Prognostic Value of Magnetic Resonance Phenotype in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy

Aquaro, Giovanni Donato; Luca, Antonio De; Cappelletto, Chiara; Raimondi, Francesca; Bianco, Francesco; Botto, Nicoletta; Lesizza, Pierluigi; Grigoratos, Chrysanthos; Minati, Monia; Dell’Omodarme, M.; Pingitore, Alessandro; Stolfo, Davide; Ferro, Matteo Dal; Merlo, Marco; Bella, Gianluca Di; Sinagra, Gianfranco

doi:10.1016/j.jacc.2020.04.023

Cited by 96 publications

(79 citation statements)

References 25 publications

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“…The median 5 year ARVC risk was of 29% (20–42) in patients with ITFC indication for ICD and 11% 5–17 in those without ( P < 0.001). Patients with HRS criteria had a median 5 year ARVC risk of 31% (21–54), while those not satisfying these criteria had a median of 19% (10–28%), P < 0.001.…”

Section: Resultsmentioning

confidence: 98%

“…Indeed, DSP and DSG2 mutations were more frequently associated with LV involvement and with a higher risk of sudden death than the PKP2 mutation. 7,14 Recently, two multicentre studies demonstrated that LV involvement is associated with worse prognosis than lone RV involvement. Our population had a greater prevalence of DSP and DSG2 mutations and LV involvement than that of Cadrin-Tourigny's study, which may explain the higher percentage of events of our study.…”

Section: Discussionmentioning

confidence: 99%

“…Because of this asymmetrical distribution of mutated genes, the effectiveness of this risk score to predict events in patients with DSP or DSG2 mutation was not guaranteed. Indeed, DSP and DSG2 mutations were more frequently associated with LV involvement and with a higher risk of sudden death than the PKP2 mutation 7,14 . Recently, two multicentre studies demonstrated that LV involvement is associated with worse prognosis than lone RV involvement.…”

Section: Discussionmentioning

confidence: 99%

“…The three risk models were retrospectively evaluated considering baseline characteristics of the population of the PROAC prospective registry. 7 In this multicentre registry, 150 consecutive patients with definite ARVC were prospectively enrolled in six different institutions to evaluate the prognostic role of cardiac magnetic resonance (CMR) presentation in patients with definite ARVC. The diagnosis of definite ARVC was based on revised Task Force criteria 8 : patients had a 'definite' diagnosis when fulfilling two or one major and two or four minor criteria from different categories.…”

Section: Methodsmentioning

confidence: 99%

“…Cardiac magnetic resonance was performed in all the patients as previously reported. 7,10 The following parameters of right ventricular (RV) involvement were considered: (i) regional wall motion abnormalities (akinesia or dyskinesia), (ii) RV end-diastolic volume to body surface area ≥110 mL/m 2 in men or ≥100 mL/m 2 in women, or (iii) RV ejection fraction ≤45%.…”

Section: Methodsmentioning

confidence: 99%

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Comparison of different prediction models for the indication of implanted cardioverter defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy

et al. 2020

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Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with a high risk of sudden cardiac death. Three different prediction models for the indication of implanted cardioverter defibrillator (ICD) are now available: the 5 year ARVC risk score, the International Task Force Consensus (ITFC) criteria, and the Heart Rhythm Society (HRS) criteria. We compared these three prediction models in a validation cohort of patients with definite ARVC. Methods and results In a cohort of 140 patients with definite ARVC, the 5 year ARVC risk score and the ITFC and HRS criteria were compared for the prediction of a major combined endpoint of sudden cardiac death, appropriate ICD intervention, resuscitated cardiac arrest, and sustained ventricular tachycardia. During the follow-up, 65 major events occurred. The 5 year ARVC risk score with a threshold >10%, derived from the maximally selected rank statistic, predicted 62 (95%) events [odds ratio (OR) 9.1, 95% confidence interval (CI) 2.6-32, P = 0.0006], the ITFC criteria 53 (81%, OR 4.8, 95% CI 2.2-10.3, P = 0.0001), and the HRS criteria 29 (45%, OR 4.2, 95% CI 1.9-9.3, P = 0.0003). At the analysis of decision curve for ICD implantation, a 5 year ARVC risk score >10% showed a greater net benefit than the ITFC and HRS criteria over a wide range of threshold probability of events. Finally, at multivariate analysis, the 5 year ARVC risk score >10% was the only independent predictor of major events. Conclusions The 5 year score with a threshold of >10% was more effective for predicting events than the ITFC and HRS criteria.

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Section: Resultsmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

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Comparison of different prediction models for the indication of implanted cardioverter defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy

et al. 2020

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Association of Premature Ventricular Contraction Burden on Serial Holter Monitoring With Arrhythmic Risk in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy

et al. 2022

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IMPORTANCEA high burden of premature ventricular contractions (PVCs) at disease diagnosis has been associated with an overall higher risk of ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy (ARVC). Data regarding dynamic modification of PVC burden at follow-up with Holter monitoring and its impact on arrhythmic risk in ARVC are scarce.OBJECTIVE To describe changes in the PVC burden and to assess whether serial Holter monitoring is dynamically associated with sustained ventricular arrhythmias during follow-up in patients with ARVC. DESIGN, SETTINGS, AND PARTICIPANTSIn this cohort study, patients with a definite ARVC diagnosis, available Holter monitoring results at disease diagnosis, and at least 2 additional results of Holter monitoring during follow-up were enrolled from 6 ARVC registries in North America and Europe. Data were collected from June 1 to September 15, 2021. MAIN OUTCOMES AND MEASURESThe association between prespecified variables retrieved at each Holter monitoring follow-up (ie, overall PVC burden; presence of sudden PVC spikes, defined as absolute increase in PVC burden Ն5000 per 24 hours or a relative Ն75% increase, with an absolute increase of Ն1000 PVCs; presence of nonsustained ventricular tachycardia [NSVT]; and use of β-blockers and class III antiarrhythmic drugs) and sustained ventricular arrhythmias occurring within 12 months after that Holter examination was assessed using a mixed logistical model. RESULTSIn 169 enrolled patients with ARVC (mean [SD] age, 36.3 [15.0] years; 95 men [56.2%]), a total of 723 Holter examinations (median, 4 [IQR,(4)(5) per patient) were performed during a median follow-up of 54 (IQR, 42-63) months and detected 75 PVC spikes and 67 sustained ventricular arrhythmias. The PVC burden decreased significantly from the first to the second Holter examination (mean, 2906(mean, [95% CI, 1581 PVCs per 24 hours; P < .001). A model including 24-hour PVC burden (odds ratio [OR] 1.50 [95% CI, 1.10-2.03]; P = .01), PVC spikes (OR, 6.20 [95 CI,]; P < .001), and NSVT (OR, 2.29 [95% CI, 1.10-4.51]; P = .03) at each follow-up Holter examination was associated with sustained ventricular arrhythmia occurrence in the following 12 months.CONCLUSIONS AND RELEVANCE These findings suggest that in patients with ARVC, changes in parameters derived from each Holter examination performed during follow-up are associated with the risk of sustained ventricular arrhythmias within 12 months of disease diagnosis.

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A Young Man With Wide Complex Tachycardia—The Devil Is in the Details

2021

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A man in his 20s presented to the emergency department with complaints of intermittent palpitations for 2 days, which had worsened in the previous hour. He had no history of chest pain, dyspnea, congestion, or fever. There was no past cardiac history or family history of similar complaints. On presentation, the patient's blood pressure was 110/72 mm Hg, and respiratory rate was 14 bpm. On examination, results of cardiovascular system checks were unremarkable. A 12-lead electrocardiogram (ECG) (Figure , A) was obtained. The patient was given intravenous amiodarone, which was followed by conversion into sinus rhythm (Figure , B).Question: According to this patient's ECG results, what is the most likely diagnosis, and how would you treat the patient? Interpretation

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Prognostic Value of Magnetic Resonance Phenotype in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy

Cited by 96 publications

References 25 publications

Comparison of different prediction models for the indication of implanted cardioverter defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy

Comparison of different prediction models for the indication of implanted cardioverter defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy

Association of Premature Ventricular Contraction Burden on Serial Holter Monitoring With Arrhythmic Risk in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy

A Young Man With Wide Complex Tachycardia—The Devil Is in the Details

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