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Pathomorphologic findings in left ventricular hypertrabeculation/noncompaction of adults in relation to neuromuscular

Daniel Gerger1, Claudia Stöllberger, Martin Grassberger

  • 1Krankenanstalt Rudolfstiftung, Juchgasse 25, A-1030 Wien, Austria.

International Journal of Cardiology
|November 5, 2013
PubMed

Insights

Pathomorphologic assessment of left ventricular hypertrabeculation/noncompaction (LVHT) requires formaldehyde fixation and both long- and short-axis cardiac cuts. Subendocardial fibrosis is a consistent finding in LVHT.

Area of Science:

  • Cardiology
  • Pathology
  • Medical Imaging

Background:

  • Left ventricular hypertrabeculation/noncompaction (LVHT) is a congenital cardiomyopathy.
  • Echocardiography (ECHO) is commonly used for LVHT diagnosis.

Purpose of the Study:

  • To correlate echocardiographic (ECHO) findings with pathomorphologic (PATHO) features of LVHT.
  • To evaluate optimal methods for pathomorphologic assessment of LVHT.

Main Methods:

  • Echocardiographic criteria for LVHT included >3 trabeculations, synchronous movement, and a two-layered myocardium.
  • Autopsy hearts were examined fresh or formaldehyde-fixed, with long-axis and short-axis cuts.
  • Pathologists' preferences guided specimen investigation.

Main Results:

  • Twelve patients with LVHT were studied; 7 had neuromuscular disorders.
  • Formaldehyde fixation improved visualization of trabecular meshwork.
  • Long-axis cuts aided differentiation from papillary muscles; short-axis cuts revealed the two-layered structure.
  • Subendocardial fibrosis was present in all cases; trabeculation counting was impossible due to complex geometry.

Conclusions:

  • Formaldehyde fixation is recommended for comparing ECHO and PATHO findings in LVHT.
  • Both long- and short-axis cuts are essential for assessing trabeculations and myocardial structure.
  • Subendocardial fibrosis in LVHT warrants further investigation.
Abstract

Keywords:
AutopsyCardiomyopathyMuscular dystrophyMyopathyNoncompactionPathology

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