Limitations in the Diagnosis of Noncompaction Cardiomyopathy by
            Echocardiography

Hotta, Viviane Tiemi; Tendolo, Sabrina Cunha; Rodrigues, Ana Clara Tude; Fernandes, Fábio; Nastari, Luciano; Mady, Charles

doi:10.5935/abc.20170152

Cited by 6 publications

(15 citation statements)

References 26 publications

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“…295,296 Its incidence and prevalence are uncertain, ranging from 0.02 to 0.05% according to some echocardiographic records. 297 Clinically, it can be asymptomatic or present with symptoms of HF, ventricular and/or atrial arrhythmias, pre-excitation, thromboembolic events, or SCD. There are no universally accepted criteria for morphological diagnosis; however, a ratio between noncompacted/compacted myocardium greater than 2.1:1 at the end of systole on echocardiography or 2.3:1 at the end of systole on MRI has become the most widely accepted proposed criterion.…”

Section: Noncompaction Cardiomyopathymentioning

confidence: 99%

Diretriz Brasileira de Reabilitação Cardiovascular – 2020

Carvalho¹,

Milani²,

Ferraz³

et al. 2020

Arquivos Brasileiros De Cardiologia

116

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Section: Noncompaction Cardiomyopathymentioning

confidence: 99%

Diretriz Brasileira de Reabilitação Cardiovascular – 2020

Carvalho¹,

Milani²,

Ferraz³

et al. 2020

Arquivos Brasileiros De Cardiologia

116

View full text Add to dashboard Cite

show abstract

“…Among HF patients, the prevalence of non-compacted cardiomyopathy is 4%. 148 Diagnosis is quite difficult due to the lack of clear criteria, as well as the condition's heterogeneous clinical spectrum and the usual need for MRI for a reliable diagnosis.…”

Section: Individuals With Cardiomyopathies and Myocarditismentioning

confidence: 99%

The Brazilian Society of Cardiology and Brazilian Society of Exercise and Sports Medicine Updated Guidelines for Sports and Exercise Cardiology - 2019

Ghorayeb¹,

Stein²,

Daher³

et al. 2019

Arquivos Brasileiros De Cardiologia

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“…These criteria are based on the relationship between the compaction and non-compaction layers; however, they differ regarding the time of the cardiac cycle in which the measurement must be performed 8 . The criterion proposed by Jenni 11 consists of a ratio greater than two at the end of systole, being the most frequently used in studies available in the literature 2,28,29 .…”

Section: Discussionmentioning

confidence: 99%

“…In 1986, the first echocardiographic report characterized it as spongy myocardium, and only in 2006 was it classified as a primary genetic CM by the American Heart Association (AHA). However, it remains a familial CM not classified by the European Society of Cardiology (ESC) 2,[5][6][7][8] . NCM is morphologically characterized by a myocardium with trabeculations and deep recesses that communicate with the ventricular cavity.…”

Section: Introductionmentioning

confidence: 99%

“…NCM can occur at any age, with a very heterogeneous clinical presentation ranging from asymptomatic patients to heart failure (HF), thromboembolic events, arrhythmias, and sudden death 2,6,9 . Different echocardiographic and magnetic resonance diagnostic criteria have been proposed by different authors, hindering an accurate diagnosis 2,8,10 .…”

Section: Introductionmentioning

confidence: 99%

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Non-Compacted Cardiomyopathy in Children and Adolescents: From the Challenge of Echocardiographic Diagnosis to Clinical Follow-Up

Torbey¹,

Bustamante²,

Souza³

et al. 2021

Arq Bras Cardiol: Imagem Cardiovasc

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Background: Non-compacted cardiomyopathy (NCCM) is characterized by hypertrabeculations and deep recesses in the left ventricle, with a heterogeneous clinical presentation, ranging from asymptomatic patients to those with heart failure (HF), thromboembolic events and arrhythmias with risk of sudden death. As it is rare and does not have well-defined diagnostic criteria, its natural history in pediatrics is poorly understood. This study describes the clinical presentation and clinical course of patients with NCCM.Methodology: Observational, longitudinal, prospective study of pediatric patients seen at a pediatric cardiology referral center from metropolitan region II in the state of Rio de Janeiro, with NCCM phenotype on echocardiogram (ECHO) during a 2-year follow-up, from the ChARisMa registry.Results: 6 patients aged 4 to 14, with NCCM, were analyzed. Mean age 7.5 years (SD: 3.93), 3 males (50%). The patients presented HF (n=2), cardiac murmur (n=1), cardiac arrhythmia (n=1), were asymptomatic (n=1) or were under investigation for a genetic syndrome (n=1). Phenotypes on ECHO: NCCM/dilated cardiomyopathy (n=1) and NCCM/restrictive cardiomyopathy (n=1), isolated phenotype of NCCM (n=4). Cardiac magnetic resonance imaging was performed and confirmed the diagnosis (n=4). The outcomes observed were thromboembolism, indication for heart transplantation, and sustained ventricular tachycardia. Conclusions:This case series provides relevant data for pediatric NCCM as it shows its heterogeneous clinical presentation and potentially fatal complications. More prospective studies are needed for an accurate diagnosis and to allow its clinical course, therapeutic response and prognosis to be better known.

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Limitations in the Diagnosis of Noncompaction Cardiomyopathy by Echocardiography

Cited by 6 publications

References 26 publications

Diretriz Brasileira de Reabilitação Cardiovascular – 2020

Diretriz Brasileira de Reabilitação Cardiovascular – 2020

The Brazilian Society of Cardiology and Brazilian Society of Exercise and Sports Medicine Updated Guidelines for Sports and Exercise Cardiology - 2019

Non-Compacted Cardiomyopathy in Children and Adolescents: From the Challenge of Echocardiographic Diagnosis to Clinical Follow-Up

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