New Diagnosis of Non-compaction Cardiomyopathy in a 43-Year-Old Man Presenting with Syncope

Ahmad Musmar,Muhammad B Hammami,Eli Levine,Saleh Alzaraq,Reem Aboushaar

doi:10.7759/cureus.5107

Ahmad Musmar, Muhammad B Hammami + Show 3 more

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https://doi.org/10.7759/cureus.5107

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Abstract

Non-compaction cardiomyopathy (NCM) is rare congenital cardiomyopathy characterized on cardiac imaging by a two-layered ventricular wall with prominent trabeculations and intertrabecular recesses.This case highlights a patient in his fifth decade who presented from an outpatient setting for abnormal findings found on a transthoracic echocardiogram for syncopal workup. Cardiac MRI was consistent with non-compaction cardiomyopathy. A loop recorder then inserted, and he was placed on guideline-directed therapy for heart failure with reduced ejection fraction (HFrEF) and discharged with life vest since left ventricular ejection fraction (LVEF) > 35%.There are many areas of controversies in NCM, such as prevalence, diagnostic criteria, clinical features, prognosis, and management strategy. We will discuss the etiology, diagnostic criteria, and management.Physicians should be aware of NCM diagnosis when a patient presents with heart failure and structural heart changes on imaging despite the age. Cardiac magnetic resonance imaging (CMRI) is the best diagnostic modality. Patients should be recognized and started on proper management to prevent complications.

Highlights

Non-compaction cardiomyopathy (NCM) is a rare congenital cardiomyopathy which was previously called the spongy myocardium and hypertrabeculation syndrome [1,2]
Non-compaction cardiomyopathy (NCM) is rare congenital cardiomyopathy characterized on cardiac imaging by a two-layered ventricular wall with prominent trabeculations and intertrabecular recesses
A loop recorder inserted, and he was placed on guideline-directed therapy for heart failure with reduced ejection fraction (HFrEF) and discharged with life vest since left ventricular ejection fraction (LVEF) > 35%

Summary

Introduction

Non-compaction cardiomyopathy (NCM) is a rare congenital cardiomyopathy which was previously called the spongy myocardium and hypertrabeculation syndrome [1,2] It is characterized on cardiac imaging by a two-layered ventricular wall with prominent trabeculations and intertrabecular recesses that communicate with the ventricular cavity but not with the coronary circulation. This case highlights a patient in his fifth decade, presenting with syncope. Cardiac MRI showed decreased left ventricular ejection fraction, and global hypokinesis. A loop recorder was inserted, he was placed on guideline-directed therapy for heart failure with reduced ejection fraction (HFrEF) and discharged with life vest since left ventricular ejection fraction (LVEF) > 35%

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