Cardiac complications are the leading cause of death in patients with Duchenne muscular dystrophy (DMD). Two-dimensional echocardiography is the current standard for monitoring of LV systolic function in these patients, but it might not detect early systolic dysfunction. The current study examined the use of speckle tracking echocardiography (STE) to detect early signs of cardiac dysfunction in DMD patients. A retrospective review of charts and offline strain analysis of transthoracic echocardiographic studies of DMD patients at our institution from April 2014 to January 2015 were performed and compared to age-matched healthy male subjects. Nineteen DMD patients (age range 12.6 ± 3.1 years) with normal ejection fraction and shortening fraction were compared with sixteen controls. The global circumferential strain was lower in DMD patients compared with controls (-14.7 ± 4.7 vs. -23.1 ± 2.9 %, respectively, p value: 0.001). Circumferential strain measured at basal, mid-ventricular and apical parasternal short-axis views was lower in DMD patients compared with controls. Segmental circumferential strain was lower in DMD patients in most segments compared with controls. The global longitudinal strain was lower in DMD patients compared with controls (-13.6 ± 5 vs. -18.8 ± 3 %, respectively, p value: 0.001). Segmental longitudinal strain measured in various segments was lower in DMD patients compared with controls. DMD patients can have occult cardiovascular dysfunction as shown by reduction in circumferential and longitudinal strain measurements with STE despite normal standard echocardiographic parameters. The clinical significance of early detection of cardiac dysfunction in these patients warrants further studies.

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Objectives Studies have demonstrated an association between congenital heart disease (CHD) and neurodevelopmental delay, partly attributed to the risk of brain injury during cardiac surgery. However, neuroimaging studies have demonstrated a high incidence of preoperative brain abnormalities. The aim of this study was to perform a systematic review in order to quantify the non-surgical risk of brain abnormalities and neurodevelopmental delay in fetuses/newborns with CHD. Methods MEDLINE, EMBASE and The Cochrane Library, without language restrictions were searched electronically, utilising combinations of the terms congenital heart, cardiac, neurologic, neurodevelopment, MRI, ultrasound, neuroimaging, autopsy, preoperative and outcome. Reference lists of relevant articles and reviews were hand searched for additional reports. Cohort and case-control studies were included. Case reports and editorials were excluded. Between-study heterogeneity was assessed using the I2 statistic. Results The search yielded 9,129 citations. Full manuscripts were retrieved for 119, and 30 were included in the review and meta-analysis. 21 studies (n = 953) have reported brain abnormalities in fetuses, newborn or infants with CHD, either preoperatively or in those who did not undergo congenital cardiac surgery. The remaining 9 studies (n = 512) have reported preoperative data on neurodevelopmental assessment in newborn or infants with CHD. The prevalence of brain abnormalities was 36% (95% CI, 26%, 47%; I² = 90.5%) and of the neurodevelopmental delay 42% (95% CI, 34%, 51%; I² = 68.9%), though with heterogeneity between studies. Conclusions In the absence of chromosomal or genetic abnormalities, fetuses with CHD are at increased risk of brain abnormalities and neurodevelopmental delay, which are independent of the surgical risk.