B)
- Severe aortic regurgitation (AR) with associated symptoms or more than mild LV dysfunction. (Level of Evidence: C)
- A combination of multiple residual lesions (e.g., VSD and RVOT obstruction) leading to RV enlargement or reduced RV function. (Level of Evidence: C)
Recommendations for Interventional Catheterization
Class I
- Interventional catheterization in an ACHD center is indicated for patients with previously repaired tetralogy of Fallot with the following indications:
- To eliminate residual native or palliative systemic–pulmonary artery shunts. (Level of Evidence: B)
- To manage coronary artery disease. (Level of Evidence: B)
Class IIa
- Interventional catheterization in an ACHD center is reasonable in patients with repaired tetralogy of Fallot to eliminate a residual ASD or VSD with a left-to-right shunt greater than 1.5:1 if it is in an appropriate anatomic location. (Level of Evidence: C)
Key Issues to Evaluate and Follow-Up
Recommendations for Arrhythmias: Pacemaker/Electrophysiology Testing
Class I
- Annual surveillance with history, electrocardiogram (ECG), assessment of RV function, and periodic exercise testing is recommended for patients with pacemakers/automatic implantable cardioverter defibrillators. (Level of Evidence: C)
Class IIa
- Periodic Holter monitoring can be beneficial as part of routine follow-up. The frequency should be individualized depending on the hemodynamics and clinical suspicion of arrhythmia. (Level of Evidence: C)
Class IIb
- Electrophysiology testing in an ACHD center may be reasonable to define suspected arrhythmias in adults with tetralogy of Fallot. (Level of Evidence: C)
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