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与肥厚型心肌病相关的主动脉下间隔心肌切除术的外科病理学。204例病例研究(1996 - 2000年)

Surgical pathology of subaortic septal myectomy associated with hypertrophic cardiomyopathy. A study of 204 cases (1996-2000).

作者信息

Lamke Geoffrey T, Allen Rachel D, Edwards William D, Tazelaar Henry D, Danielson Gordon K

机构信息

Mayo Medical School, Mayo Clinic, 200 First Street S.W., Rochester, MN, USA.

出版信息

Cardiovasc Pathol. 2003 May-Jun;12(3):149-58. doi: 10.1016/s1054-8807(03)00036-x.

DOI:10.1016/s1054-8807(03)00036-x
PMID:12763554
Abstract

BACKGROUND

No large surgical series have qualitatively examined all histopathologic features of hypertrophic cardiomyopathy (HCM).

METHODS

Medical records and microscopic slides were reviewed from 204 patients undergoing septal myectomy for HCM at Mayo Clinic Rochester.

RESULTS

The 108 females and 96 males (1-86 years old; mean, 48) included 133 patients <60 years old (Group 1) and 71 patients > or =60 years (Group 2). Clinical features more prevalent in Group 2 than in Group 1 included female gender (68% vs. 45%; P=.003), aortic valve disease (31% vs. 12%; P=.01) and severe coronary atherosclerosis (43% vs. 9%; P=.001). Overall, microscopic abnormalities included myocyte hypertrophy (100%), endocardial (96%) and myocardial (93%) fibrosis, myocyte disarray (79%) and vacuolization (60%), endocardial inflammation (48%), arterial thickening (46%), dilated venules (28%), arterial dysplasia (16%), left bundle branch tissue (12%), infarction (2%), endocardial (1%) and myocardial (<1%) calcium, and amyloid (1%; senile type in all three). Lesions more prevalent in Group 1 than Group 2 were vacuolization (68% vs. 45%; P=.002), disarray (87% vs. 65%; P=.0003) and dilated venules (33% vs. 18%; P=.02). In contrast, lesions more frequent in Group 2 than in Group 1 included left bundle branch (20% vs. 8%; P=.02) and amyloid and endocardial calcium (4% vs. 0%; P=.04, each).

CONCLUSIONS

Among patients undergoing septal myectomy for HCM, 53% were women and 35% were >/=60 years old. The most common microscopic features were hypertrophy, disarray, fibrosis, inflammation and vascular alterations. Disarray cannot be used as a morphologic hallmark for HCM in small surgical myectomy specimens because it was absent in 21% of the patients. Because amyloid unexpectedly affected three elderly patients, routine amyloid staining is recommended for patients >/=65 years old.

摘要

背景

尚无大型手术系列研究对肥厚型心肌病(HCM)的所有组织病理学特征进行定性分析。

方法

回顾了梅奥诊所罗切斯特分院204例因HCM接受室间隔心肌切除术患者的病历和显微镜切片。

结果

108例女性和96例男性(年龄1 - 86岁;平均48岁),其中133例年龄<60岁(第1组),71例年龄≥60岁(第2组)。第2组比第1组更常见的临床特征包括女性(68%对45%;P = 0.003)、主动脉瓣疾病(31%对12%;P = 0.01)和严重冠状动脉粥样硬化(43%对9%;P = 0.001)。总体而言,显微镜下异常包括心肌细胞肥大(100%)、心内膜(96%)和心肌(93%)纤维化、心肌细胞排列紊乱(79%)和空泡化(60%)、心内膜炎症(48%)、动脉增厚(46%)、小静脉扩张(28%)、动脉发育异常(16%)、左束支组织(12%)、梗死(2%)、心内膜(1%)和心肌(<1%)钙化以及淀粉样变(1%;均为老年型)。第1组比第2组更常见的病变是空泡化(68%对45%;P = 0.002)、排列紊乱(87%对65%;P = 0.0003)和小静脉扩张(33%对18%;P = 0.02)。相比之下,第2组比第1组更常见的病变包括左束支(20%对8%;P = 0.02)以及淀粉样变和心内膜钙化(均为4%对0%;P = 0.04)。

结论

在因HCM接受室间隔心肌切除术的患者中,53%为女性,35%年龄≥60岁。最常见的显微镜下特征是肥大、排列紊乱、纤维化、炎症和血管改变。在小型手术心肌切除标本中,排列紊乱不能用作HCM的形态学标志,因为21%的患者不存在该特征。由于淀粉样变意外影响了3例老年患者,建议对年龄≥65岁的患者进行常规淀粉样变染色。

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