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Journal of Rare Cardiovascular Diseases
ISSN: 2299-3711 (Print)
e-ISSN: 2300-5505 (Online)
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The 51‐year‐old patient with a non‐compaction cardiomiopathy and multi‐vessel coronary artery disease (RCD code: III-5A.1)
Aleksandra Lenart
,  
Klaudia Knap
,  
Michał Pacia
,  
Lidia Tomkiewicz‑Pająk
,  
Małgorzata Urbańczyk-Zawadzka
,  
Robert Paweł Banyś
,  
Maria Olszowska
,  
Piotr Podolec
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Abstract

Left ventricular non-compaction (LVNC) or “spongy myocardium”, is a rare form of a primary genetic cardiomyopathy considered to be the result of abnormal intrauterine arrest of the myocardial compaction process [1]. Left ventricular noncompaction belongs to the group of unclassified cardiomiopathies by the ESC Position Statement [2]. It is is a rare disorder characterised by a thin, compacted epicardial layer and an extensive non-compacted endocardial layer, with prominent trabeculation and deep recesses that communicate with the left ventricular cavity but not with the coronary circulation [3]. Clinical presentation of patients with LVNC is highly variable – it ranges from completely asymptomatic patients who are accidentally diagnosed (e.g. during familiar screening) to patients in need of heart transplantation [4]. Most commonly patients present with symptoms from the spectrum of the classic triad of LVNC complications: heart failure, arrhythmias and systemic thromboembolic events [5]. We describe a case of accidental discovery of isolated left ventricular non-compaction in a patient with an acute coronary syndrome.




Keywords
spongy myocardium; congenital heart defects; acute coronary syndrome; bradycardia
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Classification of Rare Cardiovascular Diseases anticoagulation atrial fibrillation atrial septal defect cardiomyopathy computed tomography congenital heart disease echocardiography electrocardiogram electrocardiography heart failure implantable cardioverter‑defibrillator magnetic resonance imaging pregnancy pulmonary arterial hypertension pulmonary hypertension rare cardiovascular disease rare disease right heart catheterization right ventricular failure
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