Refractory Heart Failure in Noncompaction Cardiomyopathy Despite Guideline-Directed Therapy: A Case Series
Abstract
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.
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Evidence Hub reads this as a case series, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
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