Önder ÖZTÜRK, ÜNAL ÖZTÜRK, Nizamettin TOPRAK

Biventricular myocardial noncompaction

İzole ventriküler miyokardiyal non-compaction, embriyogenezin erken döneminde görülen aşırı belirgin trabeküler yapı ve trabeküller arası derin girintiler ile karakterizedir. İzole ventriküler myokardiyal non compactionın klinik bulguları; bozulmuş sol ventrikül sistolik fonksiyonu, ventriküler aritmi ve sistemik embolidir. Ekokardiyografik özellikleri, birlikte başka bir kardiyak anomali olmaksızın, derin endomiyokardiyal boşluklar ile birlikte trabeküler endokard süngerimsidir. Trabekülasyon belirgin olarak midlateral, apikal ve mid-inferiyor segmentlerde lokalizedir. Trabeküller arası girintilerde renkli doppler ile kan akımı saptanır. Bu çalışmada, 17 yaşında çarpıntı ve bayılma şikayeti ile gelen erkek hastada, sol ve sağ ventriküler miyokardiyal non-compaction olgusu sunulmuştur.

Anahtar Kelimeler:

Ekokardiyografi, Senkop, Miyokard

Her iki ventrikülü tutan miyokardiyal non-compaction

The isolated noncompacted ventricular myocardium (NCVM), characterized by excessively prominent trabecular meshwork and deep intertrabecular recesses, is seen in the early period of embryogenesis. Clinical manifestations of NCVM are symptoms associated with depressed left ventricular systolic function, with ventricular arrhythmias, and with systemic embolization. Characteristics on echocardiography have been defined as absent coexisting cardiac abnormalities, non-compacted trabecular endocardium with deep endomyocardial spaces, predominant localisation of the trabeculation to mid-lateral, apical and mid-inferior segments, and a colour Doppler evidence of deep perfused intertrabecular recesses. We describe a case of isolated noncompaction of the left and right ventricular myocardium in a 17-year-old man who presented initially with palpitation and syncope.

Keywords:

Echocardiography, Syncope, Myocardium,

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1. Laat LE, Galema TW, Krenning BJ, Roelandt JR. Diagnosis of non-compaction cardiomyopathy with contrast echocardiography. Int J Card 2004; 94: 127-128. 2. Dusek J, Ostadal B, Duskova M. Postnatal persistence of spongy myocardium with embryonic blood supply. Arch Pathol 1975; 99: 312–317. 3. Ichida F, Hamamichi 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. 4. Shah CP, Nagi KS, Thakur RK, Boughner DR, Xie B. Spongy Left Ventricular Myocardium in an Adult. Tex Heart Inst J 1998; 25: 150-151. 5. Hamamichi Y, Ichida F, Hashimoto I, et al. Isolated noncompaction of the ventricular myocardium: ultrafast computed tomography and magnetic resonance imaging. Int J card Imaging 2001; 17: 305–314. 6. Jenni R, Oechslin E, Schneider J, Attenhofer Jost C, Kaufmann PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular noncompaction: A step towards classification as a distinct cardiomyopathy. Heart 2001; 86:666–671.