Refractory Heart Failure in Noncompaction Cardiomyopathy Despite Guideline-Directed Therapy: A Case Series
Varshitha Tumkur Panduranga1, Asher Gorantla1, Nana Osei1
1Internal Medicine, State University of New York Downstate Health Sciences University, Brooklyn, USA.
Insights
Noncompaction cardiomyopathy (NCCM) with heart failure with reduced ejection fraction (HFrEF) shows limited response to guideline-directed medical therapy (GDMT). Advanced interventions may be necessary for improved outcomes in these rare cases.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Noncompaction cardiomyopathy (NCCM) is a rare congenital heart muscle disease.
- It is characterized by excessive left ventricular trabeculations and deep intertrabecular recesses.
- NCCM is associated with significant morbidity and mortality, including heart failure, arrhythmias, and thromboembolism.
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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