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Heart rate reduction strategy using ivabradine in end-stage Duchenne cardiomyopathy
Rachele Adorisio1, Camilla Calvieri2, Nicoletta Cantarutti1
1Department of Pediatric Cardiology and Cardiac Surgery, Cardiology Unit, Bambino Gesù Hospital & Research Institute, Rome, Italy.
Insights
Ivabradine therapy in Duchenne Muscular Dystrophy (DMD) patients with end-stage dilated cardiomyopathy (DCM) significantly reduced major adverse cardiovascular events (MACEs). This heart rate-reducing strategy improved long-term outcomes in this vulnerable population.
Area of Science:
- Cardiology
- Neuromuscular Disorders
- Pharmacology
Background:
- End-stage dilated cardiomyopathy (DCM) is a critical complication in Duchenne Muscular Dystrophy (DMD), leading to significant morbidity and mortality.
- Limited research exists on the long-term effects of pharmacological interventions, such as ivabradine, in DMD patients with advanced DCM.
Purpose of the Study:
- To investigate the impact of ivabradine on long-term cardiovascular outcomes in patients with end-stage Duchenne Muscular Dystrophy and dilated cardiomyopathy.
- To evaluate the efficacy of a heart rate-reducing (HRR) strategy, including ivabradine, in managing advanced DMD/DCM.
Main Methods:
- A prospective cohort study enrolled 20 male patients with end-stage DMD/DCM (left ventricular ejection fraction <40%) receiving chronic heart failure treatment.
- Patients were divided into groups based on ivabradine therapy, with comprehensive clinical, imaging, and biomarker data collected over one year.
- Kaplan-Meier survival analysis and multivariate Cox regression were used to assess the impact of ivabradine on major adverse cardiovascular events (MACEs).
Main Results:
- Patients treated with ivabradine exhibited a significantly lower incidence of MACEs (12.5%) compared to those not receiving it (87.5%).
- Kaplan-Meier analysis revealed a higher rate of MACE-free survival in the ivabradine group (log rank p=0.017).
- Multivariate Cox regression identified ivabradine therapy as an independent predictor of freedom from MACEs (H.R. 0.078, p=0.039).
Conclusions:
- A heart rate-reducing strategy, particularly with ivabradine, appears effective in reducing acute adverse events in DMD/DCM patients.
- This approach can help achieve optimal heart rate targets and improve left ventricular function in this patient population.
- Ivabradine shows promise as a therapeutic agent for improving long-term cardiovascular outcomes in end-stage DMD/DCM.
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 Gesù Children's Hospital. In each patient, before starting HRR strategy and after 1 year, 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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