Journal of the Korean Society of Radiology (대한영상의학회지)

The Korean Society of Radiology (대한영상의학회)
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Cardiac MRI and Transthoracic Echocardiography of Left Ventricular Myocardial Noncompaction in A Patient with Congestive Heart Failure: A Case Report

심부전 환자에서 발견된 좌심실 비치밀심근의 심장 자기공명영상과 경흉부 심초음파: 증례 보고

  • Cho, Eui-Min (Department of Radiology, Chosun University Hospital) ;
  • Byun, Joo-Nam (Department of Radiology, Chosun University Hospital) ;
  • Kim, Dong-Hun (Department of Radiology, Soonchunhyang University Hospital Bucheon)
  • 조의민 (조선대학교병원 영상의학과) ;
  • 변주남 (조선대학교병원 영상의학과) ;
  • 김동훈 (순천향대학교 부천병원 영상의학과)
  • Received : 2010.05.09
  • Accepted : 2010.07.26
  • Published : 2010.11.01
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Abstract

We report a case of a 38-year-old male presenting with new-onset dyspnea, that was diagnosed as left ventricular noncompaction by transthoracic echocardiographic and cardiac MR. The tests revealed left ventricular systolic dysfunction with prominent trabeculations associated with deep intertrabecular recesses and an enddiastolic noncompacted to compacted ratio of 2.5 in the whole apical wall and mid-ventricular anterolateral and inferolateral walls. Delayed gadolinium contrast-enhanced MRI revealed subepicardial mid-wall hyperenhancement of the midventricular anteroseptal and inferoseptal walls, which suggested myocardial fibrosis. We review the pathophysiology, clinical characteristics, and diagnostic approach of the left ventricular noncompaction associated with congestive heart failure.

호흡곤란을 주소로 내원한 38세 남자의 자기공명영상과 경흉부 심초음파를 이용하여 진단한 비치밀심근 증례를 보고하고자 한다. 자기공명영상과 경흉부 심초음파에 심실 첨부 전체벽, 심실 중간부 전벽과 하벽에 과도하게 돌출된 잔기둥과 좌심실 벽에 깊은 함요를 보였고, 이완기에 측정한 비치밀근과 치밀근의 비율(noncompaction/compaction ratio)이 2.5로 증가하였으며 좌심실 수축기 장애가 관찰되었다. 또한, 가돌리늄 조영제 주입 후 15분 지연 영상에서 심실 중간부 중격 전벽과 중격하벽에 경계가 불분명한 비정상적인 지연조영증강이 관찰되었다. 저자들은 심부전이 동반된 비치밀심근의 병리기전, 임상양상, 진단적 접근법에 대해 고찰하였다.

Keywords

References

  1. Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation 1990;82:507-513 https://doi.org/10.1161/01.CIR.82.2.507
  2. Varnava AM. Isolated left ventricular non-compaction: a distinct cardiomyopathy? Heart 2001;86:599-600 https://doi.org/10.1136/heart.86.6.599
  3. Jenni R, Oechslin E, Schneider J, Attenhofer Jost C, Kaufmann PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart 2001;86:666-671 https://doi.org/10.1136/heart.86.6.666
  4. 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 https://doi.org/10.1016/S0735-1097(00)00755-5
  5. Pignatelli RH, McMahon CJ, Dreyer WJ, Denfield SW, Price J, Belmont JW, et al. Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy. Circulation 2003;108:2672-2678 https://doi.org/10.1161/01.CIR.0000100664.10777.B8
  6. Petersen SE, Selvanayagam JB, Wiesmann F, Robson MD, Francis JM, Anderson RH, et al. Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol 2005;46:101-105 https://doi.org/10.1016/j.jacc.2005.03.045
  7. Alhabshan F, Smallhorn JF, Golding F, Musewe N, Freedom RM, Yoo SJ. Extent of myocardial non-compaction: comparison between MRI and echocardiographic evaluation. Pediatr Radiol 2005;35:1147-1151 https://doi.org/10.1007/s00247-005-1551-2
  8. Richardson P, McKenna W, Bristow M, Maisch B, Mautner B, O'Connell J, et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies. Circulation 1996;93:841-842 https://doi.org/10.1161/01.CIR.93.5.841
  9. Aras D, Tufekcioglu O, Ergun K, Ozeke O, Yildiz A, Topaloglu S, et al. Clinical features of isolated ventricular noncompaction in adults long-term clinical course, echocardiographic properties, and predictors of left ventricular failure. J Card Fail 2006;12:726-733 https://doi.org/10.1016/j.cardfail.2006.08.002
  10. Espinola-Zavaleta N, Soto ME, Castellanos LM, Jativa-Chavez S, Keirns C. Non-compacted cardiomyopathy: clinical-echocardiographic study. Cardiovasc Ultrasound 2006;4:35 https://doi.org/10.1186/1476-7120-4-35