Cardiovascular magnetic resonance of mitral valve length in hypertrophic cardiomyopathy

Journal of Cardiovascular Magnetic Resonance

Research

Johanna Kuusisto¹  Kirsi Lauerma²  Mika Tarkiainen³  Petri Sipola⁴  Vesa Järvinen⁵  Mikko Jalanko⁶  Mika Laine⁶  Tiina Heliö⁶  Kaisu Häyrinen⁷ 

[1] Centre for Medicine and Clinical Research, University of Eastern Finland and Kuopio University Hospital, Kuopio, Finland;Department of Radiology, Helsinki University Central Hospital, Helsinki, Finland;Department of Radiology, Kuopio University Hospital, Kuopio, Finland;Department of Radiology, Kuopio University Hospital, Kuopio, Finland;University of Eastern Finland, Kuopio, Finland;HUS Medical Imaging Center, Clinical Physiology and Nuclear Medicine, Hyvinkää Hospital, Hyvinkää, Finland;Heart and Lung Center, Department of Cardiology, Helsinki University Central Hospital, Helsinki, Finland;University of Eastern Finland, Kuopio, Finland;

关键词: Cardiomyopathy;    Hypertrophic;    Mitral valve;    Cardiovascular magnetic resonance;   

DOI  :  10.1186/s12968-016-0250-5

 received in 2016-02-26, accepted in 2016-05-17,  发布年份 2016

来源: Springer

【 摘 要 】

BackgroundPrevious data suggest that mitral valve leaflets are elongated in hypertrophic cardiomyopathy (HCM), and mitral valve leaflet elongation may constitute a primary phenotypic expression of HCM. Our objective was to measure the length of mitral valve leaflets by cardiovascular magnetic resonance (CMR) in subjects with HCM caused by a Finnish founder mutation in the myosin-binding protein C gene (MYBPC3-Q1061X), carriers of the same mutation without left ventricular hypertrophy, as well as in unselected consecutive patients with HCM, and respective controls.MethodsAnterior mitral valve leaflet (AML) and posterior mitral valve leaflet (PML) lengths were measured by CMR in 47 subjects with the Q1061X mutation in the gene encoding MYBPC3 and in 20 healthy relatives without the mutation. In addition, mitral valve leaflet lengths were measured by CMR in 80 consecutive non-genotyped patients with HCM in CMR and 71 age- and gender-matched healthy subjects.ResultsOf the subjects with the MYBPC-Q1016X mutation, 32 had left ventricular hypertrophy (LVH, LV maximal wall thickness ≥ 13 mm in CMR) and 15 had no hypertrophy. PML was longer in patients with the MYBPC3-Q1061X mutation and LVH than in controls of the MYBPC group (12.8 ± 2.8 vs 10.6 ± 1.9 mm, P = 0.013), but the difference between the groups was not statistically significant when PML was indexed for BSA (P = 0.066), or when PML length was adjusted for BSA, age, gender, LV mass and ejection fraction (P = 0.195). There was no significant difference in the PML length in mutation carriers without LVH and controls (11.1 ± 3.4 vs 10.6 ± 1.9, P = 0.52). We found no difference in AML lengths between the MYBPC mutation carriers with or without hypertrophy and controls. In 80 consecutive non-genotyped patients with HCM, there was no difference either in AML or PML lengths in subjects with HCM compared to respective control subjects.ConclusionsIn subjects with HCM caused by the Q1061X mutation in the MYBPC3 gene, the posterior mitral valve leaflets may be elongated, but mitral valve elongation does not constitute primary phenotypic expression of the disease. Instead, elongated mitral valve leaflets seem to be associated with body size and left ventricular remodeling.

【 授权许可】

CC BY   
© The Author(s). 2016