The following are key points to remember from an American Heart Association scientific statement on the long-term management of right ventricular outflow tract (RVOT) dysfunction in repaired tetralogy of Fallot (TOF):

1. The severity of pulmonary regurgitation (PR) after repair of TOF is determined by: a) regurgitation orifice area, b) right ventricular (RV) compliance, c) diastolic pressure difference between the main pulmonary artery (PA) and the RV, d) capacitance and resistance of the PA system, and e) duration of diastole.
2. PR regurgitant fraction can increase over time because of increased size and capacitance of the central PAs and RV compliance, as well as the prolongation of diastole with slower heart rates associated with increasing age.
3. With long-standing PR, the right ventricle initially compensates, but eventually there are failure of compensatory mechanisms with increasing end-diastolic volume and ultimately irreversible myocardial injury and decreased function.
4. Electrocardiography, ambulatory cardiac monitoring, exercise testing, and echocardiography all have an important role in the follow-up of adults with TOF and RVOT dysfunction.
5. Cardiac magnetic resonance (CMR) is the reference standard for the assessment of RV size and function. Goals of the CMR study include: a) quantification of biventricular volumes, function, and mass; b) characterization of regional wall motion abnormalities; c) assessment of RVOT and PA morphology; d) measurement of PR volume and fraction; e) measurement of tricuspid regurgitation; f) identification of residual shunts; g) delineation of proximal coronary arteries; and h) measurement of myocardial viability for presence of focal scar.
6. Pulmonary valve replacement (PVR) generally results in significant reductions in RV end-diastolic and end-systolic volumes as well as symptomatic improvement, as demonstrated by improvement in New York Heart Association functional class. Studies have not consistently demonstrated improvement in ventricular function, objective exercise capacity, and arrhythmia risk.
7. Both surgical and transcatheter approaches are reasonable for PVR. The decision as to which type of valve should be made in a multidisciplinary fashion, while considering a patient’s lifelong need for repeat procedures.
8. The authors propose a framework for clinical decision-making in patients with repaired TOF. The risk criteria include INDICATOR risk score ≥3, RV ejection fraction ≤46%, left ventricular ejection fraction ≤50%, Peak VO₂ ≤70% predicted, and RV systolic pressure ≥2/3 systemic. The authors recommend that asymptomatic patients with one or more of these criteria be considered for PVR. Asymptomatic patients with ≥2 criteria should be considered for PVR.
9. The indicator score includes age at evaluation, obesity, repair with RV-to-PA conduit, repair type other than RV-to-PA conduit or transannular patch, RV end-systolic volume index, and biventricular global function index.
10. All patients with repaired TOF require lifelong surveillance. Frequency of follow-up and appropriate testing are based on age and acuity of illness, as designated by physiological stage.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, Interventions and Imaging, Interventions and Structural Heart Disease

Keywords: Cardiac Surgical Procedures, Diagnostic Imaging, Heart Defects, Congenital, Tetralogy of Fallot

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