Thomas P Mast1, Arco J Teske1, Anneline Sjm Te Riele1,2, Judith A Groeneweg1, Jeroen F Van Der Heijden1, Birgitta K Velthuis3, Peter Loh1, Pieter A Doevendans1, Toon A Van Veen4, Dennis Dooijes5, Jacques M De Bakker4,6,7, Richard N Hauer1,7, Maarten J Cramer1. 1. Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands. 2. Division of Cardiology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. 3. Department of Radiology, University Medical Centre Utrecht, Utrecht, The Netherlands. 4. Department of Medical Physiology and Cardiology, University Medical Center Utrecht, The Netherlands. 5. Department of Medical Genetics, University Medical Center Utrecht, Utrecht, The Netherlands. 6. Department of Experimental Cardiology, Heart Center, Academic Medical Center, Amsterdam, The Netherlands. 7. ICIN-Netherlands Heart Institute, Utrecht, The Netherlands.
Abstract
INTRODUCTION: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is characterized by high incidence of ventricular arrhythmias. Overt ARVD/C is preceded by a subclinical stage with lack of detectable ECG and structural abnormalities. Activation delay is present before structural abnormalities and is a hallmark of arrhythmogenesis. Deformation imaging may unmask activation delay in the subclinical stage. METHODS: Three groups were compared: (1) mutation-positive definite ARVD/C-patients fulfilling 2010 Task Force criteria (TFC) (n = 44); (2) asymptomatic mutation carriers not fulfilling TFC and without history of ventricular arrhythmias (n = 31); and (3) controls (n = 30). All underwent ECG and echocardiographic deformation imaging. As a surrogate for local activation delay the electromechanical interval (EMI) was measured, defined as time between onset-QRS and onset of shortening. Arrhythmic outcome (PVC-count, VT) of asymptomatic mutation carriers was correlated with EMI and ECG TFC. RESULTS: In definite ARVD/C-patients, EMI was prolonged in all lateral RV segments. In asymptomatic mutation carriers, prolonged EMI was detected in the subtricuspid area in 14/31. Terminal activation duration ≥55 milliseconds (definition: supporting information) was the only ECG abnormality in this group (8/31). After a mean follow-up of 4.2 ± 3.1 years 10/31 asymptomatic mutation carriers experienced arrhythmic outcome. Prolonged subtricuspid EMI was the only parameter significantly associated with arrhythmogenesis during follow-up. CONCLUSION: In ARVD/C-patients, EMI prolongation was present throughout the RV. In asymptomatic mutation carriers, prolonged EMI in the subtricuspid area is often detected without any additional abnormalities. These preliminary results indicate that prolonged EMI is a new parameter unmasking activation delay in the subclinical stage and may contribute to risk stratification.
INTRODUCTION:Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is characterized by high incidence of ventricular arrhythmias. Overt ARVD/C is preceded by a subclinical stage with lack of detectable ECG and structural abnormalities. Activation delay is present before structural abnormalities and is a hallmark of arrhythmogenesis. Deformation imaging may unmask activation delay in the subclinical stage. METHODS: Three groups were compared: (1) mutation-positive definite ARVD/C-patients fulfilling 2010 Task Force criteria (TFC) (n = 44); (2) asymptomatic mutation carriers not fulfilling TFC and without history of ventricular arrhythmias (n = 31); and (3) controls (n = 30). All underwent ECG and echocardiographic deformation imaging. As a surrogate for local activation delay the electromechanical interval (EMI) was measured, defined as time between onset-QRS and onset of shortening. Arrhythmic outcome (PVC-count, VT) of asymptomatic mutation carriers was correlated with EMI and ECG TFC. RESULTS: In definite ARVD/C-patients, EMI was prolonged in all lateral RV segments. In asymptomatic mutation carriers, prolonged EMI was detected in the subtricuspid area in 14/31. Terminal activation duration ≥55 milliseconds (definition: supporting information) was the only ECG abnormality in this group (8/31). After a mean follow-up of 4.2 ± 3.1 years 10/31 asymptomatic mutation carriers experienced arrhythmic outcome. Prolonged subtricuspid EMI was the only parameter significantly associated with arrhythmogenesis during follow-up. CONCLUSION: In ARVD/C-patients, EMI prolongation was present throughout the RV. In asymptomatic mutation carriers, prolonged EMI in the subtricuspid area is often detected without any additional abnormalities. These preliminary results indicate that prolonged EMI is a new parameter unmasking activation delay in the subclinical stage and may contribute to risk stratification.
Authors: Marijn H A Groen; Laurens P Bosman; Arco J Teske; Thomas P Mast; Karim Taha; Frebus J Van Slochteren; Maarten J Cramer; Pieter A Doevendans; René van Es Journal: Echocardiography Date: 2020-05-03 Impact factor: 1.724
Authors: Karim Taha; Mimount Bourfiss; Anneline S J M Te Riele; Maarten-Jan M Cramer; Jeroen F van der Heijden; Folkert W Asselbergs; Birgitta K Velthuis; Arco J Teske Journal: Eur Heart J Cardiovasc Imaging Date: 2021-07-20 Impact factor: 6.875
Authors: Stephanie M van der Voorn; Anneline S J M Te Riele; Cristina Basso; Hugh Calkins; Carol Ann Remme; Toon A B van Veen Journal: Cardiovasc Res Date: 2020-07-15 Impact factor: 10.787
Authors: Gabriel Cismaru; Alin Grosu; Sabina Istratoaie; Laura Mada; Maria Ilea; Gabriel Gusetu; Dumitru Zdrenghea; Dana Pop; Radu Rosu Journal: Medicine (Baltimore) Date: 2020-04 Impact factor: 1.817