Arrhythmogenic cardiomyopathy (ACM) is a genetically determined heart muscle disease characterized by fibro-fatty myocardial replacement, clinically associated with malignant ventricular arrhythmias and sudden cardiac death. Originally described a disease with a prevalent right ventricular (RV) involvement, subsequently two other phenotypes have been recognized, such as the left dominant and the biventricular phenotypes, for which a recent International Expert consensus document provided upgrade diagnostic criteria (the 2020 “Padua Criteria”). In this novel workup for the diagnosis of the entire spectrum of phenotypic variants of ACM, including left ventricular (LV) variants, cardiac magnetic resonance (CMR) has emerged as the cardiac imaging technique of choice, due to its capability of detailed morpho-functional and tissue characterization evaluation of both RV and LV. In this review, the key role of CMR in the diagnosis of ACM is outlined, including the supplemental value for the characterization of the disease variants. An ACM-specific CMR study protocol, as well as strengths and weaknesses of each imaging technique, is also provided. Key Points • Arrhythmogenic cardiomyopathy includes three different phenotypes: dominant right, biventricular, and dominant left. • In 2020, diagnostic criteria have been updated and cardiac magnetic resonance has emerged as the cardiac imaging technique of choice. • This aim of this review is to provide an update of the current state of art regarding the use of CMR in ACM, with a particular focus on novel diagnostic criteria, CMR protocols, and prognostic significance of CMR findings in ACM.
IntroductionPredictors of late life-threatening arrhythmic events in Brugada syndrome (BrS) patients who received a prophylactic ICD implantation remain to be evaluated. The aim of the present long-term multicenter study was to assess the incidence and clinical-electrocardiographic predictors of late life-threatening arrhythmic events in BrS patients with a prophylactic implantable cardioverter defibrillator (ICD) and undergoing generator replacement (GR).MethodsThe study population included 105 patients (75% males; mean age 45 ± 14years) who received a prophylactic ICD and had no arrhythmic event up to first GR.ResultsThe median period from first ICD implantation to last follow-up was 155 (128–181) months and from first ICD Implantation to the GR was 84 (61–102) months. During a median follow-up of 57 (38–102) months after GR, 10 patients (9%) received successful appropriate ICD intervention (1.6%/year). ICD interventions included shock on ventricular fibrillation (n = 8 patients), shock on ventricular tachycardia (n = 1 patient), and antitachycardia pacing on ventricular tachycardia (n = 1 patient). At survival analysis, history of atrial fibrillation (log-rank test; P = 0.02), conduction disturbances (log-rank test; P < 0.01), S wave in lead I (log-rank test; P = 0.01) and first-degree atrioventricular block (log-rank test; P = 0.04) were significantly associated with the occurrence of late appropriate ICD intervention. At Cox-regression multivariate analysis, S-wave in lead I was the only independent predictor of late appropriate ICD intervention (HR: 9.17; 95%CI: 1.15–73.07; P = 0.03).ConclusionsThe present study indicates that BrS patient receiving a prophylactic ICD may experience late appropriate intervention after GR in a clinically relevant proportion of cases. S-wave in lead I at the time of first clinical evaluation was the only independent predictor of persistent risk of life-threatening arrhythmic events. These findings support the need for GR at the end of service regardless of previous appropriate intervention, mostly in BrS patients with conduction abnormalities.
Ischaemic preconditioning can protect the myocardium against ischaemic injury by opening of the adenosine triphosphate (ATP)-sensitive potassium (K(ATP)) channel. Isoflurane is also thought to open this channel. The present investigation tested the hypothesis that pre-ischaemic treatment with isoflurane mimics ischaemic preconditioning (producing chemical preconditioning) and thereby protects the myocardium against ischaemic injury in an isolated rat heart model. Control hearts underwent 30 min of global no-flow ischaemia followed by 60 min of reperfusion. The hearts of the preconditioning group underwent two 5 min periods of no-flow ischaemia interspersed with 5 min of reperfusion before the sustained ischaemia. In three additional groups, hearts were subjected to 15 min of 1.5 minimal alveolar concentration (MAC) of isoflurane (ISO-1), 15 min 3 MAC (ISO-2) or 25 min 1.5 MAC (ISO-3) of isoflurane followed by 5 min washout before the global ischaemia. Left ventricular (LV) developed pressure and creatine kinase release were measured as variables of myocardial performance and cellular injury, respectively. Recovery of LV developed pressure was improved after ischaemic preconditioning [after 60 min reperfusion, mean 63 (SEM 6)% of baseline] compared with the control group [18 (4)% P<0.01] but not by isoflurane, independently of concentration or duration of administration [ISO-1, 17 (2)%, P=0.99 vs control; ISO-2, 12 (3)%, P=0.64; ISO-3, 4 (1)%, P=0.06]. Total creatine kinase release over 1 h of reperfusion was not significantly different between control [251 (36) U g(-1) dry weight] and all isoflurane groups [ISO-1, 346 (24) U g(-1), P=0.30; ISO-2, 313 (33) U g(-1), P=0.73; ISO-3, 407 (40) U g(-1), P=0.03]. These results indicate that pre-ischaemic administration of isoflurane does not cause anaesthetic-induced preconditioning in the isolated rat heart.
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