Preprint Article Version 1 Preserved in Portico This version is not peer-reviewed

Sudden Unexpected Death Associated with Arrhythmogenic Cardiomyopathy: Study of the Cardiac Conduction System

Version 1 : Received: 7 June 2021 / Approved: 9 June 2021 / Online: 9 June 2021 (08:49:09 CEST)

A peer-reviewed article of this Preprint also exists.

Ottaviani, G.; Alfonsi, G.; Ramos, S.G.; Buja, L.M. Sudden Unexpected Death Associated with Arrhythmogenic Cardiomyopathy: Study of the Cardiac Conduction System. Diagnostics 2021, 11, 1323. Ottaviani, G.; Alfonsi, G.; Ramos, S.G.; Buja, L.M. Sudden Unexpected Death Associated with Arrhythmogenic Cardiomyopathy: Study of the Cardiac Conduction System. Diagnostics 2021, 11, 1323.

Journal reference: Diagnostics 2021, 11, 1323
DOI: 10.3390/diagnostics11081323

Abstract

A retrospective study was conducted on pathologically diagnosed arrhythmogenic cardiomyopathy (ACM) from consecutive cases over the past 34 years (n = 1,109). The cardiac conduction system (CCS) was removed in two blocks, containing the following structures: Sino-atrial node (SAN), atrio-ventricular junction (AVJ) including the atrio-ventricular node (AVN), the His bundle (HB), the bifurcation (BIF), the left bundle branch (LBB) and the right bundle branch (RBB). The ACM cases (2.07% of the total cases) consisted of 20 (86.96%) sudden unexpected cardiac death (SUCD) and 3 (13.04%) native explanted hearts; 16 (69.56%) were males and 7 (30.44%) were females, ranging in age from 5 to 65 (mean age ± SD, 36.13 ± 16.06) years. The following anomalies of the CCS, displayed as percentages of ACM SUCD cases, have been detected: Hypoplasia of SAN (80%) and/or AVJ (86.67%) due to fatty-fibrous involvement, AVJ dispersion and/or septation (46.67%), central fibrous body (CFB) hypoplasia (33.33%), fibromuscular dysplasia of SAN (20%) and/or AVN (26.67%) arteries, hemorrhage and infarct-like lesions of CCS (13.33%), islands of conduction tissue in CFB (13.33%), Mahaim fibers (13.33%), LBB block by fibrosis (13.33%), AVN tongue (13.33%), HB duplicity (6.67%%), CFB cartilaginous meta-hyperplasia (6.67%), and right sided HB (6.67%). Arrhythmias are the hallmark of ACM, not only from the fatty-fibrous disruption of the ventricular myocardium that accounts for reentrant ventricular tachycardia, but also from the fatty-fibrous involvement of CCS itself. The careful examination of the cardiac conduction system on serial sections was crucial in documenting the fatty-fibrous infiltration of CCS in ACM.

Keywords

Arrhythmogenic cardiomyopathy; arrhythmogenic right ventricular cardiomyopathy; cardiac conduction system; sudden unexpected cardiac death; autopsy

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