Diagnostic value of myocardial deformation pattern in children with noncompaction cardiomyopathy
- 101 Downloads
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.
KeywordsMyocardial deformation Noncompaction Cardiomyopathy Speckle tracking echocardiography
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.
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 was obtained from all individual participants included in the study.
- 1.Maron BJ, Towbin JA, Thiene G et al (2006) Contemporary definitions and classification of the cardiomyopathies: An American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation 113(14):1807–1816CrossRefPubMedGoogle Scholar
- 8.Cerqueira MD, Weissman NJ, Dilsizian V et al (2002) Standardized myocardial segmentation and nomenclature for tomographic imaging of the heart: a statement for Healthcare Professionals from the Cardiac Imaging Committee of the Council on Clinical Cardiology of the American Heart Association. Circulation 105:539–542CrossRefPubMedGoogle Scholar
- 17.Cheng H, Lu M, Hou C, Chen X et al (2015) Comparison of cardiovascular magnetic resonance characteristics and clinical consequences in children and adolescents with isolated left ventricular non-compaction with and without late gadolinium enhancement. J Cardiovasc Magn Reson 17:44CrossRefPubMedPubMedCentralGoogle Scholar