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心肌致密化不全的临床病理分析 被引量:5

Clinicopathologic analyses of non-compaction of ventricular myocardium
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摘要 目的探讨心肌致密化不全的临床病理特征。方法收集5例心肌致密化不全患者的临床症状、体征、心电图及超声心动图情况,测量心脏的大体情况,包括心脏的重量、纵长、横宽及各瓣膜环的长度、心室流人道和流出道的长度、心室壁的厚度。常规制片,磷钨酸苏木精染色、弹力纤维染色,光镜观察心肌情况及冠状动脉的改变。结果5例患者均为男性,29—57岁,临床均有胸闷症状,心电图显示心律失常,超声心动图检查3例诊断为心肌致密化不全,病理分析可见受累心腔内显示有异常粗大的肌小梁和交错深陷的隐窝,从心底到心尖致密心肌逐渐变薄;心内膜为增厚的纤维组织,内层非致密心肌肌束粗大紊乱、细胞核畸形,外层致密心肌肌束走行及形态基本正常,细胞核大小均匀。结论心肌致密化不全具有特有的病理组织学变化,临床无特异性表现,超声心动图是临床诊断心肌致密化不全的主要辅助手段之一。 Objective To investigate the clinicopathologic manifestations of non-compaction of ventricular myocardium(NVM). Methods Clinical data, electrocardiograms, echocardiography images and pathologic changes were studied in five cases of non-compaction of ventricular myocardium. Results The patient' s ages ranged from 29 to 57 years old, all were males. Abnormal electrocardiograms were obtained in all of the 5 cases. Among them, 3 were diagnosed using echocardiography. Histopathologic examination showed that there were abnormally coarse muscle trabeculation and deep recesses, interlacing in arrangement, over the inner wall of the heart chambers. The compacted myocardium became thinning down gradually from the base to the apex of the heart. The non-compacted myocardium bundles locating close to the endocardium were coarse and orderless in arrangement, nuclei were irregular and abnormal, nevertheless, the arrangement and appearance of the muscle bundles near by the pericardium part were essentially normal and the cell nuclei were evenly distributed. Conclusion There are no specific clinical manifestations obtained in patients with non-compaction of ventricular myocardium, however, the pathologic changes are characteristic and a clinical diagnosis can be made by using echocardiography.
出处 《中华病理学杂志》 CAS CSCD 北大核心 2009年第5期333-336,共4页 Chinese Journal of Pathology
关键词 心肌疾病 病理学 临床 Cardiomyopathies Pathology, clinical
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参考文献10

  • 1朱清於 金崇厚.先天性心脏病病理解剖学[M].北京:人民军医出版社,2001.279-284.
  • 2Maron BJ, Towbin JA, Thiene G, et al. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation, 2006, 113(14):1807-1816.
  • 3Ritter M, Oechslin E, Stitsch G, et al. Isolated noncompaction of the myocardium in adults. Mayo Clin Proe, 1997,72 (1) :26-31.
  • 4Stollberger C, Finsterer J. Left ventricular hypertrabeculation/ noncompaction. J Am Soc Echocardiogr, 2004, 17 ( 1 ) :91-100.
  • 5Zambrano E, Marshalko SJ, Jaffe CC, et al. Isolated noncompaction of the ventricular myocardium: clinical and molecular aspects of a rare eardiomyopathy. Lab Invest, 2002, 82 (2) :117-122.
  • 6Oechslin 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 Coil Cardiol, 2000, 36(2) :493-500.
  • 7Pignatelli RH, McMahon C J, Dreyer WJ, et al. Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy. Circulation, 2003,108 (21) :2672-2678.
  • 8Chin TK, Perloff JK, Williams RG, et al. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation, 1990,82 (2) :507-513.
  • 9杨作成.心肌致密化不全[J].实用儿科临床杂志,2007,22(1):76-78. 被引量:12
  • 10陈东,张慧信,方微,武迎,商建峰,王福,石凤茹.心内膜弹力纤维增生症临床病理特征及误诊原因分析[J].临床与实验病理学杂志,2008,24(1):43-46. 被引量:12

二级参考文献19

  • 1苗翠莲,张兆琪,郭曦,王珏,宴子旭,赵轶柯.孤立性心肌致密化不全的MRI诊断[J].中华放射学杂志,2005,39(6):588-592. 被引量:19
  • 2Maron BJ,Towbin JA,Thiene G,et al.Contemporary definitions and classification of the cardiomyopathies[J].Circulation,2006,113 (14):1807-1816.
  • 3Rirrer M,Oechslin E,Sutsch G,et al.Isolated noncompaction of the myocardium in adults[J].Mayo Clin Proc,1997,72 (1):26-31.
  • 4Stollberger C,Finsterer J.Left ventricular hypertrabeculation/noncompaction[J].J Am Soc Echocardiogr,2004,17(1):91-100.
  • 5Oechslin 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].J Am Coll Cardiol,2000,36(2):493 -500.
  • 6Ichida F,Tsubata S,Bowles KR,et al.Novel gene mutations in patients with left ventricular noncompaction or barth syndrome[J].Circulation,2001,103(9):1256-1263.
  • 7Sasse-Klaassen S,Probst S,Gerull B,et al.Novel gene locus for autosomal dominant left ventricular noncompaction maps to chromosome 11p15[J].Circulation,2004,109 (22):2720-2723.
  • 8Ksnemoto N,Horigome H,Nakayama J,et al.Interstitial 1q43 -q43 deletion with left ventricular noncompaction myocardium[J].Eur J Med Genet,2006,49 (3):247-253.
  • 9Shou W,Aghdasi B,Armstrong DL,et al.Cardiac defects and altered ryanodine receptor function in mice lacking FKBP12[J].Nature,1998,391 (6666):489 -492.
  • 10King T,Blang Y,Webb S,et al.Expression of Peg1 (Mest) in the developing mouse heart:Involvement in trabeculation[J].Dev Dyn,2002,225(2):212 -215.

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