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Left bundle branch block in Duchenne muscular dystrophy: Prevalence, genetic relationship and prognosis.

ABSTRACT: Duchenne muscular dystrophy (DMD) is an inherited myogenic disorder due to mutations in the dystrophin gene on chromosome Xp21.1. We designed this study to determine the prevalence of left bundle branch block (LBBB), whether there is a relationship between LBBB and genetic pattern, and to assess predictive factors for acute cardiac events and mortality in adult DMD patients.We reviewed the charts of DMD followed at the Home Mechanical Ventilation Unit of the Raymond Poincare University Hospital.A total of 121 patients, aged from 18 to 41 years have been included in our study. Median vital capacity (VC) was 12% [7; 19.5] of predicted. Almost all patients were on home mechanical ventilation (95%). LBBB was present in 15 patients (13%); among them, 10 disclosed exonic deletions. After a median follow up of 6 years, 21 patients (17%) experienced acute heart failure (AHF), 7 patients (6%) supraventricular arrhythmia, 3 patients (2.4%) ventricular tachycardia, 4 patients (3%) significant electrical disturbances. LBBB was significantly associated with cardiac events (OR = 12.7; 95%CI [3.78-42.7]; p <0.0001) and mortality (OR = 4.4; 95%CI [1.44-13.7]; p 0.009). Presence of residual dystrophin protein was not associated with significant less cardiac events. Age and LVEF were also predictive factors for cardiac events and mortality.LBBB is relatively frequent in DMD and is a major predictive factor for cardiac events and mortality. Presence of residual dystrophin protein was not associated with a lower incidence of cardiac events.

SUBMITTER: Fayssoil A

PROVIDER: S-EPMC5755816 | biostudies-literature | 2018

REPOSITORIES: biostudies-literature

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Left bundle branch block in Duchenne muscular dystrophy: Prevalence, genetic relationship and prognosis.

Fayssoil Abdallah A Ben Yaou Rabah R Ogna Adam A Chaffaut Cendrine C Leturcq France F Nardi Olivier O Wahbi Karim K Duboc Denis D Lofaso Frederic F Prigent Helene H Clair Bernard B Crenn Pascal P Nicolas Guillaume G Laforet Pascal P Behin Anthony A Chevret Sylvie S Orlikowski David D Annane Djillali D

PloS one 20180105 1

<h4>Background</h4>Duchenne muscular dystrophy (DMD) is an inherited myogenic disorder due to mutations in the dystrophin gene on chromosome Xp21.1. We designed this study to determine the prevalence of left bundle branch block (LBBB), whether there is a relationship between LBBB and genetic pattern, and to assess predictive factors for acute cardiac events and mortality in adult DMD patients.<h4>Methods</h4>We reviewed the charts of DMD followed at the Home Mechanical Ventilation Unit of the Ra ...[more]

PMID: 29304097

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Project description:BackgroundComplete bundle branch block in individuals without structural heart disease is known as isolated complete bundle branch block. Isolated complete left bundle branch block (CLBBB) is correlated with ventricular dysfunction secondary to dyssynchrony; however, few studies have investigated isolated complete right bundle branch block (CRBBB), which was previously considered benign but was recently found to be associated with adverse cardiovascular outcomes. This study aimed to evaluate cardiac mechanical synchrony, and systolic and diastolic function in patients with isolated CRBBB and compare cardiac synchrony and function to patients with isolated CLBBB.MethodsThis cross-sectional study was conducted at The First Hospital of China Medical University in Shenyang, China, from 2020 to 2021. A total of 44 isolated CRBBB patients, 44 isolated CLBBB patients, and 42 healthy subjects were enrolled in the study. Transthoracic echocardiography was performed in all subjects. Synchrony parameters, including the mechanical dispersion of the right ventricle [the standard deviation of time to the peak longitudinal strain of six right ventricular (RV) segments] and atrioventricular dyssynchrony parameter [the ratio of left ventricular (LV) diastolic filling time to the time interval between two adjacent R waves (RR interval) measured by tissue Doppler imaging]. RV and LV function were assessed by the global longitudinal strain (GLS) of six RV segments and 18 LV segments, and the ratio of the peak early diastolic flow velocity to annular velocity (E/e') of the tricuspid valve and mitral valve. Statistical analyses were performed, including an analysis of variance, Pearson correlation analysis, and linear regression analysis.ResultsCompared with the healthy subjects, the mechanical dispersion of the right ventricle was significantly increased, and ventricular function was impaired as evidenced by the decreased RV GLS and LV GLS, and the increased E/e' of the tricuspid valve and mitral valve in the isolated CRBBB patients (all P<0.001). Moreover, compared with the isolated CLBBB patients, the mechanical dispersion of the right ventricle and E/e' of the tricuspid valve were increased, and RV GLS was significantly reduced in the isolated CRBBB patients (all P<0.001). Mechanical dispersion of the right ventricle was independently associated with RV GLS [coefficient, 0.13; 95% confidence interval (CI): 0.004-0.26; P=0.04] in the isolated CRBBB patients. RV GLS (coefficient, 0.10; 95% CI: 0.01-0.20; P=0.03) and the ratio of the LV diastolic filling time to the RR interval measured (coefficient, -0.30; 95% CI: -0.53 to -0.07; P=0.01) were independent factors of LV GLS.ConclusionsThe isolated CRBBB patients had impaired cardiac mechanical synchrony and ventricular function, and more decreased RV synchrony and function than the isolated CLBBB patients. Right intraventricular synchrony was independently associated with RV systolic dysfunction in patients with isolated CRBBB. Atrioventricular synchrony and RV systolic function were independently associated with the LV systolic function. Therefore, comprehensive evaluations of echocardiography results and close monitoring is required for isolated CRBBB patients.

Project description:AimTo evaluate ventricular synchronization and function in patients with right bundle-branch block after left bundle-branch-area pacing (LBBAP) by echocardiography.MethodsForty patients who successfully received LBBAP were selected and divided into the right bundle-branch block group (RBBB group) and the non-RBBB group by pre-operation ECG. Echocardiography and follow-up were performed 1 month after operation. Interventricular synchronization was evaluated by tissue Doppler (TDI), tissue mitral annular displacement (TMAD), and interventricular mechanical delay. The tricuspid annular plane systolic excursion (TAPSE), right ventricular fractional area change (RVFAC), tricuspid annulus sidewall systolic velocity (TV-s'), left ventricular global ventricular longitudinal strain (GLS), right ventricular free wall longitudinal strain (LS-RV), standard deviation of left ventricular 18 segments peak time difference (SDt-L) and standard deviation of right ventricular free wall 3 segments peak time difference (SDt-R) were applied to evaluate intraventricular synchronization and ventricular function.ResultsThe difference of displacement peak time of the tricuspid and mitral valves, namely ΔPTTV-MV measured by TMAD, the difference of systolic time to peak of the tricuspid and mitral valves, namely ΔTsTV-MV measured by TDI, were statistically different between the two groups (P < 0.05). Compared with the non-RBBB group, there were no statistically significant differences in the GLS, RVFAC, LS-RV, TAPSE, TV-s', SDt-L, SDt-R (P > 0.05).ConclusionEchocardiography technology including two-dimensional speckle tracking imaging (2D-STI), TDI, and TMAD can effectively analyze interventricular synchronization, intraventricular synchronization, and ventricular function. Although the movement of the right ventricular myocardium in the RBBB group was slightly later than that of the left ventricular myocardium after LBBAP, LBBAP could still be applied in RBBB patients with pacing indication.

Dataset Information

Left bundle branch block in Duchenne muscular dystrophy: Prevalence, genetic relationship and prognosis.

Publications

Left bundle branch block in Duchenne muscular dystrophy: Prevalence, genetic relationship and prognosis.

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