Increased left ventricular torsion in hypertrophic cardiomyopathy mutation carriers with normal wall thickness

Journal of Cardiovascular Magnetic Resonance

Research

Paul Knaapen¹  Albert C van Rossum¹  Wessel P Brouwer²  Tjeerd Germans²  Iris K Rüssel³  J Tim Marcus⁴  Jolanda van der Velden⁵  Marco JW Götte⁶ 

[1] Department of Cardiology, VU University Medical Center, Amsterdam, The Netherlands;Department of Cardiology, VU University Medical Center, Amsterdam, The Netherlands;Interuniversity Cardiology Institute of the Netherlands (ICIN), Utrecht, The Netherlands;Department of Clinical Physics, Hagaziekenhuis, The Hague, The Netherlands;Department of Physics and Medical Technology, VU University Medical Center, Amsterdam, The Netherlands;Department of Physiology, VU University Medical Center, Amsterdam, The Netherlands;Department of cardiology, Hagaziekenhuis, The Hague, The Netherlands;

关键词: Cardiovascular Magnetic Resonance;    Mutation Carrier;    Late Gadolinium Enhancement;    Left Atrial;    Left Ventricular Volume;   

DOI  :  10.1186/1532-429X-13-3

 received in 2010-09-08, accepted in 2011-01-10,  发布年份 2011

来源: Springer

【 摘 要 】

BackgroundIncreased left ventricular (LV) torsion has been observed in patients with manifest familial hypertrophic cardiomyopathy (HCM), and is thought to be caused by subendocardial dysfunction. We hypothesize that increased LV torsion is already present in healthy mutation carriers with normal wall thickness.MethodsSeventeen carriers with an LV wall thickness <10 mm, and seventeen age and gender matched controls had cardiovascular magnetic resonance (CMR) cine imaging and tissue tagging. LV volumes and mass were calculated from the cine images. LV torsion, torsion rate, endocardial circumferential strain and torsion-to-endocardial-circumferential-shortening (TECS) ratio, which reflects the transmural distribution in contractile function, were determined using tissue tagging.ResultsLV volumes, mass and circumferential strain were comparable between groups, whereas LV ejection fraction, torsion and TECS-ratio were increased in carriers compared to controls (63 ± 3% vs. 60 ± 3%, p = 0.04, 10.1 ± 2.5° vs. 7.7 ± 1.2°, p = 0.001, and 0.52 ± 0.14°/% vs. 0.42 ± 0.10°/%, p = 0.02, respectively).ConclusionsCarriers with normal wall thickness display increased LV torsion and TECS-ratio with respect to controls, which might be due to subendocardial myocardial dysfunction. As similar abnormalities are observed in patients with manifest HCM, the changes in healthy carriers may be target for clinical intervention to delay or prevent the onset of hypertrophy.

【 授权许可】

CC BY   
© Rüssel et al; licensee BioMed Central Ltd. 2011