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Morphological and Functional Assessment of the Right Ventricle Using 3D Echocardiography
Published on: October 28, 2020
Clinical Characteristics and Outcomes of Patients With Biventricular and Left-Dominant Arrhythmogenic Cardiomyopathy
Nicolò Martini1, Raimondo Pittorru1, Rudi Celeghin2
1Department of Cardio-Thoraco-Vascular Sciences and Public Health, University of Padua, Padua, Italy; PhD Program in Translation Specialistic Medicine "G.B. Morgagni," Curriculum Cardiovascular Sciences, Padua, Italy.
Insights
Ring-like late gadolinium enhancement (RL-LGE) on cardiac MRI independently predicts life-threatening arrhythmic events in arrhythmogenic cardiomyopathy (ACM). This imaging marker improves risk prediction models, aiding early identification of high-risk patients for potential implantable cardioverter-defibrillator (ICD) therapy.
Area of Science:
- Cardiology
- Medical Imaging
- Genetics
Background:
- The ring-like pattern of late gadolinium enhancement (RL-LGE) on cardiac magnetic resonance (CMR) is a proposed marker for arrhythmogenic cardiomyopathy (ACM) with left ventricular (LV) involvement.
- However, its clinical characteristics and prognostic value require further investigation.
Purpose of the Study:
- To evaluate the clinical profile, genetic associations, and prognostic significance of RL-LGE in patients with ACM involving the LV.
Main Methods:
- An observational cohort study included 149 patients diagnosed with biventricular or left-dominant ACM (BIV-ACM or LD-ACM).
- RL-LGE was defined as subepicardial or midmyocardial LGE in at least three contiguous LV segments.
- The primary endpoint comprised sudden cardiac arrest, sustained ventricular tachycardia, or implantable cardioverter-defibrillator (ICD) interventions.
Main Results:
- RL-LGE was present in 49% of patients, often associated with DSP gene variants and more prevalent in BIV-ACM.
- During a median follow-up of 31 months, 24 patients reached the primary endpoint, with 67% exhibiting RL-LGE.
- RL-LGE independently predicted the primary endpoint (HR: 2.47), alongside nonsustained ventricular tachycardia, while genetic status did not offer additional prognostic information.
Conclusions:
- RL-LGE is an independent predictor of arrhythmic events in ACM, irrespective of genetic background.
- Its inclusion enhances the predictive accuracy of existing risk models for ACM.
- Detecting RL-LGE can assist in identifying high-risk individuals and guiding decisions on primary-prevention ICD therapy.
Background:
The ring-like pattern of late gadolinium enhancement (RL-LGE) on cardiac magnetic resonance (CMR) has been proposed as a distinctive imaging marker of arrhythmogenic cardiomyopathy (ACM) with left ventricular (LV) involvement. However, the clinical characteristics and prognostic significance remain to be further clarified.
Objectives:
This study sought to assess the clinical profile, genetic background, and prognostic significance of RL-LGE in ACM with LV involvement.
Methods:
In this observational cohort study, we included consecutive patients with a diagnosis of biventricular or left-dominant ACM (BIV-ACM or LD-ACM). RL-LGE was defined as subepicardial or midmyocardial LGE involving ≥3 contiguous LV segments on the same short-axis slice. The primary endpoint was a composite of sudden cardiac arrest, sustained ventricular tachycardia, or implantable cardioverter-defibrillator (ICD) interventions.
Results:
Among 149 patients (mean age 36 ± 12 years, 66% male), RL-LGE was identified in 73 (49%), most frequently in association with DSP variants, with a higher prevalence in BIV-ACM (70%) than in LD-ACM (30%). Over a median follow-up of 31 months, 24 patients experienced the primary endpoint, 67% of whom had RL-LGE. In multivariable Cox regression, RL-LGE emerged as an independent predictor of the primary endpoint (HR: 2.47; 95% CI: 1.10-6.02; P = 0.042), along with nonsustained ventricular tachycardia (HR: 2.62; 95% CI: 1.17-6.41; P = 0.033), whereas genetic status did not provide additional prognostic information. Incorporating RL-LGE into the arrhythmogenic right ventricular cardiomyopathy risk model significantly improved its predictive performance (likelihood ratio test: P = 0.006).
Conclusions:
RL-LGE independently predicts arrhythmic events in ACM, regardless of genotype status, and refines the prognostic performance of traditional risk models. Its detection may aid in early recognition of high-risk patients and inform primary-prevention ICD therapy.
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