Cardiogenetics (Apr 2022)

Left Ventricular Non-Compaction Spectrum in Adults and Children: From a Morphological Trait to a Structural Muscular Disease

  • Flavia Fusco,
  • Nunzia Borrelli,
  • Rosaria Barracano,
  • Giovanni Domenico Ciriello,
  • Federica Verrillo,
  • Giancarlo Scognamiglio,
  • Berardo Sarubbi

DOI
https://doi.org/10.3390/cardiogenetics12020016
Journal volume & issue
Vol. 12, no. 2
pp. 170 – 184

Abstract

Read online

Left ventricular non-compaction (LVNC) is an extremely heterogeneous disorder with a highly variable clinical presentation, morphologic appearance at imaging testing, and prognosis. It is still unclear whether LVNC should be classified as a separate cardiomyopathy or if it is a mere morphological trait shared by many phenotypically distinct cardiomyopathies. Moreover, the hypertrabeculated phenotype may be reversible in some cases, possibly reflecting the left ventricular physiological response of the cardiac muscle to chronic overload. The current diagnostic criteria have several limitations, leaving many patients in a grey area. Here, we review the available literature on LVNC in order to provide an overview of the current knowledge on this complex disorder.

Keywords