Arrhythmogenic Left Ventricular Cardiomyopathy Associated With Noncompaction

Matsukuma, Seiji; Eishi, Kiyoyuki; Hashizume, Koji; Oshitomi, Takashi; Ariyoshi, Tsuneo; Taniguchi, Shinichiro; Hisatomi, Kazuki; Hayashi, Tomayoshi; Abe, Kuniko

doi:10.1016/j.athoracsur.2010.06.004

Cited by 10 publications

(7 citation statements)

References 8 publications

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“…Although there are rare cases of AC associated with LV-NC in literature, 3) 5) 6) 7) it has been debatable that it is a cardiomyopathy as a consequence of an underlying same genetic factor or a co-existence of two different entities. 6) …”

Section: Discussionmentioning

confidence: 99%

Arrhythmogenic Noncompaction Cardiomyopathy: Is There an Echocardiographic Phenotypic Overlap of Two Distinct Cardiomyopathies?

Aras

Özeke

Çay

et al. 2015

J Cardiovasc Ultrasound

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The clinical diagnosis of right ventricular (RV) cardiomyopathies is often challenging. It is difficult to differentiate the isolated left ventricular (LV) noncompaction cardiomyopathy (NC) from biventricular NC or from coexisting arrhythmogenic ventricular cardiomyopathy (AC). There are currently few established morphologic criteria for the diagnosis other than RV dilation and presence of excessive regional trabeculation. The gross and microscopic changes suggest pathological similarities between, or coexistence of, RV-NC and AC. Therefore, the term arrhythmogenic right ventricular cardiomyopathy is somewhat misleading as isolated LV or biventricular involvement may be present and thus a broader term such as AC should be preferred. We describe an unusual case of AC associated with a NC in a 27-year-old man who had a history of permanent pacemaker 7 years ago due to second-degree atrioventricular block.

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Section: Discussionmentioning

confidence: 99%

Arrhythmogenic Noncompaction Cardiomyopathy: Is There an Echocardiographic Phenotypic Overlap of Two Distinct Cardiomyopathies?

Aras

Özeke

Çay

et al. 2015

J Cardiovasc Ultrasound

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“…Imaging modalities usually reveal severe LV dysfunction with preserved to mildly impaired RV function. Often this entity has been misdiagnosed as dilated cardiomyopathy, chronic myocarditis, myocardial infarction, or double ventricular involvement type ARVC 12,13 . Although there have been a handful publications detailing this rare disease, the majority of these have been case reports 7,[13][14][15] , and both the clinical cardiac magnetic resonance (CMR) characteristics remain poorly studied and consistent nomenclature for this cardiomyopathy is not present.…”

mentioning

confidence: 99%

“…Often this entity has been misdiagnosed as dilated cardiomyopathy, chronic myocarditis, myocardial infarction, or double ventricular involvement type ARVC 12,13 . Although there have been a handful publications detailing this rare disease, the majority of these have been case reports 7,[13][14][15] , and both the clinical cardiac magnetic resonance (CMR) characteristics remain poorly studied and consistent nomenclature for this cardiomyopathy is not present. It has been referred to as both arrhythmogenic left ventricular cardiomyopathy(ALVC) 7,12,16 and desmosomal cardiomyopathy 17 .…”

mentioning

confidence: 99%

Arrhythmogenic Left Ventricular Cardiomyopathy: A Clinical and CMR Study

et al. 2020

Sci Rep

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The clinical features, CMR characteristics and outcomes of arrhythmogenic left ventricular cardiomyopathy (ALVC), which is a very rare nonischemic cardiomyopathy, are currently not well studied. The purpose of the study is to investigate the clinical and cardiovascular magnetic resonance (CMR) imaging characteristics of arrhythmogenic left ventricular cardiomyopathy (ALVC). Fiftythree consecutive patients with ALVC were divided into two groups: ALVC patients without right ventricular (RV) involvement (n = 36, group 1) and those with RV involvement (n = 17, group 2). Clinical symptoms, cardiac electrophysiological findings, and CMR parameters (morphology, ventricular function, and myocardial fibrosis and fatty infiltration) were evaluated in both groups. The two groups showed no significant difference in age, gender, or presenting symptoms (P > 0.05). Right bundle branch block ventricular arrhythmia was less common in patients without RV involvement (50.0% vs.64.7%, P = 0.031). There were no significant differences in left ventricular function between the two groups, however right ventricular ejection fraction was significantly lower in group 2 (40.1 ± 4.0% vs. 48.7 ± 3.9%, P < 0.001). Inverse correlations of left ventricular ejection fraction with fat volume (r = −0.883, p = 0.001), late gadolinium enhancement (LGE) volume (r = −0.892, 0.013), ratio of fat/ LGE (r = −0.848, p < 0.001), indexed left ventricular end diastolic volume (r = −0.877, p < 0.001) and indexed left ventricular end systolic volume (r = −0.943, p < 0.001) were all significant. ALVC is a rare disease with fibro-fatty replacement predominantly in the left ventricle, impaired left ventricular systolic function, and ventricular arrhythmias originating from the left ventricle. ALVC with right ventricular involvement may have a worse prognosis. Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare cardiomyopathy with a prevalence of 1/1000 to 1/5000 1. It has been described as an inherited cardiomyopathy characterized by progressive fibro-fatty or fatty replacement of the right ventricular (RV) myocardium resulting in abnormalities of RV morphology and function 2-5. Left ventricular function is often preserved at early stages of ARVC and even with end-stage disease, left ventricular morphology and function are much less affected than the right ventricle 4,6. However, cases of isolated

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“…Сочетание различных форм некомпактной кардиомиопатии с аритмогенной кардиомиопатией. В литературе сообщают о единичных наблюдениях НМЛЖ, ассоциированного с аритмогенной желудочковой кардиомиопатией [45][46][47][48]. Однако остается спорным вопрос, имеет ли место сосуществование двух различных заболеваний или аритмия является симптомом НМЛЖ [49].…”

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Noncompaction Cardiomyopathy

Romero-Rodríguez

Bansmann²,

Beyer³

2019

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научный центр трансплантологии и искусственных органов имени академика В.И. Шумакова» Минздрава России, Москва, Российская Федерация 2 Кафедра трансплантологии и искусственных органов ГБОУ ВПО «Первый Московский государственный медицинский университет имени И.М. Сеченова» Минздрава России, Москва, Российская Федерация В статье представлен обзор литературы по различным аспектам некомпактной кардиомиопатии. Некомпактная кардиомиопатия -относительно редкое врожденное заболевание сердца, характеризующееся чрезмерным развитием трабекулярной сети и глубокими межтрабекулярными впадинами миокарда желудочков. Наиболее часто встречается неуплотненный миокард левого желудочка (НМЛЖ). НМЛЖ может быть изолированным или сосуществовать с другими кардиальными и системными аномалиями. Неуплотнение диагностируют, используя эхокардиографию или магнитно-резонансную томографию. Клинические проявления варьируют в широких пределах -от бессимптомных пациентов до пациентов с желудочковыми аритмиями, застойной сердечной недостаточностью и тромбоэмболиями. Некомпактный миокард является нерешенной клинической задачей в силу недостаточной точности неинвазивной диагностики и стратификации пациентов, определения прогноза и в ряде случаев -тактики их лечения.

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Arrhythmogenic Left Ventricular Cardiomyopathy Associated With Noncompaction

Cited by 10 publications

References 8 publications

Arrhythmogenic Noncompaction Cardiomyopathy: Is There an Echocardiographic Phenotypic Overlap of Two Distinct Cardiomyopathies?

Arrhythmogenic Noncompaction Cardiomyopathy: Is There an Echocardiographic Phenotypic Overlap of Two Distinct Cardiomyopathies?

Arrhythmogenic Left Ventricular Cardiomyopathy: A Clinical and CMR Study

Noncompaction Cardiomyopathy

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