Noncompaction cardiomyopathy, a frequently overlooked entity (…but beware of over diagnosis!)

Çalışkan, Kadir

doi:10.1007/s12471-012-0314-6

Cited by 5 publications

(5 citation statements)

References 14 publications

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“…Familial occurrence of LVNC, particularly in first-degree relatives of affected patients, has been reported in 13% to 50% of the cases, 11,30,38,64,65 and, for this reason, it is recommended that first degree relatives of patients with LVNC be investigated for LVNC (or other cardiomyopathy) as well. Genetic transmission of LVNC is usually an autosomal dominant, but autosomal recessive, X-linked, or mitochondrial inheritance has been reported.…”

Section: Geneticsmentioning

confidence: 99%

Cardiomyopathies Due to Left Ventricular Noncompaction, Mitochondrial and Storage Diseases, and Inborn Errors of Metabolism

Towbin

Jefferies

2017

Circulation Research

119

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The normal function of the human myocardium requires the proper generation and utilization of energy and relies on a series of complex metabolic processes to achieve this normal function. When metabolic processes fail to work properly or effectively, heart muscle dysfunction can occur with or without accompanying functional abnormalities of other organ systems, particularly skeletal muscle. These metabolic derangements can result in structural, functional, and infiltrative deficiencies of the heart muscle. Mitochondrial and enzyme defects predominate as disease-related etiologies. In this review, left ventricular noncompaction cardiomyopathy, which is often caused by mutations in sarcomere and cytoskeletal proteins and is also associated with metabolic abnormalities, is discussed. In addition, cardiomyopathies resulting from mitochondrial dysfunction, metabolic abnormalities, storage diseases, and inborn errors of metabolism are described.

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Section: Geneticsmentioning

confidence: 99%

Cardiomyopathies Due to Left Ventricular Noncompaction, Mitochondrial and Storage Diseases, and Inborn Errors of Metabolism

Towbin

Jefferies

2017

Circulation Research

119

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Section: Discussionmentioning

confidence: 99%

“…The patient of this case report suffered from syncope induced by a ventricular tachycardia as a consequence of noncompaction cardiomyopathy (NCCM). NCCM is a relatively new and frequently overlooked diagnosis [ 9 , 10 ]. Even with the help of modern imaging modalities, such as contrast echocardiography and MRI, diagnosing NCCM can be difficult [ 10 ].…”

Section: Discussionmentioning

confidence: 99%

“…NCCM is a relatively new and frequently overlooked diagnosis [ 9 , 10 ]. Even with the help of modern imaging modalities, such as contrast echocardiography and MRI, diagnosing NCCM can be difficult [ 10 ]. The clinical spectrum of NCCM shows a highly variable clinical presentation, ranging from asymptomatic to severely symptomatic with end-stage heart failure, lethal arrhythmias, and/or thromboembolic events [ 11 ].…”

Section: Discussionmentioning

confidence: 99%

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Loss of consciousness and convulsion induced by a ventricular tachycardia mimicking epilepsy in a patient with noncompaction cardiomyopathy: a case report

et al. 2013

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Convulsions and loss of consciousness can be caused by, among other things, arrhythmias, conduction disorders or epilepsy. In clinical practice it can be difficult to distinguish between these causes of syncope, even for well-trained specialists. Patients with cardiac syncope have a substantial risk of subsequent sudden death. We present a patient with previously unknown noncompaction cardiomyopathy in whom syncope induced by ventricular tachycardia was misinterpreted as epilepsy. We present this case report in order to underline the necessity for cardiological assessment in patients with assumed mild epilepsy or syncope of unknown origin.

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“…Left ventricular non-compaction cardiomyopathy (LVNC) is perceived as a rare genetic cardiomyopathy characterized by abnormal arrest of in-utero myocardial compaction (1). Tertiary center cohorts of LVNC report association with lifethreatening arrhythmias, thromboembolism, and left ventricular (LV) dysfunction (2)(3)(4)(5). LVNC is recognized as a "genetic cardiomyopathy" by the American Heart Association and as an "unclassified cardiomyopathy" by the European Society of Cardiology (6,7).…”

Section: Introductionmentioning

confidence: 99%

Left Ventricular Hypertrabeculation Is Not Associated With Cardiovascular Morbity or Mortality: Insights From the Eurocmr Registry

Zemrak

Raisi‐Estabragh

Khanji

et al. 2020

Front. Cardiovasc. Med.

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Aim: Left ventricular non-compaction (LVNC) is perceived as a rare high-risk cardiomyopathy characterized by excess left ventricular (LV) trabeculation. However, there is increasing evidence contesting the clinical significance of LV hyper-trabeculation and the existence of LVNC as a distinct cardiomyopathy. The aim of this study is to assess the association of LV trabeculation extent with cardiovascular morbidity and all-cause mortality in patients undergoing clinical cardiac magnetic resonance (CMR) scans across 57 European centers from the EuroCMR registry. Methods and Results: We studied 822 randomly selected cases from the EuroCMR registry. Image acquisition was according to international guidelines. We manually segmented images for LV chamber quantification and measurement of LV trabeculation (as per Petersen criteria). We report the association between LV trabeculation extent and important cardiovascular morbidities (stroke, atrial fibrillation, heart failure) and all-cause mortality prospectively recorded over 404 ± 82 days of follow-up. Maximal non-compaction to compaction ratio (NC/C) was mean (standard deviation) 1.81 ± 0.67, from these, 17% were above the threshold for hyper-trabeculation (NC/C > 2.3). LV trabeculation extent was not associated with increased risk of the defined outcomes (morbidities, mortality, LV CMR indices) in the whole cohort, or in sub-analyses of individuals without ischaemic heart disease, or those with NC/C > 2.3. Zemrak et al. LV Hypertrabeculation and Cardiovascular Outcomes Conclusion: Among 882 patients undergoing clinical CMR, excess LV trabeculation was not associated with a range of important cardiovascular morbidities or all-cause mortality over ∼12 months of prospective follow-up. These findings suggest that LV hyper-trabeculation alone is not an indicator for worse cardiovascular prognosis.

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Noncompaction cardiomyopathy, a frequently overlooked entity (…but beware of over diagnosis!)

Cited by 5 publications

References 14 publications

Cardiomyopathies Due to Left Ventricular Noncompaction, Mitochondrial and Storage Diseases, and Inborn Errors of Metabolism

Cardiomyopathies Due to Left Ventricular Noncompaction, Mitochondrial and Storage Diseases, and Inborn Errors of Metabolism

Loss of consciousness and convulsion induced by a ventricular tachycardia mimicking epilepsy in a patient with noncompaction cardiomyopathy: a case report

Left Ventricular Hypertrabeculation Is Not Associated With Cardiovascular Morbity or Mortality: Insights From the Eurocmr Registry

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