Accidentally discovered non‑compaction cardiomyopathy in patient with initial diagnosis of acute coronary syndrome (RCD code: III-5A)

Aleksander Trąbka‑Zawicki, Andrzej Gackowski, Marek Andres, Aleksander Zeliaś, Magdalena Kostkiewicz, Igor Tomala, Jacek Lelakowski, Krzysztof Żmudka

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Abstract


Isolated non‑compaction cardiomyopathy is a rare congenital developmental disorder, which belongs to the group of unclassified cardiomyopathies by the ESC Position Statement. Typical echocardiographic findings include multiple trabeculations of the left ventricular muscle, separated by deep lacunae which communicate with left ventricular lumen. We present a case of accidentally discovered non‑compaction cardiomyopathy in a 46‑year‑old male, who was initially diagnosed with an acute coronary syndrome. The diagnosis of the left ventricular non‑compaction was confirmed by transthoracic echocardiography and cardiac magnetic resonance. Occupational contribution (lead and zinc exposure and repeated electric shocks) to the onset of the heart failure was additionally evaluated. JRCD 2013; 1 (3): 17–22


Keywords


cardiomyopathy non‑compaction; congenital heart defects; heart failure

References


Podolec P., Tracz W., Hoffman P., et al. Echokardiografia praktyczna, tom IV. Medycyna Praktyczna 2006, 459–464.

Oechslin EN, Attenhofer Jost CH, Rojas JR, et al. Long‑term follow‑up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol 2000;36:493–500.

Erwin Oechslin, Rolf Jenni. Left ventricular non‑compaction revisited: a distinct phenotype with genetic heterogeneity? European Heart Journal ;32:1446–1456.

Pignatelli RH, McMahon CJ, Dreyer WJ, et al. Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy. Circulation 2003; 108(21): 2672–8.

Lilje C, Razek V, Joyce JJ, et al. Complications of noncompaction of the left ventricular myocardium in a paediatric population: a prospective study. EurHeart J 2006;27:1855–1860.

Jenni R, Rojas J, Oechslin E. Isolated noncompaction of the myocardium. N Engl J Med 1999;340:966–7.

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

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

Ichida F, Hanamichi Y, Miyawaki T, et al. Clinical features of isolated noncompaction of the ventricular myocardium: long‑term clinical course, hemodynamic properties, and genetic background. J Am Coll Cardiol. 1999;34: 233–240.

Nugent AW, Daubeney PE, Chondros P, et al. The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med 2003;348(17):1639–46.

Ichida F, Tsubata S, Bowles KR, et al. Novel gene mutations in patients with left ventricular noncompaction or Barth syndrome, Circulation ;103:1256–1263.

Hermida‑Prieto M, Monserrat L, Castro‑Beiras A, et al. Familial dilated cardiomyopathy and isolated left ventricular noncompaction associated with lamin A/C gene mutations, Am. J. Cardiol. 2004;94:50–54.

Shan L, Makita N, Xing Y, et al. SCN5A variants in Japanese patients with left ventricular noncompaction and arrhythmia, Mol. Genet. Metab. ;93:468–474.

Kenton AB, Sanchez X, Coveler KJ, et al. Isolated left ventricular noncompaction is rarely caused by mutations in G4.5, alpha‑dystrobrevin and FK Binding Protein‑12. Mol Genet Metab 2004;82(2):162–6.

Alper G, NarayananV. Friedreich’s ataxia. Pediatr Neurol 2003;28:335–41.

Junga G., Kneifel S., von Smekal A. et al. Myocardial ischemia in children with isolated ventricular non‑co mpaction. Eur Heart J 1999;20:910–916.

Jenni R., Oechslin E., Schneider J. et al. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non‑compaction: a step towards classification as a distinct cardiomiopathy. Heart 2001;86:666–671.

Stöllberger C, Finsterer J. Left ventricular hypertrabeculation/noncompaction. J Am Soc Echocardiogr 2004;17:91-100

Petersen SE, Selvanayagam JB, Wiesmann F, et al. Left ventricular non‑compaction: 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.

Williams SG, Barker D, Goldspink DF, et al. A reappraisal of concepts in heart failure: Central role of cardiac power reserve. Arch Med Sci 2005;1(2): 65–74.

Boyd MT, Seward JB, Tajik AJ, et al. Frequency and location of prominent left ventricular trabeculations at autopsy in 474 normal human hearts: implications for evaluation of mural thrombi by two‑dimensional echocardiography. J Am Coll Cardiol, 1987;9:323–326.

Stöllberger C, Winkler‑Dworak M, Blazek G, et al. Prognosis of left ventricular hypertrabeculation/noncompaction is dependent on cardiac and neuromuscular comorbidity. Int J Cardiol, 2007;121:189–193.

Lofiego C, Biagini E, Pasquale F, et al. Wide spectrum of presentation and variable outcomes of isolated left ventricular non‑compaction. Heart ;93:65–71.

Ichida F. Left Ventricular Noncompaction Circ J 2009;73:19–26.

Sato Y, Matsumoto N, Takahashi H, et al. Cardioverter defibrillator implantation in an adult with isolated noncompaction of the ventricular myocardium. Int J Cardiol 2006;110:417–9.

Kobza R, Steffel J, Erne P, et al. Implantable cardioverter–defibrillator and cardiac resy‑nchronization therapy in patients with left ventricular noncompaction. Heart Rhythm 2010;7:1545–9.

Lorsheyd A, Cramer MJ,. Velthuis BK, et al. Familial occurrence of isolated non‑compaction cardiomyopathy. Eur J Heart Fail 2006;8:826–831.

Rosa LV, Salemi VM, Alexandre LM, et al. Noncompaction cardiomyopathy: a current view. Arq Bras Cardiol. 2011;97(1):e13‑9.

Stanton C, Bruce C, Connolly H, et al. Isolated left ventricular noncompaction syndrome. Am J Cardiol 2009;104:1135–1138.




DOI: http://dx.doi.org/10.20418%2Fjrcd.vol1no3.51

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