scholarly journals P4505Anterior mitral valve leaflet in sarcomere gene mutation carriers without left ventricular hypertrophy and healthy controls

2017 ◽  
Vol 38 (suppl_1) ◽  
Author(s):  
H.G. Van Velzen ◽  
A.F.L. Schinkel ◽  
M.E. Menting ◽  
A.E. Van Den Bosch ◽  
M. Michels
2021 ◽  
Author(s):  
Li Yu ◽  
Qichang Zhou ◽  
Xiangdang Long ◽  
Qinghai Peng ◽  
Zurong Yang

Abstract Background: To investigate whether familial hypertrophic cardiomyopathy (HCM) gene mutation carriers without overt left ventricular hypertrophy have subclinical changes in left ventricular function.Methods: We studied Eighteen HCM families with pathogenic mutations, 45 patients with overt HCM (gene positive/phenotype positive (G+/P+)), 40 patients without myocardial hypertrophy (gene positive/phenotype negative G+/P-)), and 48 genotype-negative related healthy controls. Conventional echocardiography and velocity vector imaging (VVI) were performed, and blood levels of N- terminal pro- brain natriuretic peptide (NT- pro- BNP) were analyzed.Results: Although the global longitudinal, circumferential and radial strain was similar between the G+/P- group and the control group, the longitudinal strain of basal inferoseptum and basal anteroseptum was lower in G+/P- patients than in controls, while the basal and middle inferolateral longitudinal strains were significantly higher. Compared with the controls, G+/P+ patients had significantly lower global and segmental longitudinal and radial strains. There were no significant differences between the normal control and G+/P+ groups for global and segmental circumferential strains. The middle of the left ventricle (LV) was clockwise in G+/P+ patients (opposite to normal).The rotation angle of the mid LV rotation in the G+/P+ group were significantly higher than those in the G+/P- subjects and controls. The NT-proBNP levels were higher in G+/P+ patients than in G+/P- people and controls.Conclusions: Sarcomere gene mutation carriers without overt left ventricular hypertrophy have subclinical segmental systolic dysfunction. Velocity vector imaging is feasible for differentiating HCM, G+/P- patients from controls.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
S Okutucu ◽  
S.G Fatihoglu ◽  
N Bursa ◽  
H Aksoy ◽  
B Yetis Sayin ◽  
...  

Abstract Background Mitral annulus disjunction (MAD) is an abnormal atrial displacement of the mitral valve leaflet hinge point. MAD has been associated with mitral valve prolapse (MVP) and arrhythmic events. T-wave peak to T-wave end interval (Tp-e) and Tp-e/QTc are electrocardiographic (ECG) indices to predict ventricular tachyarrhythmia and cardiovascular mortality. Purpose We aimed to evaluate ventricular repolarization dispersion by using the Tp-e interval and Tp-e/QT ratio in patients with MAD and healthy controls. Methods A total of 35 patients with MAD (age 40.4±8.2 years; 66% female) and 37 healthy controls (age 37.6±9.5 years; 68% female) were enrolled. All subjects were evaluated by 12 lead standard ECG, 24-hour ambulatory ECG, and transthoracic echocardiography. The MAD distance was measured from the left atrial wall-mitral valve leaflet junction to the top of the left ventricular wall during end-systole in the parasternal long-axis view. The standard 12-lead electrocardiograms were analyzed; QTc, Tp-e and Tp-e/QTc were calculated. The Tp-e interval was defined as the interval from the peak of the T wave to the end of the T wave from precordial leads. Finally, the Tp-e/QT ratio was calculated from these measurements. The normality assumption was checked with the Shapiro-Wilk test and the Mann–Whitney U test was used for inter-group comparisons. Results Tp-e interval (61.9±6.0 ms vs. 77.1±5.9 ms, p<0.001) and Tp-e/QT ratio (0.14±0.01 vs. 0.17±0.02, p<0.001) were significantly prolonged in patients with MAD than in the control group. MVP was present in 24 (69%) patients with MAD. The prevalence of 30 premature ventricular contractions / hour were higher in MAD subgroup without MVP than those MAD with MVP (54.5% vs 16.7%, p=0.041). Mean MAD distance measured by echocardiography was 5.7±1.5 mm. The Spearman's rank-order correlation analyses revealed positive correlations of MAD distance with Tp-e interval (r=0.620, p=0.001) and Tp-e/QT ratio (r=0.372, p=0.028). Conclusions The patients with MAD had a prolonged Tp-e interval and Tp-e/QT ratio compared with normal subjects. Furthermore, this prolongation was well correlated with MAD distance. Patients with MAD, particularly with higher MAD distance, should be followed closely for arrhythmic outcomes. MAD distance and Tp-e interval Funding Acknowledgement Type of funding source: None


2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
TP Craven ◽  
PG Chew ◽  
M Gorecka ◽  
LAE Brown ◽  
A Das ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background Percutaneous mitral valve leaflet repair can be an effective treatment for primary mitral regurgitation (MR) patients deemed high-risk for surgery. Accurate assessment of cardiac reverse remodelling is essential to optimise future patient selection. Cardiovascular magnetic resonance (CMR) is the reference standard for cardiac volumetric assessment and compared to transthoracic echocardiography (TTE) provides superior reproducibility in MR quantification. Prior CMR studies have analysed cardiac reverse remodelling following percutaneous intervention in combined cohorts of primary and secondary MR patients. However, as aetiology of MR can significantly impact outcomes, focused studies are warranted. Purpose Assess cardiac reverse remodelling and quantify changes in MR following percutaneous mitral valve leaflet repair for primary MR using the reference standard (CMR). Methods 12 patients with at least moderate-severe MR on TTE were prospectively recruited to undergo CMR imaging and 6-minute walk tests (6MWT) at baseline and 6 months following percutaneous mitral valve leaflet repair (MitraClip). CMR protocol involved: left-ventricular (LV) short axis cines (bSSFP, SENSE-2, 10mm, no gap), transaxial right-ventricular (RV) cines (bSSFP, SENSE-2, 8mm, no gap), two and four chamber cines and aortic through-plane phase contrast imaging, planned at the sino-tubular junction. MR was quantified indirectly using LV and aortic stroke volumes. Results 12 patients underwent percutaneous mitral valve leaflet repair (MitraClip) for posterior mitral valve leaflet prolapse, however 1 patient declined follow up after single-leaflet clip detachment resulting in 11 patients (age 83 ± 5years, 9 male) completing follow up imaging. At 6-months: significant improvements occurred in New York Heart Association functional class (Table 1) and 6MWT distances (223 ± 71m to 281 ± 65m, p = 0.005) and significant reductions occurred in indexed left ventricular end-diastolic volumes (LVEDVi) (118 ± 21ml/m2 to 94 ± 27ml/m2, p = 0.001), indexed left ventricular end-systolic volumes (58 ± 19ml/m2 to 48 ± 21ml/m2, p = 0.007) and quantitated MR volume (55 ± 22ml to 24 ± 12ml, p = 0.003) and MR fraction (49 ± 9.4% to 29 ± 14%, p= <0.001). There were no statistically significant changes in left ventricular ejection fraction (LVEF), right ventricular dimensions/ejection fraction or bi-atrial dimensions (Table 1). All patients demonstrated decreased LVEDVi and quantified MR (Figure 1). Conclusion Successful percutaneous mitral valve leaflet repair for primary MR results in reduction in MR, positive LV reverse remodelling, preservation of LVEF, and functional improvements. Larger CMR studies are now required to further guide optimal patient selection.


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