European Heart Journal Advance Access originally published online on October 30, 2007
European Heart Journal 2007 28(23):2886-2894; doi:10.1093/eurheartj/ehm444
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Regional myocardial deformation in children with hypertrophic cardiomyopathy: morphological and clinical correlations
1 Department of Pediatric Cardiology, University Hospitals Leuven, Leuven, Belgium
2 Division of Pediatric Cardiology, Mayo Clinic College of Medicine, Rochester, MN, USA
3 Cardiology Department, University Hospitals Leuven, Leuven, Belgium
4 Lillie Frank Abercrombie Section of Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, 6621 Fannin, Houston, TX 77030, USA
5 Division of Biostatistics and Epidemiology, Texas Heart Institute, Houston, TX, USA
Received 9 March 2007; revised 10 August 2007; accepted 13 September 2007; online publish-ahead-of-print 30 October 2007.
* Corresponding author. Tel: +1 832 826 5659; fax: +1 832 825 5899. E-mail address: cardop{at}bcm.tmc.edu or rhpignat{at}texaschildrenshospital.org
Aims: Hypertrophic cardiomyopathy (HCM) is a disease with marked regional differences in wall thickness. However, the relation between myocardial function and wall thickness has not been well studied. Ultrasonic strain rate (SR) imaging makes it possible to study the regional myocardial deformation. We investigated whether regional systolic deformation is reduced in paediatric patients with HCM and evaluated its relation with wall thickness, electrocardiographic pattern, and exercise capacity.
Methods and results: We studied 41 children with asymmetric HCM (mean age 12.3 years) and 29 controls. Electrocardiograms, exercise testing (when feasible), and echocardiograms with tissue Doppler imaging were performed. Peak systolic SR, strain, post-systolic shortening, and time to maximal strain were calculated in the longitudinal direction from the basal septum, mid-septum, and basal lateral myocardial segments and in the radial direction from the basal antero-septal and infero-lateral myocardial segments. Children with HCM had a significant reduction in deformation in all myocardial segments when compared with controls. In the HCM group, peak systolic SR and strain were significantly lower in the basal septum when compared with the mid-septal and basal lateral myocardial segments. In the basal septum, post-systolic shortening was significantly higher and time to maximal strain significantly longer than in mid-septal and lateral myocardial segments. A strong inverse curvilinear relation between peak systolic strain and wall thickness was found (r = –0.86, P < 0.001), with no further decrease in the regional myocardial function demonstrated once maximal wall thickness exceeded a Z-score of 3.5. Peak systolic strain in the basal part of the septum correlated inversely with exercise capacity (r = 0.68, P < 0.01).
Conclusion: Systolic deformation is significantly and inhomogeneously reduced in children with HCM. This reduction in myocardial function is related to maximal wall thickness and decreased exercise capacity.
Key Words: Hypertrophy cardiomyopathy • Strain rate • Tissue Doppler • Echocardiography • Electrocardiogram
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