Introduction

Left Ventricular Noncompaction Cardiomyopathy (LVNC) is a rare congenital myocardial disorder characterized by prominent trabeculations and deep intertrabecular recesses resulting from arrest of normal embryonic compaction of the myocardium. It has been increasingly recognized with advancements in imaging techniques such as echocardiography and cardiac MRI. Clinical presentation is highly variable, ranging from asymptomatic incidental findings to heart failure, arrhythmias, thromboembolic events, and sudden cardiac death. The diagnostic challenges stem from its overlap with other cardiomyopathies and the heterogeneity of clinical features [1].

Description

A 42-year-old male presented with progressive exertional dyspnea, palpitations, and occasional presyncope, but without a history of hypertension, coronary artery disease, or prior cardiac illness. Initial ECG revealed nonspecific ST-T changes and episodes of non-sustained ventricular tachycardia. Chest radiography demonstrated cardiomegaly, while transthoracic echocardiography showed reduced left ventricular ejection fraction (35%) with hypertrabeculation and deep intertrabecular recesses in the apical and mid-ventricular segments. These findings raised suspicion of LVNC, which was further confirmed by cardiac MRI using Petersenâ??s criteria, showing a noncompacted-to-compacted myocardium ratio >2.3 during diastole [2]. Differential diagnoses such as dilated cardiomyopathy, hypertrophic cardiomyopathy with apical involvement, and infiltrative myocardial disease were excluded based on imaging and laboratory data. Importantly, ambulatory Holter monitoring revealed frequent ventricular ectopy and short runs of ventricular tachycardia, increasing the patientâ??s risk of arrhythmic events. Family screening was advised, given the potential genetic basis of LVNC [3]. The patient was managed with optimal guideline-directed medical therapy for heart failure, including beta-blockers and ACE inhibitors, along with anticoagulation to prevent thromboembolic complications. Due to the documented ventricular arrhythmias and reduced ejection fraction, an Implantable Cardioverter-Defibrillator (ICD) was placed for primary prevention of sudden cardiac death. The patient showed symptomatic improvement on follow-up, with stable cardiac function and no recurrence of arrhythmic events [4]. Furthermore, the addition of device-based therapy to optimized pharmacological management highlighted the importance of a tailored, multifaceted strategy in advanced heart failure care. Continuous monitoring and regular follow-up enabled early detection of subtle changes in cardiac performance, ensuring therapy remained aligned with evolving clinical needsThe ICD not only provided protection against life-threatening arrhythmias but also contributed to patient confidence and improved quality of life. This case reinforces how the integration of evidence-based medications device therapy [5].

Conclusion

This case highlights the diagnostic and therapeutic challenges of left ventricular noncompaction cardiomyopathy, a rare and often underrecognized condition. Multimodal cardiac imaging, particularly cardiac MRI, is indispensable in establishing the diagnosis and differentiating it from other cardiomyopathies. Early recognition and appropriate management, including guideline-directed medical therapy and device implantation when indicated, are crucial in improving prognosis and preventing life-threatening complications. LVNC exemplifies the critical interplay between clinical suspicion, imaging, and tailored cardiology interventions.

References

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