18 research outputs found
Cumulative incidence of the studied outcomes.
<p>Cumulative incidence of the studied outcomes.</p
Factors associated with respiratory events.
<p>Factors associated with respiratory events.</p
Evolution of respiratory vital capacity (VC) and left ventricular ejection fraction (LVEF) over the follow-up time.
<p>Follow up time was 65.6 [IQR 46.28; 80.68] months between VC1 and VC2 and 80.8 months [IQR 53.1; 89] months between LVEF1 and LVEF2. VC: pulmonary vital capacity. LVEF: left ventricular ejection fraction.</p
Left bundle branch block in Duchenne muscular dystrophy: Prevalence, genetic relationship and prognosis
<div><p>Background</p><p>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.</p><p>Methods</p><p>We reviewed the charts of DMD followed at the Home Mechanical Ventilation Unit of the Raymond Poincare University Hospital.</p><p>Results</p><p>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.</p><p>Conclusion</p><p>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.</p></div
Bland-Altman plots for LVEF measurement (intra-observer variability analysis).
<p>LVEF: left ventricular ejection fraction.</p
Predictive factors for cardiac events using multivariable logistic regression.
<p>Predictive factors for cardiac events using multivariable logistic regression.</p