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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DOI: http://dx.doi.org/10.20418%2Fjrcd.vol2no4.191

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