Repaired tetralogy of Fallot across adulthood: from right ventricular remodelling to targeted intervention

Adults with repaired tetralogy of Fallot (rTOF) constitute a rapidly growing adult congenital heart disease population with excellent early survival but substantial late morbidity. Chronic pulmonary regurgitation and/or residual right ventricular outflow tract obstruction drive progressive right ventricular dilatation, dysfunction, dyssynchrony, and myocardial fibrosis, predisposing to heart failure and ventricular arrhythmias. This review summarizes contemporary concepts in the surveillance and management of adults with rTOF, focusing on multimodality imaging, pulmonary valve replacement (PVR), arrhythmia risk stratification, aortopathy, and emerging pharmacological strategies. Echocardiography remains the first-line modality for assessing residual lesions and aortic dimensions, while cardiac magnetic resonance is central for quantifying right ventricular volumes, regurgitant fraction, and fibrosis; computed tomography complements anatomical evaluation when magnetic resonance imaging is not feasible. PVR, increasingly performed using transcatheter techniques, is a cornerstone intervention, with guideline-based decisions integrating symptoms, right ventricular volumes, functional testing, and ventricular function. Arrhythmia management combines risk stratification, catheter ablation, implantable cardioverter-defibrillators in selected patients, and individualized medical and pacing therapies. Aortic root dilatation is common, but dissection is rare; surveillance and surgery are guided by diameter thresholds and growth rate. Evidence for routine renin-angiotensin-aldosterone inhibition to improve right ventricular function remains limited, although selected agents and exercise-based interventions are under investigation. Infective endocarditis risk differs by pulmonary valve replacement modality and valve type, being generally higher after transcatheter than surgical replacement, and warrants individualized antiplatelet therapy and vigilant surveillance after implantation. Lifelong multidisciplinary follow-up in specialized adult congenital heart disease centers is essential to optimize reintervention timing and reduce arrhythmic and aortic complications in adults with rTOF. Graphical abstract summarises the overall pathophysiology and long-term management of repaired tetralogy of Fallot.