Arrhythmic risk stratification in patients with left ventricular ring-like scar

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

doi:10.1093/eurjpc/zwae353

Arrhythmic risk stratification in patients with left ventricular ring-like scar

Eur J Prev Cardiol. 2024 Nov 1:zwae353. doi: 10.1093/eurjpc/zwae353. Online ahead of print.

Authors

Vanda Parisi^{1

2

3}, Maddalena Graziosi^{1

3}, Luis R Lopes^{4

5}, Antonio De Luca⁶, Ferdinando Pasquale^{1

3}, Giacomo Tini⁷, Mattia Targetti⁸, Maria R Cueto^{4

9}, Ana R Moura^{4

10}, Raffaello Ditaranto^{1

2

3}, Camilla Torlasco¹¹, Nevio Taglieri^{1

3}, Elena Nardi², Luigi Lovato¹², João B Augusto^{5

13

14}, Nazzareno Galiè^{1

2

3}, Lia Crotti^{3

11

15}, Alessio Gasperetti¹⁶, Mauro Biffi^{1

3}, Camillo Autore⁷, Marco Merlo⁶, Iacopo Olivotto¹⁷, Gianfranco Sinagra⁶, Perry M Elliott^{4

5}, Elena Biagini^{1

3}

Affiliations

¹ Cardiology Unit, Cardiac Thoracic and Vascular Department, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.
² Department of Medical and Surgical Sciences (DIMEC), University of Bologna, Bologna, Italy.
³ European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart.
⁴ Barts Heart Centre, St Bartholomew's Hospital, London, UK.
⁵ Institute of Cardiovascular Science, University College London, London, UK.
⁶ Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano Isontina and University of Trieste, Trieste, Italy.
⁷ Cardiology, Department of Clinical and Molecular Medicine, Faculty of Medicine and Psychology, Sapienza University, Rome, Italy.
⁸ Cardiomyopathy Unit, Careggi University Hospital, Florence, Italy.
⁹ Heart Failure and Cardiomyopathies Clinic, Germans Trias i Pujol University Hospital, Badalona, Spain.
¹⁰ Unidade Local de Saúde de Matosinhos, Portugal.
¹¹ IRCCS, Istituto Auxologico Italiano, Department of Cardiology, Cardiomyopathy Unit, San Luca Hospital, Milan, Italy.
¹² Pediatric and Adult Cardio-Thoracic and Vascular, Onco-Hematologic and Emergency Radiology Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna.
¹³ Cardiology Department, Hospital Prof Doutor Fernando Fonseca, Amadora, Portugal.
¹⁴ Católica Medical School, Universidade Católica Portuguesa, Lisbon, Portugal.
¹⁵ Department of Medicine and Surgery, University of Milano-Bicocca, Milan, Italy.
¹⁶ Division of Cardiology, School of Medicine, Johns Hopkins University, 600 N. Wolfe St. Blalock 545, Baltimore, MD 21287, USA.
¹⁷ Meyer Children Hospital and Careggi University Hospital, University of Florence, Florence, Italy.

PMID: 39486037
DOI: 10.1093/eurjpc/zwae353

Abstract

Aims: Left ventricular (LV) ring-like scar on cardiac magnetic resonance (CMR) has been linked to malignant arrhythmias in patients with non-ischemic cardiomyopathy. This study aimed to perform a comprehensive evaluation of this phenotype and to identify risk factors for life-threatening arrhythmic events (LAEs), a composite of sudden cardiac death (SCD), aborted SCD, and sustained ventricular tachycardia.

Methods and results: One-hundred-fifteen patients (median age 39 [IQR 28-52], 42% females) were identified at 6 referral centres. Inclusion criteria were ring-like LV scar (≥ 3 contiguous segments with subepicardial/midwall late gadolinium enhancement (LGE) in the same slice) and one among: pathogenic/likely pathogenic genetic variant, family history for cardiomyopathy, or arrhythmogenic cardiomyopathy diagnosis. During the study follow-up, survival-free from LAEs was 60% (3.8 events/100 patients/year); at a median follow-up of 4.6 years (IQR 1.7-8.4) it was 84%. On multivariable analysis, anterior Q waves (HR:1.030, 95% CI:1.014-1.046, p < 0.001), QRS width (HR:4.642, 95% CI:1.296-16.628, p=0.018), and LV end-diastolic volume index (LVEDVi) (HR:1.011, 95% CI:1.001-1.021, per mL/m2 increase, p=0.040) were independently associated with LAEs; with good discrimination power (Harrell's C-index=0.796). Three risk categories were identified: normal ECG, abnormal ECG and no LAEs predictive variables, abnormal ECG and ≥ 1 LAEs predictive variables, with a decreasing survival from 100% to 65% and 49%, respectively (Log-rank test = 0.015).

Conclusions: In this study, the LV ring-like scar phenotype was associated with a high rate of malignant arrhythmias in presence of anterior Q waves, QRS prolongation, and increased LVEDVi. A normal ECG identified a lower risk subgroup.

Keywords: arrhythmias; cardiac magnetic resonance; cardiomyopathy; electrocardiogram; prognosis; ring-like scar; risk stratification.

Plain language summary

Left ventricular (LV) ring-like scar represents the cardiac magnetic resonance (CMR) expression of different genetic substrates and several clinical scenarios. Arrhythmic risk stratification predictors are still not well understood. In this study: LV ring-like scar exhibits a high rate of ventricular arrhythmias, particularly in the presence of ECG abnormalities (anterior Q waves and QRS enlargement) together with increased LV volumes Other commonly used risk predictors (such as LV systolic function) did not add significant prognostic information.