Abstract

Left ventricular non-compaction (LVNC) is a rare congenital cardiomyopathy caused by arrest of normal endomyocardial embryogenesis and characterized by the persistence of ventricular hypertrabeculation, isolated or associated to other congenital defects. A 33-year-old male, with family history of sudden cardiac death (SCD), presented to our ER with typical chest pain and was diagnosed with anterior STEMI. Coronary angiography showed an anomalous origin of the circumflex artery from the right coronary artery and a critical stenosis on the proximal left anterior descending artery, treated with primary percutaneous coronary intervention. The echocardiogram documented left ventricular severe dysfunction with lateral wall hypertrabeculation, strongly suggestive for non-compaction, confirmed by cardiac MRI. At 3 months follow up, for the persistence of the severely depressed EF (30%) and the family history for SCD, the patient underwent subcutaneous ICD (sICD) implantation for primary prevention. To the best of our knowledge, this is the first case of LVNC associated with anomalous coronary artery origin and STEMI reported in the literature. Arrhythmias are common in LVNC due to endocardial hypoperfusion and fibrosis. sICD overcomes the risks of transvenous ICD, and it is a valuable option when there is no need for pacing therapy for bradycardia, cardiac resynchronization therapy and anti-tachycardia pacing.

Highlights

  • Left ventricular non-compaction (LVNC) is a rare congenital cardiomyopathy thought to be caused by arrest of normal endomyocardial embryogenesis, leading to the persistence of numerous and deep trabeculations communicating with the ventricular cavity

  • To the best of our knowledge, this is the first case of LVNC associated with anomalous coronary artery origin and ST elevation myocardial infarction (STEMI) reported in the literature

  • Further studies are necessary in order to assess the possible pathophysiological mechanisms that correlate LVNC and coronary atherosclerosis

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Summary

A Rare Case of Left Ventricular Non-Compaction with Coronary

Francesca Romana Prandi 1, * , Federica Illuminato 1 , Chiara Galluccio 1 , Marialucia Milite 1 , Massimiliano Macrini 1 , Alessio Di Landro 1 , Gaetano Idone 1 , Marcello Chiocchi 2 , Francesco Paolo Sbordone 2 , Domenico Sergi 1 , Francesco Romeo 1,3 and Francesco Barillà 1. Artery Anomaly Complicated by ST-Elevation Myocardial Infarction and Subcutaneous Defibrillator.

Introduction
Case Report
Coronary
Cardiac
LVNC and Coronary Artery Anomalies
LVNC and Acute Coronary Syndrome
LVNC and Arrhythmic Risk
Findings
LVNC Diagnostic and Prognostic Challenges
Conclusions
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