Guideline-directed medical therapy failed to improve left ventricular ejection fraction in three patients with noncompaction cardiomyopathy and HFrEF, indicating limited efficacy in this population.
Case Report (n=3)
No
Does guideline-directed medical therapy improve LVEF and clinical outcomes in patients with noncompaction cardiomyopathy and HFrEF?
Patients with noncompaction cardiomyopathy and HFrEF may demonstrate limited responsiveness to standard guideline-directed medical therapy, warranting early consideration of advanced therapies like ICDs or LVADs.
Noncompaction cardiomyopathy (NCCM) is a rare myocardial disorder characterized by excessive trabeculations and thinning of the compacted layer of the left ventricle. It is associated with heart failure, arrhythmias, thromboembolism, and sudden cardiac death. Patients with NCCM-related heart failure with reduced ejection fraction (HFrEF) are typically treated with standard guideline-directed medical therapy (GDMT). Here, we present three cases of patients with NCCM-related HFrEF who were managed with GDMT. Despite optimal treatment, left ventricular ejection fraction (LVEF) demonstrated minimal improvement, with no significant change in clinical outcomes. These cases illustrate the limited and inconsistent response of NCCM-related HFrEF to standard GDMT. Although some patients may experience partial reverse remodeling, the overall benefit of medical therapy remains variable. Patients with NCCM and HFrEF may demonstrate limited responsiveness to standard therapies, warranting close monitoring and early consideration of advanced interventions such as implantable cardioverter-defibrillators (ICDs), left ventricular assist devices (LVADs), or heart transplantation. Tailored therapeutic strategies and further research are needed to improve outcomes.
Panduranga et al. (Fri,) conducted a case report in Noncompaction cardiomyopathy with heart failure with reduced ejection fraction (HFrEF) (n=3). Guideline-directed medical therapy (ARNIs, beta-blockers, MRAs, SGLT2 inhibitors, and diuretics) was evaluated on Improvement in left ventricular ejection fraction (LVEF) and clinical outcomes after guideline-directed medical therapy. Guideline-directed medical therapy failed to improve left ventricular ejection fraction in three patients with noncompaction cardiomyopathy and HFrEF, indicating limited efficacy in this population.