MECNUN ÇETİN, Münevver YILDIRIMER, Adnan Taner KURDAL, Şenol COŞKUN

Miyokardiyal “noncompaction”: İki olgu

Miyokardial ‘‘noncompaction’’ bir ya da daha fazla ventrikül segmentinde belirgin trabeküler ağ ve derin intertrabeküler girintilerle karakterize, nadir görülen bir doğumsal kardiyomiyopatidir. Hastalar genellikle kalp yetersizliği, ventriküler aritmiler ve sistemik embolilerin yol açtığı semptom ve bulgularla başvurur. Bu çalışmada birisinde sol ventrikül tutulumu gösteren ve sol ventriküler disfonksiyon gelişen, diğerinde ise biventriküler tutulum gösteren ve sağ ventrikül çıkım yolunda darlık yapan trabeküler yapılar nedeniyle cerrahi girişim gerektirmiş iki olgu sunuldu. Bu olgular başvuru yakınmaları ve tedavi yaklaşımları açısından farklı bir klinik tablo sergilemeleri nedeniyle dikkat çekicidir.

Myocardial noncompaction: Report of two cases

Myocardial noncompaction is a rare type of congenital cardiomyopathy, characterized by trabecular network and deep trabecular recesses located in one or more ventricular segments. Patients generally present with cardiac failure, ventricular arrhythmias, and signs and symptoms related to systemic embolies. We presented two cases of myocardial noncompaction, one with left ventricular involvement and dysfunction, and the other with biventricular involvement and trabecular structures, causing right ventricular outflow tract stenosis which required surgical correction. These cases are remarkable as they had atypical clinical picture including presentation findings and treatment protocols.

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1. Brian C. Weiford, Vijay D. Subbarao, Kevin M. Mulhern. Noncompaction of the ventricular myocardium. Circulation 2004; 109: 2965-2971.
2. Celik S, Görgülü S, Gürol T, Dağdeviren B, Eren M, Tezel T. Miyokardiyal ‘‘noncompaction’’: İki olgu nedeniyle derleme. Anadolu Kardiyol Derg, 2003;3:48-53.
3. 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.
4. Murphy RT, Thaman R, Blanes JG, et al. Natural history and familial characteristics of isolated left ventricular non-compaction. Eur Heart J 2005; 26: 187-192.
5. Maron BJ, Towbin JA, Thiene G, et al. Contemporary definition and classification of the cardiomyopathies. Circulation 2006; 113: 1807-1816.
6. Weiford BC, Subbarao VD, Mulhern KM. Noncompaction of the ventricular myocardium. Circulation 2004; 109: 2965-2971.
7. Karabulut A. Erden İ. Erden E. Çakmak M. Geçici iskemik atak sonrası saptanan süngerimsi miyokart (noncompaction). Arch Turk Soc Cardiol 2009; 37: 205-208.
8. Pauli RM, Scheib-Wixted S, Cripe L, Izumo S, Sekhon GS. Ventricular noncompaction and distal chromosome 5q deletion. Am J Med Genet 1999;85:419-423.
9. Sasse-Klaassen S, Probst S, Gerull B, et al. Novel gene locus for autosomal dominant left ventricular noncompaction maps to chromosome 11p15. Circulation 2004; 109: 2720-2723.
10. Ichida F, Hamamichi Y, Miyawaki T, et al. Clinical features of isolated noncompaction of the ventricular myocardium. J Am Coll Cardiol 1999; 34: 233-40.
11. Menon SC, O’Leary PW, Wright GB, Rios R, MacLellan-Tobert SG, Cabalka AK. Fetal and neonatal presentation of noncompacted ventricular myocardium: expanding the clinical spectrum. J Am Soc Echocardiogr 2007; 20: 1344-1350.
12. Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R. 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.
13. Espinola-Zavaleta N, Soto ME, Castellanos LM, Játiva- Chávez S, Keirns C. Non-compacted cardiomyopathy:clinical-echocardiographic study. Cardiovasc Ultrasound 2006; 4: 35-39.
14. Shimamoto T, Marui A, Yamanaka K,et al. Left ventricular restoration surgery for isolated left ventricular noncompaction: report of the first successful case. J Thorac Cardiovasc Surg 2007; 134: 246-247.
15. Ohki S, Moriyama Y, Mohara J, Kimura C, Sata N, Miyahara K. Aortic valve replacement for aortic regurgitation in a patient with left ventricular noncompaction. Ann Thorac Surg. 2009; 87: 290-292.
16. Alehan D. Clinical features of isolated left ventricular noncompaction in children. Int J Cardiol 2004; 97: 233-237.
17. Ritter M, Oechslin E, Sütsch G, Attenhofer C, Schneider J, Jenni R. Isolated noncompaction of the myocardium in adults. Mayo Clin Proc 1997; 72: 26-31.