Heart rate reduction strategy using ivabradine in end-stage Duchenne cardiomyopathy

Adorisio R, Calvieri C, Cantarutti N, et al. Heart rate reduction strategy using ivabradine in end-stage Duchenne cardiomyopathy. International journal of cardiology 2019.

Nowadays cardiomyopathy is one of the leading causes of dead in DMD. Ivabradine is a compound that has demonstrated effects in other populations suffering from heart failure. It reduces the heart rate. In this study it was tested in patients with end stages of cardiomyopathy. Heart rate reduction by ivabradine showed potential to delay heart failure; however, this was only a short study in which patients were not randomly divided over the groups. So more extensive research should investigate the real potential of heart rate reduction strategy.

Adorisio_2019_Int J Cardiol. Jan 17



BACKGROUND: End-stage dilated cardiomyopathy (DCM) is the leading cause of morbidity and mortality in patients with Duchenne Muscular Dystrophy (DMD). No studies are available on the effect of ivabradine on long-term outcomes in end-stage DMD/DCM.

METHODS: We prospectively enrolled a cohort of end-stage DMD/DCM patients with LV ejection fraction <40%, on chronic HF treatment with an ACE inhibitor referred consecutively from 2012 to 2017 to Bambino Gesu Children’s Hospital. In each patient, before starting HRR strategy and after 1year, we collected medical records comprehensive of clinical, demographic and imaging parameters, BNP levels, neurological and respiratory assessment.

RESULTS: Twenty male patients with DMD/DCM with a mean age of 15.0+/-3.5 (13-19 IQR) years were enrolled and divided into 2 groups according to ivabradine therapy. This group showed a higher incidence of MACEs compared to others in treatment with ivabradine (87.5% vs 12.5%, p=0.025). At Kaplan Meier survival analysis curves, the rate free from MACEs was higher in patients treated with ivabradine (log rank p=0.017). At multivariate Cox regression analysis, ivabradine therapy was an independent predictor of freedom from MACEs (H.R. 0.078, 95% CI 0.007-0.877, p=0.039).

CONCLUSION: HRR strategy, whether achieved by beta blockers alone or in combination with ivabradine, seemed to be effective in reducing the incidence of acute adverse events, reaching optimal target heart rate and improving left ventricular function in DMD/DCM patients.

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