Non-Compacted Cardiomyopathy: Is there a Need of
a New Cardiomyopathy?
Volume 1 - Issue 5
Francesco Bianco1,2*, Piergiusto Vitulli1, Valentina Bucciarelli1, Alvin Chandra1,2, Masatoshi Minamisawa2,3 and
Sabina Gallina1
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- 1Institute of Cardiology, Chieti, Italy
- 2Brigham and Women’s Hospital, Boston, USA
- 3Department of Cardiovascular Medicine, Matsumoto, Japan
*Corresponding author:
Francesco Bianco, Institute of Cardiology, Chieti, Italy
Received: April 04, 2019; Published: April 09, 2019
DOI: 10.32474/ACR.2018.01.000129
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Abstract
Left ventricular non-compaction (LVNC) is a myocardial disorder, classically defined as a double-layered myocardium, consisting
of a thick, spongy/hypertrabeculated, non-compacted endocardial segment and a thin, compacted, epicardial portion. The
American Heart Association (AHA) classifies LVNC as a distinct primary genetic cardiomyopathy, while the European Association
of Cardiology (ESC) as an unclassified cardiomyopathy. Despite the magnitude of the entire literature yield on this topic, to date
the pathogenesis, prognosis, and treatment are still unclear. Prevalence and mortality can range respectively from 0.05% to 0.26%
and 5% to 47%, but they are affected by the imaging criteria adopted for the diagnosis. In fact, LVNC has been for years incidentally
discovered during autopsy of unexplained sudden cardiac deaths. Conversely, with the advent of increasingly sophisticated
cardiac imaging techniques, the presence of hypertrabeculated myocardium has become very common. Both echocardiographic
and magnetic resonance criteria have been proven to overestimate the diagnosis, which shares a peculiar phenotype with other
pathologies. It is known that a hypertrabeculated left ventricle leads to a symptomatic triad consisting of heart failure, arrhythmias
and thromboembolisms. Therefore, it is current opinion of the authors that a “non-compaction cardiomyopathy” (NC-CMP) seems
to be the most comprehensive definition of a disease that, similarly to the other cardiomyopathies, and regardless of its etiology,
beyond a peculiar phenotype shares a distinct symptomatology and deserves to be listed as an entity between cardiomyopathies.
Keywords: Left Ventricle Non-Compaction; Non-Compacted Cardiomyopathy; Cardiomyopathies; Heart Failure; Sudden Cardiac
Death
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Introduction|
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Epidemiology|
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