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儿童期不对称性室间隔肥厚。组织学标准在区分梗阻性和非梗阻性形式方面的不可靠性。

Asymmetric septal hypertrophy in childhood. Unreliability of histologic criteria for differentiation of obstructive and nonobstructive forms.

作者信息

Edwards W D, Zakheim R, Mattioli L

出版信息

Hum Pathol. 1977 May;8(3):277-84. doi: 10.1016/s0046-8177(77)80024-5.

Abstract

Two cases of obstructive asymmetric septal hypertrophy with biventricular outflow obstruction in childhood are presented. Both patients were treated first with propranolol and later by ventriculoseptal myotomy-myectomy. The first patient died two and one-half years following surgery, and the second patient died in the immediate postoperative period. In the first patient the 690 gm. heart had an interventricular septum to posterior left ventricular wall ratio of 1.9, and in the second patient the ratio in the 460 gm. heart was 1.8. In both cases the posterobasal left ventricular free wall was rounded and hypertrophied, as has been reported grossly in obstructive asymmetric septal hypertrophy. However, in both cases, the bizarre disoriented cardiocytes typical of asymmetric septal hypertrophy were present in both ventricular free walls as well as in the interventricular septum, as has been reported in the nonobstructive variety. In these two cases, previously reported morphologic criteria for the differentiation of obstructive and nonobstructive disease are not considered reliable.

摘要

本文报告两例儿童梗阻性不对称性室间隔肥厚合并双心室流出道梗阻的病例。两名患者均首先接受普萘洛尔治疗,随后接受室间隔肌切开-心肌切除术。第一名患者术后两年半死亡,第二名患者在术后即刻死亡。第一名患者690克的心脏,室间隔与左心室后壁的比例为1.9,第二名患者460克的心脏该比例为1.8。在这两例中,左心室后壁基底部均呈圆形且肥厚,这与梗阻性不对称性室间隔肥厚的大体表现一致。然而,在这两例中,如非梗阻性类型所报道的那样,不对称性室间隔肥厚典型的奇异、排列紊乱的心肌细胞在两个心室游离壁以及室间隔中均有出现。在这两例中,先前报道的用于区分梗阻性和非梗阻性疾病的形态学标准并不可靠。

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