After cardiopulmonary resuscitation in this pediatric CICU, the rate of success was 63% and the rate of survival was 42%. Prior cardiac surgery and use of epinephrine before arrest did not influence the outcome of CPR. The availability of effective mechanical cardiopulmonary support can improve the outcome of CPR.

BackgroundDuchenne muscular dystrophy (DMD) cardiomyopathy is a progressive disease for which there is no cure. Disease-specific therapies are needed that can be initiated before irreversible myocardial damage ensues. In order to evaluate therapeutic efficacy, surrogate endpoints other than ejection fraction must be found. The hypothesis of this study is that T1 and extracellular volume fraction (ECV) mapping using cardiovascular magnetic resonance (CMR) can detect diffuse extracellular matrix expansion in DMD patients with normal left ventricular ejection fraction (LVEF) and without myocardial late gadolinium enhancement (LGE).MethodsThirty-one DMD and 11 healthy control participants were prospectively enrolled. CMR using a modified Look-Locker (MOLLI) sequence was performed in all participants before and after contrast administration. T1 and ECV maps of the mid left ventricular myocardium were generated and regions of interest were contoured using the standard 6-segment AHA model. Global and segmental values were compared between DMD and controls using a Wilcoxon rank-sum test.ResultsThe DMD participants had significantly higher mean native T1 compared with controls (1045 ms vs 988 ms, p = 0.001). DMD participants with normal LVEF and without evidence of LGE also demonstrated elevated mean native T1 (1039 ms vs 988 ms, p = 0.002, and 1038 ms vs 988 ms, p = 0.011). DMD participants had a significantly greater mean ECV than controls (0.31 vs 0.24, p < 0.001), even in the settings of normal LVEF (0.28 vs 0.24, p < 0.001) and negative LGE (0.29 vs 0.24, p = 0.001).ConclusionsDMD participants have elevated LV myocardial native T1 and ECV, even in the setting of normal LVEF and in the absence of LGE. T1 and ECV mapping in DMD have potential to serve as surrogate cardiomyopathy outcome measures for clinical trials.Electronic supplementary materialThe online version of this article (doi:10.1186/s12968-016-0224-7) contains supplementary material, which is available to authorized users.

Background Patients with Duchenne muscular dystrophy (DMD) require frequent imaging to assess left ventricular (LV) function. Poor imaging windows can limit the diagnostic utility of echocardiography. Cardiac MRI (CMR) is the gold standard for assessment of LV function but has not been universally adopted in DMD patients. The study objectives were: 1) evaluate reproducibility of echocardiographic measures of LV function; 2) evaluate which echocardiographic methods correlate best with CMR LVEF; 3) evaluate whether CMR provides additional value compared with echocardiography. Methods 28 DMD participants prospectively underwent echocardiography and CMR. Two blinded readers measured: fractional shortening (FS) from M-mode and 2-dimensional images and LV ejection fraction (LVEF) using 4-chamber, biplane Simpson, 5/6 area-length, and 3-dimensional methods. Speckle tracking echocardiography was used to analyze circumferential strain. Readers subjectively rated function and segmental wall motion. Agreement was assessed using intraclass correlation coefficient, Bland-Altman plots, Spearman correlation, and weighted Kappa. Results 2-dimensional FS and 5/6 area-length LVEF had the best combination of reproducibility and correlation with CMR LVEF, though both misclassified approximately 20% as either normal or abnormal function. Other measures of LV function were less reproducible with worse correlation to CMR LVEF. 37% of segments not visible on echocardiography were felt to have wall motion abnormalities by CMR. Conclusions 2-dimensional FS and 5/6 area-length LVEF represent the most accurate and reproducible echocardiographic measures of LV function in patients with DMD. CMR should be considered when neither of these techniques are measurable or when it is necessary to detect more subtle cardiovascular changes.

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