Understanding left ventricular hypertrabeculation/noncompaction: pathomorphologic findings and prognostic impact of

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Claudia Stöllberger1, Josef Finsterer2

  • 1a 2nd Medical Department with Cardiology and Intensive Care Medicine , Rudolfstifung Hospital , Vienna , Austria.

Expert Review of Cardiovascular Therapy
|December 21, 2018
PubMed

Insights

Left ventricular hypertrabeculation/noncompaction (LVHT) is linked to neuromuscular disorders (NMDs). Systematic investigation reveals NMDs in 80% of LVHT patients, impacting prognosis for both conditions.

Area of Science:

  • Cardiology
  • Neurology
  • Pathology

Background:

  • Left ventricular hypertrabeculation/noncompaction (LVHT) is diagnosed via cardiac imaging and can occur with extracardiac disorders, particularly neuromuscular disorders (NMDs).
  • Despite its detection across all ages, many aspects of LVHT remain unclear, necessitating further investigation into its pathology and associations.

Purpose of the Study:

  • To review pathomorphologic findings in LVHT patients over 14 years old.
  • To elucidate the role of NMDs in the context of LVHT.

Main Methods:

  • A comprehensive PubMed search was conducted using terms related to noncompaction, hypertrabeculation, autopsy, biopsy, ultrastructure, electron microscopy, and neuromuscular disorders.
  • Analysis included macroanatomic, histopathologic, and ultrastructural reports, with comparisons to echocardiography where available.

Main Results:

  • Endocardial, subendocardial, and interstitial fibrosis were common findings in LVHT hearts.
  • Systematic investigation of LVHT patients identified NMDs in 80%, with mitochondrial disorders, Barth syndrome, X-linked myopathy with postural muscle atrophy (XMPMA), and myotonic dystrophy type 1 being most frequent.
  • LVHT is not exclusively a cardiac manifestation of NMDs, and bidirectional prognostic effects between LVHT and NMDs (e.g., Duchenne muscular dystrophy) were observed.

Conclusions:

  • LVHT is frequently associated with NMDs, suggesting a complex interplay rather than LVHT being a specific cardiac manifestation of NMDs.
  • The presence of NMDs influences the prognosis of LVHT, and conversely, LVHT impacts the prognosis in certain NMDs.
Abstract

Keywords:
Autopsycardiomyopathyechocardiographyhistologymagnetic resonance imagingpathomorphology

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