Arrhythmogenic cardiomyopathy of left ventricle. A rare event, but possible

Abstract

Please cite this article as: M. Scarano, et al., Arrhythmogenic cardiomyopathy of left ventricle. A rare event, but possible, Cor et Vasa 60 (2018) e508-e511 as published in the online version of Cor et SOUHRN Arytmogenní dyplazie pravé komory (arrhythmogenic right ventricular dysplasia, ARVD) je formou dědičné kardiomyopatie charakterizované nahrazováním svaloviny převážně pravé komory fi brózní a tukovou tkání. U osob s tímto postižením může dojít k rozvoji život ohrožujících komorových arytmií a srdečnímu selhání. Ještě častěji se s ní lze setkat u sportovců, kteří prodělají náhlou srdeční smrt. Postižena však může být nejen pravá, ale i levá komora. Popisujeme případ 38letého muže se dvěma epizodami synkop v anamnéze. Po echokardiografi ckém vyšetření byl pacient odeslán na vyšetření srdce magnetickou rezonancí. Morfologické zobrazení srdce prokázalo tukovou infi ltraci epikardu postranní stěny levé komory (střední a apikální segmenty). Byla stanovena diagnóza izolované levostranné formy arytmogenní kardiomyopatie. Po implantaci kardioverteru-defi brilátoru byla zahájena farmakoterapie enalaprilem a bisoprololem.ABSTRACT Arrhythmogenic right ventricular dysplasia (ARVD) is a form of inherited cardiomyopathy characterized by fi bro-fatty substitution mainly right ventricular (RV). Affected patients may succumb to life-threatening ventricular arrhythmias and heart failure. It is even more common among athletes who experience sudden cardiac death (SCD). The disease involvement is not limited only to the RV, but the left ventricle (LV) can also be involved. We have reported a case of a 38-year-old man, with two episodes of syncope in his history. After echocardiographic investigations, the patient was referred to cardiac magnetic resonance (CMR). Morphological images showed fatty infi ltration of the epicardial layer of LV lateral wall (mid and apical segment).

Arrhythmogenic Cardiomyopathy: A Review of a Rare Case of Biventricular Phenotype

“…Our case presented with syncope but denied chest pain, dyspnoea, and orthopnoea. Syncope is also a common but inconsistent presentation as some case reports [10,28,29] did not document it while others [30][31][32][33] did. Bariani et al [34] described at least a single episode of chest pain in about 90% of the subjects they studied; however, this was absent in our case.…”

“…Bariani et al [34] described at least a single episode of chest pain in about 90% of the subjects they studied; however, this was absent in our case. Other documented findings that precede the diagnosis of ACM include heart failure [10,29,31], ventricular arrhythmias or cardiac arrest [10,30,32,33], and sudden cardiac death [10,31,33].…”

Usefulness of Sacubitril/Valsartan in reduction of atrial fibrillation burden in a patient with ICD delivering inappropriate therapies. A new possibility?

“…Many conditions different from coronary artery disease leading to HF may have benefi t from advanced non-pharmacological therapy. 12,13 While the exact impact in different cardiac disorders 14,15 is currently under investigation and new interesting therapeutic perspectives are currently emerging, 16 it is clear that HF is more complex than believed. The use of cardiac devices poses problems deriving both from patients 17 and from the risk of infections 18 even if it is often necessary.…”