Dear Colleagues,

Although the survival rate after Tetralogy of Fallot repair in infants has greatly increased, reaching over 95–97% with survival after 20 years of over 90%, the pulmonary regurgitation consequence of the right ventricular outflow tract reconstruction can lead to serious problems in the long-term follow-up of these patients. Indeed, even if the majority of cases are asymptomatic and those affected live a satisfactory life, the chronic volume overload can lead to right ventricle (RV) dilation, which in turn can determine biventricular dysfunction, heart failure symptoms, arrhythmias, and sudden death. Therefore, they need to undergo a further operation for the replacement of the pulmonary valve (PVR) in a certain moment of their life to avoid these adverse outcomes. PVR is not a risk-free procedure and the prosthetic valves currently available have a limited lifespan. Therefore, it would be desirable to perform PVR in the asymptomatic patient, just before the onset of ventricular dysfunction, if we could know at what threshold of RV dilation it arises. In addition, it is still not fully understood at what threshold of dysfunction, whether it is right or left, the percentage of having a fatal arrhythmia, significantly increases. Consequently, it continues to be complicated in asymptomatic repaired tetralogy of Fallot, to choose the right timing between a potentially fatal arrhythmia and/or a not-recoverable ventricular dysfunction and an early PVR with the increased risk related to repeated prosthetic valve replacement and endocarditis. Hence, this Special Issue will highlight recent advances in support of the assessment of biventricular function (echocardiographic and cardiac magnetic resonance strain) and the objective evaluation of the functional capacity (cardiopulmonary exercise testing).

Dr. Benedetta Leonardi
Dr. Lorenzo Galletti
Guest Editors

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• Right ventricular dysfunction
• Left ventricular dysfunction
• Cardiopulmonary exercise test
• Pulmonary valve replacement