# Refractory Heart Failure in Noncompaction Cardiomyopathy Despite Guideline-Directed Therapy: A Case Series

## TL;DR

This case series shows that patients with noncompaction cardiomyopathy and heart failure often do not respond well to standard treatments, requiring more advanced interventions.

## Contribution

Highlights the limited effectiveness of guideline-directed therapy in NCCM-related heart failure through real-world patient cases.

## Key findings

- Three patients with NCCM-related HFrEF showed minimal improvement in LVEF despite optimal GDMT.
- Medical therapy for NCCM-related HFrEF has variable and inconsistent clinical outcomes.
- Advanced interventions may be necessary for patients with NCCM who do not respond to standard treatments.

## 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.