Diagnostic value of myocardial deformation pattern in children with noncompaction cardiomyopathy

  • Putri Yubbu
  • Hythem M. Nawaytou
  • Renzo Calderon-Anyosa
  • Anirban Banerjee
Original Paper
  • 36 Downloads

Abstract

The current echocardiographic diagnostic criteria for noncompaction cardiomyopathy (NCC) have variable sensitivity and low specificity. Moreover, there are limited data on the use of myocardial deformation imaging for early detection of myocardial dysfunction in children with NCC. We describe left ventricular (LV) deformation patterns in children with NCC, with the goal of identifying a potential diagnostic pattern. We prospectively enrolled 30 children with NCC (47% male; mean age 7.2 years) and 30 age- and gender-matched controls. Extent and severity of non compaction in each segment were evaluated in LV 16-segment model. Regional (base, mid and apex) and segmental (16 segments) longitudinal strain (LS), circumferential strain (CS) and radial strain (RS) were measured using speckle tracking echocardiography. In all patients with NCC, regional and segmental CS and RS at the apex were significantly decreased compared to controls (CS apex: − 19.2 ± 5.4% vs. − 30.2 ± 6.9%, p < 0.001/RS apex: 23.5 ± 8.6% vs. 44.1 ± 14.5%, p < 0.001). Thirty percent (9/30) of patients had an EF < 50%. In these patients, there was additional decrease in CS in basal segments and in LS in basal, mid-cavity and apical segments (CS base: − 16.4 ± 4.7% vs. − 24.6 ± 3.9%, p < 0.001/LS (average all LV segments): − 13.9 ± 3.1% vs. − 20.7 ± 4.7%, p < 0.001). A cut-off value of CS at the apex of − 24.5% was a strong differentiating feature between patients with NCC and EF > 50% and controls (sensitivity: 87%, specificity 79%, AUC 0.88, p < 0.001). Children with NCC exhibit a deformation pattern characterized by decreased apical circumferential strain, which may serve as a potential diagnostic tool for NCC. The role of decreased global LV longitudinal and basal circumferential strain should be further evaluated as a potential prognostic tool.

Keywords

Myocardial deformation Noncompaction Cardiomyopathy Speckle tracking echocardiography 

Abbreviations

NCC

Noncompaction cardiomyopathy

EF

Ejection fraction

LV

Left ventricle

CS

Circumferential strain

LS

Longitudinal strain

RS

Radial strain

Notes

Acknowledgements

The authors thank Dr. Philip C. Ursell from Department of Pathology, University of California, San Francisco who kindly provide photomicrograph of fetus with NCC.

Compliance with ethical standards

Conflict of interest

None of the authors have any conflict of interes that could have influence the manuscript.

Ethical approval

All human studies have been approved by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

Informed consent

Informed consent was obtained from all individual participants included in the study.

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Copyright information

© Springer Science+Business Media B.V., part of Springer Nature 2018

Authors and Affiliations

  1. 1.Division of Cardiology, Department of PediatricsThe Children’s Hospital of PhiladelphiaPhiladelphiaUSA
  2. 2.Department of Pediatrics, Faculty of Medicine and Health ScienceUniversity Putra MalaysiaSerdangMalaysia
  3. 3.Division of Cardiology, Department of PediatricsUniversity of California San FranciscoSan FranciscoUSA

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