Michela Casella1, Alessio Gasperetti1, Fassini Gaetano1, Mattia Busana2, Elena Sommariva3, Valentina Catto1, Rita Sicuso1, Stefania Rizzo4, Edoardo Conte5, Saima Mushtaq5, Daniele Andreini5, Luigi Di Biase6, Corrado Carbucicchio1, Andrea Natale7, Cristina Basso4, Claudio Tondo1,8, Antonio Dello Russo9. 1. Dipartimento di Aritmologia, Centro Cardiologico Monzino IRCCS, via Carlo Parea 4, 20100 Milano (MI), Italy. 2. Department of Anesthesiology, Emergency and Intensive Care Medicine, University of Göttingen, Göttingen, Germany. 3. Unit of Vascular Biology and Regenerative Medicine, Centro Cardiologico Monzino IRCCS, Milano (MI), Italy. 4. Cardiovascular Pathology Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, Azienda Ospedaliera-University of Padua, Padova (PD), Italy. 5. Dipartimento di Imaging Cardiovascolare, Centro Cardiologico Monzino IRCCS, Milano (MI), Italy. 6. Montefiore Medical Center, Albert-Einstein College of Medicine, Bronx, NY, USA. 7. Texas Cardiac Arrhyhtmia Institute (TCAI) at St. David's Hospital, Austin, TX, USA. 8. Department of Clinical Sciences and Community Health, University of Milan, Milano (MI), Italy. 9. Cardiology and Arrhythmology Clinic, Marche Polytechic University, University Hospital "Ospedali Riuniti", Ancona (AN), Italy.
Abstract
AIMS: To provide long-term outcome data on arrhythmogenic cardiomyopathy (ACM) patients with non-classical forms [left dominant ACM (LD-ACM) and biventricular ACM (Bi-ACM)] and an external validation of a recently proposed algorithm for ventricular arrhythmia (VA) prediction in ACM patients. METHODS AND RESULTS: Demographic, clinical, and outcome data were retrieved from all ACM patients encountered at our institution. Patients were classified according to disease phenotype (R-ACM; Bi-ACM; LD-ACM). Overall and by phenotype long-term survival were calculated; the novel Cadrin-Tourigny et al. algorithm was used to calculate the a priori predicted VA risk, and it was compared with the observed outcome to test its reliability. One hundred and one patients were enrolled; three subgroups were defined (R-ACM, n = 68; Bi-ACM, n = 14; LD-ACM, n = 19). Over a median of 5.41 (2.59-8.37) years, the non-classical form cohort experienced higher rates of VAs than the classical form [5-year freedom from VAs: 0.58 (0.43-0.78) vs. 0.76 (0.66-0.89), P = 0.04]. The Cadrin-Tourigny et al. predictive model adequately described the overall cohort risk [mean observed-predicted risk difference (O-PRD): +6.7 (-4.3, +17.7) %, P = 0.19]; strafing by subgroup, excellent goodness-of-fit was demonstrated for the R-ACM subgroup (mean O-PRD, P = 0.99), while in the Bi-ACM and LD-ACM ones the real observed risk appeared to be underestimated [mean O-PRD: -20.0 (-1.1, -38.9) %, P < 0.0001; -22.6 (-7.8, -37.5) %, P < 0.0001, respectively]. CONCLUSION: Non-classical ACM forms appear more prone to VAs than classical forms. The novel prediction model effectively predicted arrhythmic risk in the classical R-ACM cohort, but seemed to underestimate it in non-classical forms. Published on behalf of the European Society of Cardiology. All rights reserved.
AIMS: To provide long-term outcome data on arrhythmogenic cardiomyopathy (ACM) patients with non-classical forms [left dominant ACM (LD-ACM) and biventricular ACM (Bi-ACM)] and an external validation of a recently proposed algorithm for ventricular arrhythmia (VA) prediction in ACMpatients. METHODS AND RESULTS: Demographic, clinical, and outcome data were retrieved from all ACMpatients encountered at our institution. Patients were classified according to disease phenotype (R-ACM; Bi-ACM; LD-ACM). Overall and by phenotype long-term survival were calculated; the novel Cadrin-Tourigny et al. algorithm was used to calculate the a priori predicted VA risk, and it was compared with the observed outcome to test its reliability. One hundred and one patients were enrolled; three subgroups were defined (R-ACM, n = 68; Bi-ACM, n = 14; LD-ACM, n = 19). Over a median of 5.41 (2.59-8.37) years, the non-classical form cohort experienced higher rates of VAs than the classical form [5-year freedom from VAs: 0.58 (0.43-0.78) vs. 0.76 (0.66-0.89), P = 0.04]. The Cadrin-Tourigny et al. predictive model adequately described the overall cohort risk [mean observed-predicted risk difference (O-PRD): +6.7 (-4.3, +17.7) %, P = 0.19]; strafing by subgroup, excellent goodness-of-fit was demonstrated for the R-ACM subgroup (mean O-PRD, P = 0.99), while in the Bi-ACM and LD-ACM ones the real observed risk appeared to be underestimated [mean O-PRD: -20.0 (-1.1, -38.9) %, P < 0.0001; -22.6 (-7.8, -37.5) %, P < 0.0001, respectively]. CONCLUSION: Non-classical ACM forms appear more prone to VAs than classical forms. The novel prediction model effectively predicted arrhythmic risk in the classical R-ACM cohort, but seemed to underestimate it in non-classical forms. Published on behalf of the European Society of Cardiology. All rights reserved.
Authors: Paloma Jordà; Laurens P Bosman; Alessio Gasperetti; Andrea Mazzanti; Jean Baptiste Gourraud; Brianna Davies; Tanja Charlotte Frederiksen; Zoraida Moreno Weidmann; Andrea Di Marco; Jason D Roberts; Ciorsti MacIntyre; Colette Seifer; Antoine Delinière; Wael Alqarawi; Deni Kukavica; Damien Minois; Alessandro Trancuccio; Marine Arnaud; Mattia Targetti; Annamaria Martino; Giada Oliviero; Daniel C Pipilas; Corrado Carbucicchio; Paolo Compagnucci; Antonio Dello Russo; Iacopo Olivotto; Leonardo Calò; Steven A Lubitz; Michael J Cutler; Philippe Chevalier; Elena Arbelo; Silvia Giuliana Priori; Jeffrey S Healey; Hugh Calkins; Michela Casella; Henrik Kjærulf Jensen; Claudio Tondo; Rafik Tadros; Cynthia A James; Andrew D Krahn; Julia Cadrin-Tourigny Journal: Eur Heart J Date: 2022-08-21 Impact factor: 35.855
Authors: Michela Casella; Marco Bergonti; Antonio Dello Russo; Riccardo Maragna; Alessio Gasperetti; Paolo Compagnucci; Valentina Catto; Filippo Trombara; Antonio Frappampina; Edoardo Conte; Marco Fogante; Elena Sommariva; Stefania Rizzo; Monica De Gaspari; Andrea Giovagnoni; Daniele Andreini; Giulio Pompilio; Luigi Di Biase; Andrea Natale; Cristina Basso; Claudio Tondo Journal: J Am Heart Assoc Date: 2021-09-25 Impact factor: 5.501