Arrhythmic risk stratification in patients with left ventricular ring-like scar
By
Vanda Parisi
Maddalena Graziosi
Luis R Lopes
Antonio De Luca
Ferdinando Pasquale
Giacomo Tini
Mattia Targetti
Maria R Cueto
Ana R Moura
Raffaello Ditaranto
Camilla Torlasco
Nevio Taglieri
Elena Nardi
Luigi Lovato
João B Augusto
Nazzareno Galiè
Lia Crotti
Alessio Gasperetti
Mauro Biffi
Camillo Autore
Marco Merlo
Iacopo Olivotto
Gianfranco Sinagra
Perry M Elliott
Elena Biagini
November 1, 2024
Clinical Scorecard: Risk Assessment for Arrhythmias in Patients with Left Ventricular Ring-Like Scarring
At a Glance
Category Detail
Condition Left ventricular ring-like scar in non-ischaemic cardiomyopathy Key Mechanisms Myocardial fibrosis detected by late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) associated with malignant arrhythmias Target Population Patients with non-ischaemic cardiomyopathy exhibiting LV ring-like scar and genetic/familial criteria Care Setting Tertiary referral centers with cardiac imaging and genetic testing capabilities
Key Highlights
LV ring-like scar defined as ≥3 contiguous segments with sub-epicardial/midwall LGE on CMR Anterior Q waves, QRS prolongation, and increased LV end-diastolic volume index independently predict life-threatening arrhythmic events Normal ECG identifies a lower risk subgroup among patients with LV ring-like scar Guideline-Based Recommendations
Diagnosis
Use contrast-enhanced CMR to identify LV ring-like scar pattern Confirm presence of pathogenic/likely pathogenic genetic variants or family history of cardiomyopathy Exclude ischaemic heart disease and phenocopies through clinical and multimodality evaluation Management
Risk stratify patients based on ECG findings (anterior Q waves, QRS width) and LV end-diastolic volume index Consider close monitoring and preventive strategies for patients with abnormal ECG and increased LV volumes Monitoring & Follow-up
Regular follow-up with ECG and imaging to assess arrhythmic risk progression Monitor for life-threatening arrhythmic events including sudden cardiac death, aborted SCD, and sustained ventricular tachycardia Risks
High rate of malignant ventricular arrhythmias in presence of LV ring-like scar with ECG abnormalities and increased LV volumes Lower arrhythmic risk in patients with normal ECG despite presence of LV ring-like scar Patient & Prescribing Data
Patients with non-ischaemic cardiomyopathy and LV ring-like scar pattern on CMR
Risk stratification should guide management; traditional predictors like LV systolic function may not add prognostic value in this phenotype
Clinical Best Practices
Perform comprehensive evaluation including genetic testing, family history, and CMR imaging for patients suspected of LV ring-like scar Use ECG parameters (anterior Q waves, QRS duration) combined with LV volume measurements for arrhythmic risk stratification Exclude ischaemic heart disease and phenocopies to avoid misdiagnosis Apply European Task Force criteria for arrhythmogenic cardiomyopathy diagnosis Implement multidisciplinary follow-up in tertiary care centers experienced in cardiomyopathy management References
