BACKGROUND AND AIMS: Right bundle branch block (RBBB) and resulting right ventricular (RV) electromechanical discoordination are thought to play a role in the disease process of subpulmonary RV dysfunction that frequently occur post-repair tetralogy of Fallot (ToF). We sought to describe this disease entity, the role of pulmonary re-valvulation, and the potential added value of RV cardiac resynchronization therapy (RV-CRT). METHODS: Two patients with repaired ToF, complete RBBB, pulmonary regurgitation, and significantly decreased RV function underwent echocardiography, cardiac magnetic resonance, and an invasive study to evaluate the potential for RV-CRT as part of the management strategy. The data were used to personalize the CircAdapt model of the human heart and circulation. Resulting Digital Twins were analysed to quantify the relative effects of RV pressure and volume overload and to predict the effect of RV-CRT. RESULTS: Echocardiography showed components of a classic RV dyssynchrony pattern which could be reversed by RV-CRT during invasive study and resulted in acute improvement in RV systolic function. The Digital Twins confirmed a contribution of electromechanical RV dyssynchrony to RV dysfunction and suggested improvement of RV contraction efficiency after RV-CRT. The one patient who underwent successful permanent RV-CRT as part of the pulmonary re-valvulation procedure carried improvements that were in line with the predictions based on his Digital Twin. CONCLUSION: An integrative diagnostic approach to RV dysfunction, including the construction of Digital Twins may help to identify candidates for RV-CRT as part of the lifetime management of ToF and similar congenital heart lesions.

Children's Heart Center 2nd Faculty of Medicine Charles University Prague and Motol University Hospital 5 Úvalu 84 150 06 Prague Czech Republic

Czech Institute of Informatics Robotics and Cybernetics Czech Technical University Prague Jugoslávských partyzánů 1580 3 160 00 Prague Czech Republic

Department of Biomedical Informatics 1st Faculty of Medicine Charles University Prague Kateřinská 1660 32 121 08 Prague Czech Republic

Maastricht University Medical Center CARIM School for Cardiovascular Diseases Universiteitssingel 50 6200 MD Maastricht The Netherlands

Europace. 2024 Feb 1;26(2):euae027. doi: 10.1093/europace/euae027. PubMed

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