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Education in Heart
Left ventricular non-compaction cardiomyopathy: how many needles in the haystack?
  1. Andrew D'Silva1,2,
  2. Bjarke Jensen3
  1. 1 Department of Cardiology, Guy's and St Thomas' NHS Foundation Trust, London, UK
  2. 2 School of Cardiovascular Medicine & Sciences, King’s College London, London, UK
  3. 3 Department of Medical Biology, University of Amsterdam, Amsterdam, The Netherlands
  1. Correspondence to Dr Andrew D'Silva, Department of Cardiology, Guy's and St Thomas' NHS Foundation Trust, London SE1 7EH, UK; andrew.dsilva{at}gstt.nhs.uk

https://doi.org/10.1136/heartjnl-2020-316945

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    Learning objectives

    • Understand the origin of left ventricular trabeculation.

    • Appreciate the multiple current methods of left ventricular trabeculation assessment and their limitations.

    • Understand the current controversies in investigation and management of patients with excessive left ventricular trabeculation.

    Introduction

    Left ventricular non-compaction cardiomyopathy (LVNC) remains a subject of unsettled debate between those who perceive it to be a primary genetic cardiomyopathy1 and those who believe excessive trabeculation to be a morphological feature shared by distinct pathological processes and therefore not a single disease.2

    The defining abnormality is the presence of prominent left ventricular (LV) trabeculae and deep intertrabecular recesses, continuous with the LV cavity and separated from the epicardial coronary arteries.3 This was initially thought to be the consequence of embryological arrest of normal endomyocardial morphogenesis, but contemporary studies dispute this popular theory.

    This article showcases the most up-to-date research regarding LV trabeculation and non-compaction cardiomyopathy. Practical guidance is offered relating to cardiac diagnostic imaging and clinical management, with ambition to resolve misconceptions about the origins and diagnosis of LVNC. Finally, we aim to provide insights into future research that might illuminate persisting ambiguities.

    Embryological perspective

    In normal development, trabeculae and compact myocardium grow at different rates

    Around 4 weeks after conception, the primitive heart begins to develop its four chambers. At this early stage, there is no coronary circulation and chamber walls thicker than 50 µm or so are at risk of becoming ischaemic.4 A wall made of thin trabeculae that protrude into the cavity, however, will resist ischaemia bathing in the chamber blood.5 Thus, in the absence of coronary circulation in the fourth and fifth week after conception, trabeculae grow fast, accounting for most of the ventricular mass.6 7 Then, around the transition from embryo to fetus, trabecular growth becomes slow relative to the compact wall, and in this period, the ventricular wall acquires an almost adult-like appearance, or expressed numerically, …

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    Footnotes

    • Twitter @AndrewJMDSilva

    • Contributors AD devised the project, the main conceptual ideas and proof outline. Both authors wrote the article and contributed original material to the final manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Commissioned; externally peer reviewed.

    • Author note References that include a * are considered to be key references.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.