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[成人艾森曼格综合征的演变与预后]

[Evolution and prognosis of the adult Eisenmenger syndrome].

作者信息

Siegert D, Trenckmann H

出版信息

Z Gesamte Inn Med. 1981 Sep 1;36(17):607-11.

PMID:7345807
Abstract

From the patients of the Cardiologic Department of the Medical Clinic of Leipzig University 34 adult patients aged 21 to 62 years with the diagnosis of Eisenmenger's syndrome confirmed by heart catheterization were established. The cases in question were 20 ventricular septum defects, 8 atrial septum defects and 6 open arterial ducts. In 26 of the 34 patients first symptoms appeared already during the preschool period, in 4 patients with atrial septum defect in the 12th year as well as between the 35th and 46th year of age, in 4 patients with ventricular septum defect twice in the 8th or 26th and 50th year of age, respectively. The average time of diagnostics from the beginning of the complaints to the heart catheterization was with 15 years and 3 months very long. The average survival time after diagnosis of shunt reversal by means of the heart catheter was 9.7 years in the ventricular septum defect, 5.6 years in the atrial septum defect and 3.8 years in the open arterial ducts. Since 19 of the 34 patients with Eisenmenger's syndrome are still alive and the moment of the shunt reversal does not correspond to the moment of the diagnostics, the average life expectancy is greater. It is higher than the values given in literature, which vary between 2 and 10 years after the beginning of the shunt reversal. The progressing of the clinical picture could be objectified by annual increases of various parameters, such as hemoglobin, hematocrit, angle alpha QRS, Sokolov's index for right hypertrophy and radiologically established sizes of surface and diameter of the heart and the pulmonary vessels. Correlations of abrupt enlargements and acute diseases were found only for the prominence of the pulmonary segment and the basal diameter of the heart. The examinations showed that in the course of Eisenmenger's syndrome the pulmonary hypertension, the polyglobulia, the right heart hypertrophy, the size of the heart and last not least the complaints increase continuously or in dependence of complications (heart insufficiency, carditis, pneumonia, embolism a.o.).

摘要

从莱比锡大学医学诊所心脏病科的患者中,确定了34例年龄在21至62岁之间、经心导管检查确诊为艾森曼格综合征的成年患者。其中,室间隔缺损20例,房间隔缺损8例,动脉导管未闭6例。34例患者中,26例在学龄前就已出现首发症状,4例房间隔缺损患者分别在12岁以及35至46岁时出现症状,4例室间隔缺损患者分别在8岁、26岁和50岁时两次出现症状。从出现症状开始到进行心导管检查的平均诊断时间长达15年零3个月。经心导管检查诊断分流逆转后,室间隔缺损患者的平均生存时间为9.7年,房间隔缺损患者为5.6年,动脉导管未闭患者为3.8年。由于34例艾森曼格综合征患者中有19例仍在世,且分流逆转的时间与诊断时间不一致,因此平均预期寿命更长。该值高于文献中给出的2至10年(分流逆转开始后)的数值。通过各种参数(如血红蛋白、血细胞比容、QRS波群α角、右心室肥厚的索科洛夫指数以及通过放射学确定的心脏和肺血管的表面积和直径)的逐年增加,可以客观地反映临床症状的进展。仅在肺段突出和心脏基部直径方面发现了突然增大与急性疾病之间的相关性。检查表明,在艾森曼格综合征的病程中,肺动脉高压(肺动脉高压)、红细胞增多症、右心室肥厚、心脏大小,最后但并非最不重要的是症状会持续增加或因并发症(心力衰竭、心肌炎、肺炎、栓塞等)而加重。

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