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Monitoring of Arrhythmogenic RV Cardiomyopathy Across Age Spectrum
Quick Takes
- LV global longitudinal strain slightly worsened and RV deformation abnormalities progressed over time in all age groups with arrhythmogenic right ventricular cardiomyopathy (ARVC).
- These findings suggest that age-based tailoring of follow-up intervals would not be indicated in ARVC.
- On the contrary, since progression continued throughout all age groups and was in multiple cases followed by sustained ventricular arrhythmia, there is a need for lifelong follow-up and follow-up of ARVC patients, and family members should not stop at older age.
Study Questions:
Is there a need for age-tailoring of follow-up protocols in early arrhythmogenic right ventricular cardiomyopathy (ARVC) by evaluating myocardial disease progression in different age groups?
Methods:
The investigators stratified early-stage ARVC patients and genotype-positive relatives without overt structural disease and ventricular arrhythmia (VA) at first evaluation into three age groups: <30, 30-50, and ≥50 years old. Longitudinal biventricular deformation characteristics were used to monitor disease progression. To link deformation abnormalities to underlying myocardial disease substrates, Digital Twins were created using an imaging-based computational modeling framework. Disease progression was assessed in the three age groups by entering the echocardiographic deformation parameters into a linear mixed model regression with exchangeable covariance structure and random effects at the individual level.
Results:
Investigators included 313 echocardiographic assessments from 82 subjects (57% female, age 39 ± 17 years, 10% probands) during 6.7 ± 3.3 years of follow-up. Left ventricular global longitudinal strain slightly deteriorated similarly in all age groups (0.1%-point per year; 95% confidence interval [CI], 0.05-0.15). Disease progression in all age groups was more pronounced in the RV lateral wall, expressed by worsening in longitudinal strain (0.6%-point per year; 95% CI, 0.46-0.70) and local differences in myocardial contractility, compliance, and activation delay in the Digital Twin. Six patients experienced VA during follow-up.
Conclusions:
The authors report that disease progression was similar in all age groups and sustained VA also occurred in patients >50 years old without overt ARVC phenotype at first evaluation.
Perspective:
This study reports that LV global longitudinal strain slightly worsened and RV deformation abnormalities progressed over time in all age groups with ARVC. These findings suggest that age-based tailoring of follow-up intervals would not be indicated in ARVC. On the contrary, since progression continued throughout all age groups and was in multiple cases followed by sustained VA, there is a need for lifelong follow-up and follow-up of ARVC patients, and family members should not stop at older age. Additional research into the evolution of tissue substrates of individual patients may further improve prediction of adverse outcomes in ARVC.
Clinical Topics: Arrhythmias and Clinical EP, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Implantable Devices, Genetic Arrhythmic Conditions, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Imaging, Acute Heart Failure, Echocardiography/Ultrasound
Keywords: Arrhythmias, Cardiac, Arrhythmogenic Right Ventricular Dysplasia, Cardiomyopathies, Diagnostic Imaging, Disease Progression, Echocardiography, Genotype, Heart Defects, Congenital, Heart Failure, Phenotype
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