Isolated ventricular noncompaction mimicking arrhythmogenic right ventricular cardiomyopathy — A study of nine patients

Włodarska, Elżbieta K.; Woźniak, Olgierd; Konka, Marek; Piotrowska-Kownacka, Dorota; Walczak, Ewa; Hoffmań, Piotr

doi:10.1016/j.ijcard.2009.05.062

Cited by 27 publications

(23 citation statements)

References 18 publications

(29 reference statements)

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“…Differential diagnoses, which have to be considered before the diagnosis of LVHT can be established include: prominent normal myocardial trabeculations, which are always \4 in number, have the same echogenicity as the myocardium, protrude into the cavity, do not link to another segment, and are only rarely located in the apex; false tendons and aberrant bands, which are myocardial structures that communicate with the myocardium on both ends and can be visualised echocardiographically by using different imaging planes; LV apical thrombi, which have a lower echogenicity as the myocardium and do not move synchronously with it; myocardial abscesses, which are difficult to assess on echocardiography [49]; arrhythmogenic right ventricular cardiomyopathy [50]; hypertrophic cardiomyopathy (hCMP), which is diagnosed according to established criteria [51]; apical form of hCMP, which is best detected on cMRI; myocardial haematoma, which are difficult to diagnose on echocardiography [49]; myocardial infiltrative disease (amyloidosis), which is diagnosed upon endomyocardial biopsy [30]; and cardiac tumours or cardiac metastases, which can be confirmed only histologically.…”

Section: Differential Diagnosesmentioning

confidence: 99%

Left ventricular non-compaction and its cardiac and neurologic implications

Finsterer

2010

Heart Fail Rev

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Left ventricular non-compaction, also known as left ventricular hypertrabeculation (LVHT), is a morphological abnormality of the left ventricular myocardium, characterised by a meshwork of myocardial strings, interlacing, and orderless in arrangement. LVHT is most frequently located in the apex and the lateral wall and may occur with or without other congenital or acquired cardiac abnormalities. LVHT is believed to be congenital in the majority of the cases but may develop during life in single cases (acquired LVHT). Congenital LVHT is believed to result from defective late-stage embryonic development of the myocardial architecture. The pathogenesis of acquired LVHT remains speculative. LVHT is most frequently found on transthoracic echocardiography and cardiac MRI but may be visualised also with other imaging techniques. In the majority of the cases, LVHT is associated with hereditary cardiac, neuromuscular, non-cardiac/non-muscle disease, or chromosomal aberrations. In the majority of the cases, LVHT is complicated by ventricular arrhythmias, systolic dysfunction, cardiac embolism, or sudden cardiac death. LVHT per se does not require a specific treatment. Only in case of complications, such as ventricular arrhythmias, cardioembolism, or systolic dysfunction, adequate therapy is indicated. Though initially assessed as poor, the prognosis of LVHT has meanwhile improved, most likely due to the increased awareness for the abnormality and the timely administration of adequate therapy.

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Section: Differential Diagnosesmentioning

confidence: 99%

Left ventricular non-compaction and its cardiac and neurologic implications

Finsterer

2010

Heart Fail Rev

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“…The cause of subendocardial fibrosis in LVHT is unknown, but several hypotheses have been raised to explain the frequent abnormality. The most commonly mentioned explanation assumes that it is due to chronic ischemia, which may result from hypertrophy or insufficient vascular supply of the trabeculations [14]. In this case, subendocardial fibrosis may result from chronic ischemia of the inner myocardial layer, e.g.…”

Section: Discussionmentioning

confidence: 99%

Ultrastructural Findings in Noncompaction Prevail with Neuromuscular Disorders

Finsterer

Stöllberger²

2013

Cardiology

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Objectives: Little is known about the ultrastructural abnormalities of left ventricular hypertrabeculation/noncompaction (LVHT). This literature review aimed to summarize and discuss ultrastructural abnormalities described in LVHT so far. Methods: The literature search was conducted via MEDLINE using the search terms ‘non-compaction', ‘noncompaction', ‘left ventricular hypertrabeculation', ‘spongy myocardium' in combination with the terms ‘ultra-structural', or ‘electron microscopy'. Results: Altogether, 11 studies reporting ultrastructural investigations of LVHT were retrieved. In these 11 studies, data on 13 patients with LVHT were presented. Ultrastructural abnormalities found in these study patients were generally nonspecific and included an increase in the number of mitochondria (n = 3), abnormally shaped mitochondria (n = 2), distorted cristae (n = 3), sarcomeric derangement (n = 3), immature cardiomyocytes (n = 1), lipid-like inclusions (n = 1), enlarged interstitial spaces (n = 1), increased interstitial collagen (n = 1), or increased glycogen (n = 1). The morphological abnormalities were most prominent in patients with a neuromuscular disorder like Barth syndrome or mitochondrial myopathy. Conclusions: Only in few patients with LVHT, ultrastructural investigations have been performed so far. Ultrastructural abnormalities in LVHT are nonspecific and most prominent in patients with a neuromuscular disorder. There is a strong need to carry out thorough ultrastructural investigations of LVHT to contribute to the understanding of this still unexplained myocardial abnormality.

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“…Ventricular tachyarrhythmias had a reported incidence of 41% in a series [2]. Sustained monomorphic ventricular tachycardias with RBBB [4] or LBBB morphologies [5] or alternating morphologies [6] were reported previously, as well as polymorphic VT or VF [7]. Echocardiographic criteria for LVNC include blood-filled deep intertrabecular recessus separating hypertrabeculated noncompacted myocardium, with an endocardial to epicardial ratio of 2 or more measured at end-systole [1].…”

Section: Discussionmentioning

confidence: 99%

Exercise-Induced Right Ventricular Outflow Tract Tachycardia in a Patient with Isolated Left Ventricular Noncompaction

et al. 2011

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Isolated left ventricular noncompaction is a hereditary cardiomyopathy in which a variety of supraventricular and ventricular arrhythmias could be observed. We report a patient with exercise-induced ventricular tachycardia with left bundle branch block morphology that had characteristics of an idiopathic ventricular tachycardia who was subsequently diagnosed as left ventricular noncompaction. Successful remission of arrhythmia was ensured after the introduction of oral beta-blocker therapy.

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Isolated ventricular noncompaction mimicking arrhythmogenic right ventricular cardiomyopathy — A study of nine patients

Cited by 27 publications

References 18 publications

Left ventricular non-compaction and its cardiac and neurologic implications

Left ventricular non-compaction and its cardiac and neurologic implications

Ultrastructural Findings in Noncompaction Prevail with Neuromuscular Disorders

Exercise-Induced Right Ventricular Outflow Tract Tachycardia in a Patient with Isolated Left Ventricular Noncompaction

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