Catheter Ablation for Ventricular Tachycardia in Patients With Desmoplakin Cardiomyopathy

Alessio Gasperetti; Giovanni Peretto; Steven A. Muller; Kanae Hasegawa; Paolo Compagnucci; Michela Casella; Brittney Murray; Crystal Tichnell; Richard T. Carrick; Julia Cadrin-Tourigny; Marco Schiavone; Cynthia James; Ahmad S. Amin; Ardan M. Saguner; Antonio Dello Russo; Claudio Tondo; William Stevenson; Paolo Della Bella; Hugh Calkins; Harikrishna Tandri

doi:10.1016/j.jacep.2023.11.017

Catheter Ablation for Ventricular Tachycardia in Patients With Desmoplakin Cardiomyopathy

Alessio Gasperetti, Giovanni Peretto, Steven A. Muller, Kanae Hasegawa, Paolo Compagnucci, Michela Casella, Brittney Murray, Crystal Tichnell, Richard T. Carrick, Julia Cadrin-Tourigny, Marco Schiavone, Cynthia James, Ahmad S. Amin, Ardan M. Saguner, Antonio Dello Russo, Claudio Tondo, William Stevenson, Paolo Della Bella, Hugh Calkins, Harikrishna Tandri^*

^*Corresponding author for this work

Department of Cardiology

Research output: Contribution to journal › Article › Academic › peer-review

Abstract

Background: Desmoplakin (DSP) pathogenic/likely pathogenic (P/LP) variants are associated with malignant phenotypes of arrhythmogenic cardiomyopathy (DSP-ACM). Reports of outcomes after ventricular tachycardia (VT) ablation in DSP-ACM are scarce. Objectives: In this study, the authors sought to report on long-term outcomes of VT ablation in DSP-ACM. Methods: Patients with P/LP DSP variants at 9 institutions undergoing VT ablation were included. Demographic, clinical, and instrumental data as well as all ventricular arrhythmia (VA) events were collected. Sustained VAs after the index procedure were the primary outcome. A per-patient before and after ablation comparison of rates of VA episodes per year was performed as well. Results: Twenty-four DSP-ACM patients (39.3 ± 12.1 years of age, 62.5% male, median 6,116 [Q1-Q3: 3,362-7,760] premature ventricular complexes [PVCs] per 24 hours, median 4 [Q1-Q3: 2-11] previous VA episodes per patient at ablation) were included. Index procedure was most commonly endocardial/epicardial (19/24) The endocardium of the right ventricle (RV), the left ventricle (LV), or both ventricles were mapped in 8 (33.3%), 9 (37.5%), and 7 (29.2%) cases, respectively. Low voltage potentials were found in 10 of 15 patients in the RV and 11 of 16 in the LV. Endocardial ablation was performed in 18 patients (75.0%). Epicardial mapping in 19 patients (79.2%) identified low voltage potentials in 17, and 16 received epicardial ablation. Over the following 2.9 years (Q1-Q3: 1.8-5.5 years), 13 patients (54.2%) experienced VA recurrences. A significant reduction in per-patient event/year before and after ablation was observed (1.4 [Q1-Q3: 0.5-2.4] to 0.1 [Q1-Q3: 0.0-0.4]; P = 0.009). Two patients needed heart transplantation, and 4 died (3 of heart failure and 1 noncardiac death). Conclusions: VT ablation in DSP-ACM is effective in reducing the VA burden of the disease, but recurrences are common. Most VT circuits are epicardial, with both LV and RV low voltage abnormalities. Heart failure complicates clinical course and is an important cause of mortality.

Original language	English
Pages (from-to)	487-498
Number of pages	12
Journal	JACC: Clinical Electrophysiology
Volume	10
Issue number	3
DOIs	https://doi.org/10.1016/j.jacep.2023.11.017
Publication status	Published - Mar 2024

Keywords

arrhythmogenic cardiomyopathy
desmoplakin
epicardial procedure
SCD
VT ablation

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10.1016/j.jacep.2023.11.017

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Gasperetti, A., Peretto, G., Muller, S. A., Hasegawa, K., Compagnucci, P., Casella, M., Murray, B., Tichnell, C., Carrick, R. T., Cadrin-Tourigny, J., Schiavone, M., James, C., Amin, A. S., Saguner, A. M., Dello Russo, A., Tondo, C., Stevenson, W., Della Bella, P., Calkins, H., & Tandri, H. (2024). Catheter Ablation for Ventricular Tachycardia in Patients With Desmoplakin Cardiomyopathy. JACC: Clinical Electrophysiology, 10(3), 487-498. https://doi.org/10.1016/j.jacep.2023.11.017

@article{d02ee8d4900c4f11bb4fb152b4a8f7bc,

title = "Catheter Ablation for Ventricular Tachycardia in Patients With Desmoplakin Cardiomyopathy",

abstract = "Background: Desmoplakin (DSP) pathogenic/likely pathogenic (P/LP) variants are associated with malignant phenotypes of arrhythmogenic cardiomyopathy (DSP-ACM). Reports of outcomes after ventricular tachycardia (VT) ablation in DSP-ACM are scarce. Objectives: In this study, the authors sought to report on long-term outcomes of VT ablation in DSP-ACM. Methods: Patients with P/LP DSP variants at 9 institutions undergoing VT ablation were included. Demographic, clinical, and instrumental data as well as all ventricular arrhythmia (VA) events were collected. Sustained VAs after the index procedure were the primary outcome. A per-patient before and after ablation comparison of rates of VA episodes per year was performed as well. Results: Twenty-four DSP-ACM patients (39.3 ± 12.1 years of age, 62.5% male, median 6,116 [Q1-Q3: 3,362-7,760] premature ventricular complexes [PVCs] per 24 hours, median 4 [Q1-Q3: 2-11] previous VA episodes per patient at ablation) were included. Index procedure was most commonly endocardial/epicardial (19/24) The endocardium of the right ventricle (RV), the left ventricle (LV), or both ventricles were mapped in 8 (33.3%), 9 (37.5%), and 7 (29.2%) cases, respectively. Low voltage potentials were found in 10 of 15 patients in the RV and 11 of 16 in the LV. Endocardial ablation was performed in 18 patients (75.0%). Epicardial mapping in 19 patients (79.2%) identified low voltage potentials in 17, and 16 received epicardial ablation. Over the following 2.9 years (Q1-Q3: 1.8-5.5 years), 13 patients (54.2%) experienced VA recurrences. A significant reduction in per-patient event/year before and after ablation was observed (1.4 [Q1-Q3: 0.5-2.4] to 0.1 [Q1-Q3: 0.0-0.4]; P = 0.009). Two patients needed heart transplantation, and 4 died (3 of heart failure and 1 noncardiac death). Conclusions: VT ablation in DSP-ACM is effective in reducing the VA burden of the disease, but recurrences are common. Most VT circuits are epicardial, with both LV and RV low voltage abnormalities. Heart failure complicates clinical course and is an important cause of mortality.",

keywords = "arrhythmogenic cardiomyopathy, desmoplakin, epicardial procedure, SCD, VT ablation",

author = "Alessio Gasperetti and Giovanni Peretto and Muller, {Steven A.} and Kanae Hasegawa and Paolo Compagnucci and Michela Casella and Brittney Murray and Crystal Tichnell and Carrick, {Richard T.} and Julia Cadrin-Tourigny and Marco Schiavone and Cynthia James and Amin, {Ahmad S.} and Saguner, {Ardan M.} and {Dello Russo}, Antonio and Claudio Tondo and William Stevenson and {Della Bella}, Paolo and Hugh Calkins and Harikrishna Tandri",

note = "Publisher Copyright: {\textcopyright} 2024 American College of Cardiology Foundation",

year = "2024",

month = mar,

doi = "10.1016/j.jacep.2023.11.017",

language = "English",

volume = "10",

pages = "487--498",

number = "3",

}

Gasperetti, A, Peretto, G, Muller, SA, Hasegawa, K, Compagnucci, P, Casella, M, Murray, B, Tichnell, C, Carrick, RT, Cadrin-Tourigny, J, Schiavone, M, James, C, Amin, AS, Saguner, AM, Dello Russo, A, Tondo, C, Stevenson, W, Della Bella, P, Calkins, H & Tandri, H 2024, 'Catheter Ablation for Ventricular Tachycardia in Patients With Desmoplakin Cardiomyopathy', JACC: Clinical Electrophysiology, vol. 10, no. 3, pp. 487-498. https://doi.org/10.1016/j.jacep.2023.11.017

TY - JOUR

T1 - Catheter Ablation for Ventricular Tachycardia in Patients With Desmoplakin Cardiomyopathy

AU - Gasperetti, Alessio

AU - Peretto, Giovanni

AU - Muller, Steven A.

AU - Hasegawa, Kanae

AU - Compagnucci, Paolo

AU - Casella, Michela

AU - Murray, Brittney

AU - Tichnell, Crystal

AU - Carrick, Richard T.

AU - Cadrin-Tourigny, Julia

AU - Schiavone, Marco

AU - James, Cynthia

AU - Amin, Ahmad S.

AU - Saguner, Ardan M.

AU - Dello Russo, Antonio

AU - Tondo, Claudio

AU - Stevenson, William

AU - Della Bella, Paolo

AU - Calkins, Hugh

AU - Tandri, Harikrishna

PY - 2024/3

Y1 - 2024/3

N2 - Background: Desmoplakin (DSP) pathogenic/likely pathogenic (P/LP) variants are associated with malignant phenotypes of arrhythmogenic cardiomyopathy (DSP-ACM). Reports of outcomes after ventricular tachycardia (VT) ablation in DSP-ACM are scarce. Objectives: In this study, the authors sought to report on long-term outcomes of VT ablation in DSP-ACM. Methods: Patients with P/LP DSP variants at 9 institutions undergoing VT ablation were included. Demographic, clinical, and instrumental data as well as all ventricular arrhythmia (VA) events were collected. Sustained VAs after the index procedure were the primary outcome. A per-patient before and after ablation comparison of rates of VA episodes per year was performed as well. Results: Twenty-four DSP-ACM patients (39.3 ± 12.1 years of age, 62.5% male, median 6,116 [Q1-Q3: 3,362-7,760] premature ventricular complexes [PVCs] per 24 hours, median 4 [Q1-Q3: 2-11] previous VA episodes per patient at ablation) were included. Index procedure was most commonly endocardial/epicardial (19/24) The endocardium of the right ventricle (RV), the left ventricle (LV), or both ventricles were mapped in 8 (33.3%), 9 (37.5%), and 7 (29.2%) cases, respectively. Low voltage potentials were found in 10 of 15 patients in the RV and 11 of 16 in the LV. Endocardial ablation was performed in 18 patients (75.0%). Epicardial mapping in 19 patients (79.2%) identified low voltage potentials in 17, and 16 received epicardial ablation. Over the following 2.9 years (Q1-Q3: 1.8-5.5 years), 13 patients (54.2%) experienced VA recurrences. A significant reduction in per-patient event/year before and after ablation was observed (1.4 [Q1-Q3: 0.5-2.4] to 0.1 [Q1-Q3: 0.0-0.4]; P = 0.009). Two patients needed heart transplantation, and 4 died (3 of heart failure and 1 noncardiac death). Conclusions: VT ablation in DSP-ACM is effective in reducing the VA burden of the disease, but recurrences are common. Most VT circuits are epicardial, with both LV and RV low voltage abnormalities. Heart failure complicates clinical course and is an important cause of mortality.

AB - Background: Desmoplakin (DSP) pathogenic/likely pathogenic (P/LP) variants are associated with malignant phenotypes of arrhythmogenic cardiomyopathy (DSP-ACM). Reports of outcomes after ventricular tachycardia (VT) ablation in DSP-ACM are scarce. Objectives: In this study, the authors sought to report on long-term outcomes of VT ablation in DSP-ACM. Methods: Patients with P/LP DSP variants at 9 institutions undergoing VT ablation were included. Demographic, clinical, and instrumental data as well as all ventricular arrhythmia (VA) events were collected. Sustained VAs after the index procedure were the primary outcome. A per-patient before and after ablation comparison of rates of VA episodes per year was performed as well. Results: Twenty-four DSP-ACM patients (39.3 ± 12.1 years of age, 62.5% male, median 6,116 [Q1-Q3: 3,362-7,760] premature ventricular complexes [PVCs] per 24 hours, median 4 [Q1-Q3: 2-11] previous VA episodes per patient at ablation) were included. Index procedure was most commonly endocardial/epicardial (19/24) The endocardium of the right ventricle (RV), the left ventricle (LV), or both ventricles were mapped in 8 (33.3%), 9 (37.5%), and 7 (29.2%) cases, respectively. Low voltage potentials were found in 10 of 15 patients in the RV and 11 of 16 in the LV. Endocardial ablation was performed in 18 patients (75.0%). Epicardial mapping in 19 patients (79.2%) identified low voltage potentials in 17, and 16 received epicardial ablation. Over the following 2.9 years (Q1-Q3: 1.8-5.5 years), 13 patients (54.2%) experienced VA recurrences. A significant reduction in per-patient event/year before and after ablation was observed (1.4 [Q1-Q3: 0.5-2.4] to 0.1 [Q1-Q3: 0.0-0.4]; P = 0.009). Two patients needed heart transplantation, and 4 died (3 of heart failure and 1 noncardiac death). Conclusions: VT ablation in DSP-ACM is effective in reducing the VA burden of the disease, but recurrences are common. Most VT circuits are epicardial, with both LV and RV low voltage abnormalities. Heart failure complicates clinical course and is an important cause of mortality.

KW - arrhythmogenic cardiomyopathy

KW - desmoplakin

KW - epicardial procedure

KW - SCD

KW - VT ablation

UR - http://www.scopus.com/inward/record.url?scp=85184805989&partnerID=8YFLogxK

U2 - 10.1016/j.jacep.2023.11.017

DO - 10.1016/j.jacep.2023.11.017

M3 - Article

C2 - 38206263

AN - SCOPUS:85184805989

SN - 2405-500X

VL - 10

SP - 487

EP - 498

JO - JACC: Clinical Electrophysiology

JF - JACC: Clinical Electrophysiology

IS - 3

ER -

Catheter Ablation for Ventricular Tachycardia in Patients With Desmoplakin Cardiomyopathy

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