Reduced regional systolic function is not confined to the noncompacted segments in noncompaction cardiomyopathy

Attila Nemes1, Kadir Caliskan, Marcel L Geleijnse

  • 12nd Department of Medicine and Cardiology Centre, University of Szeged, Hungary.

Insights

Systolic dysfunction in isolated ventricular noncompaction (IVNC) affects both compacted and noncompacted left ventricular (LV) segments. This suggests LV dysfunction in IVNC is widespread, not limited to the noncompacted areas.

Area of Science:

  • Cardiology
  • Genetics
  • Medical Imaging

Background:

  • Isolated ventricular noncompaction (IVNC) is a rare genetic cardiomyopathy.
  • Understanding the contribution of different left ventricular (LV) segments to dysfunction is crucial.

Purpose of the Study:

  • To investigate regional LV dysfunction in IVNC using real-time 3D echocardiography.
  • To compare the systolic performance of noncompacted versus compacted LV segments.

Main Methods:

  • Analysis of 289 LV segments from 17 IVNC patients and 153 segments from 9 controls.
  • Assessment of systolic performance using wall motion score (2D echo) and regional volume measurements (RT3DE).

Main Results:

  • Compacted LV segments in IVNC patients showed abnormal wall motion, though less so than noncompacted segments.
  • No correlation found between the extent of noncompaction and LV ejection fraction or end-diastolic volume.
  • Both noncompacted and compacted segments exhibited increased 3D regional volumes and reduced systolic function.

Conclusions:

  • LV systolic dysfunction in IVNC is not solely attributed to noncompacted segments.
  • The findings indicate a more global impact of IVNC on LV function.
Abstract

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