AimsDuchenne muscular dystrophy (DMD), an inherited X-linked muscular disease, is associated with dilated cardiomyopathy that is responsible for death in 40% of patients. Our objective was to determine whether inotropic reserve is predictive of LV trend over time. Methods and resultsA total of 69 DMD patients (age 12.2 ± 2.3 years) were investigated. At baseline, LVEF and the presence of inotropic reserve (defined as an increase in LVEF >10% during dobutamine infusion) were investigated using radionuclide ventriculography. During follow-up (FU), LVEF was remeasured after a mean 29 ± 19 months delay. In the whole population, mean LVEF was 58 ± 8% at baseline and declined to 54 ± 11% during FU (P = 0.004). At baseline, 21 patients (30.4%) had LVEF <55% and 38 had no LV inotropic reserve. LVEF declined in the 38 patients (55.1%) without LV inotropic reserve (58 ± 8% to 52 ± 10%, P = 0.001), and not in the other patients (58 ± 8% to 57 ± 11%, P = 0.516) (P = 0.042 for trends in LVEF between groups after adjustment for age, FU duration, and baseline LVEF). Fewer patients with vs. without inotropropic reserve at baseline show a depressed LVEF <55% during follow-up (35.5% vs. 63.2%, respectively, P = 0.030). Similar findings were observed in the subgroups of patients with LVEF >45%

Background Diastolic dysfunction in hypertrophic cardiomyopathy (HCM) is common, but its assessment is difficult using conventional echocardiography. Aims To assess left atrial (LA) function in HCM by longitudinal strain and determine its role in understanding of symptoms. Methods We studied 144 patients divided into 3 age‐ and sex‐matched groups: 48 consecutive patients with HCM, 48 control subjects, and 48 athlete subjects. We assessed LA function by conventional echocardiographic parameters and by longitudinal atrial strain (early‐diastolic left atrial strain during reservoir phase [LASr]; end‐diastolic left atrial strain during conduit phase; end‐systolic peak of the left atrial strain during contraction phase). Results NYHA classification was as follows in HCM group: I in 46%, II in 31%, III in 19%, and IV in 4%. Conventional echocardiographic parameters of diastolic function were depressed in the HCM group as compared to the control and athlete groups, but not related to symptoms. All longitudinal atrial strain parameters were significantly reduced in HCM group as compared to two groups (P < .0001). LASr was significantly correlated to peak VO2 (r = 0.44, P = .01) and was the best parameter for detecting symptomatic patients presenting with HCM, with a cutoff value of 15%: Sensitivity was 71%, specificity was 79%, PPV was 77%, and NPV was 73%. Conclusion Assessment of LA function in HCM is feasible using longitudinal strain, and this technique is more reliable than conventional echocardiographic parameters for the understanding of determinants of symptoms.

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