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

Full Text:



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.


spongy myocardium; congenital heart defects; acute coronary syndrome; bradycardia


Freedom RM, Yoo S-J, Perrin D, et al. The morphological spectrum of ventricular noncompaction. Cardiol Young. 2005; 15: 345–364.

Elliott P, Andersson B, Arbustini E, et al. Classification of the cardiomyopathies: a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J 2008; 29: 270–276.

Chin TK, Perloff JK, Williams RG, et al. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation 1990; 82: 507–513.

Trąbka-Zawicki A, Gackowski A, Andres M, et al. Accidentally discovered non-compaction cardiomyopathy in patient with initial diagnosis of acute coronary syndrome. JRCD 2013; 1: 17–22.

Ritter M, Oechslin E, Sutsch G, et al. Isolated noncompaction of the myocardium in adults Mayo Clin Proc 1997; 72: 26 –31.

Ikeda U, Minamisawa M, Koyama J. Isolated left ventricular non

cardiomyopathy in adults. J Cardiol. 2015; 65: 91–97.

Jenni R, Oechslin E, Schneider J, et al. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non towards classification as a distinct cardiomyo

Stöllberger C, Finsterer J, Blazek G. Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol 2002; 90: 899–902.

Petersen SE, Selvanayagam JB, Wiesmann F, et al. Left ventricular nonpaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol 2005; 46: 101–105.

Jacquier A, Thuny F, Jop B, et al. Measurement of trabeculated left ventricular mass using cardiac magnetic resonance imaging in the diagnosis of left ventricular non-compaction. Eur Heart J 2010; 31: 1098–1104.

Brignole M, Auricchio A, Barón Esquivias G, et al. 2013 ESC guidelines on cardiac pacing and cardiac resynchronization therapy. Eur Heart J. 2013; 34: 2281–2329.

Stöllberger C, Finsterer J. Arrhythmias and left ventricular hypertrabeculation /noncompaction. Curr Pharm Des. 2010; 16: 2880–2894.

Oechslin EN, Attenhofer Jost CH, Rojas JR, et al. Long 34 adults with isolated left ventricular noncompaction: a distinct cardiomy

opathy with poor prognosis. J Am Coll Cardiol. 2000; 36: 493–500.

Gao XJ, Kang LM, Zhang J, et al. Incidence of coronary artery disease and outcome of patients with left ventricular noncompaction. Zhonghua Xin Xue Guan Bing Za Zhi. 2011; 39: 725–729.



  • There are currently no refbacks.
Journal of Rare Cardiovascular Diseases (JRCD)
John Paul II Hospital in Kraków, 80 Prądnicka Str., 31-202 Kraków, Poland
Phone: +48 (12) 614 33 99, +48 (12) 614 34 88 Fax: +48 (12) 614 34 88
Published by SoftQ sp. z o.o.
ul. Oleandry 2, 30-063 Kraków, Poland
Phone: +48 (12) 444 1650 Fax: +48 (12) 444 1659