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心脏结节病:84例未经选择的系统性结节病患者的临床病理研究

Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis.

作者信息

Silverman K J, Hutchins G M, Bulkley B H

出版信息

Circulation. 1978 Dec;58(6):1204-11. doi: 10.1161/01.cir.58.6.1204.

DOI:10.1161/01.cir.58.6.1204
PMID:709777
Abstract

Although sarcoid may involve the myocardium, there is little information on its incidence or significance. We studied 84 consecutive autopsied patients with sarcoidosis. The patients ranged in age from 18--80 years (average 46 years) and 61% were women; 23 (27%) of them had myocardial granulomas. In eight (35%) these were clinically silent, and in 15 (65%) there was a history of heart failure and/or arrhythmias and conduction defects. Of the 23 patients, only four (17%) had grossly evident, widespread myocardial lesions: three of these four (75%) had documented arrhythmias. All four had sudden, unexpected death at an average age of 36 years; in only two had sarcoid been suspected during life. The other 19 patients (83%) had microscopically evident granulomatous involvement. Of these, eight (42%) had a thythm or conduction disturbance and three (16%) sudden death, although none of those who suffered sudden death had a recognized rhythm or conduction disturbance. Nine (15%) of those without cardiac sarcoidosis had a rhythm or conduction disturbance and eight (13%) suffered a sudden death. The results show that although myocardial involvement occurs in at least 25% of patients with sarcoid, it most often involves a small portion of myocardium and is clinically silent. Since some of the 61 patients in whom myocardial lesions were not identified may still have had small microscopic granulomas, the true incidence of myocardial sarcoid may be even greater than suggested here. Rhythm and conduction disturbances are more common in the cardiac sarcoid group, but the findings suggest that only the small subset of patients with severe, grossly evident myocardial sarcoid are at increased risk for sudden death.

摘要

虽然结节病可能累及心肌,但关于其发病率或意义的信息却很少。我们对84例连续尸检的结节病患者进行了研究。患者年龄在18至80岁之间(平均46岁),61%为女性;其中23例(27%)有心肌肉芽肿。其中8例(35%)临床上无症状,15例(65%)有心力衰竭和/或心律失常及传导缺陷病史。在这23例患者中,只有4例(17%)有明显的、广泛的心肌病变:这4例中的3例(75%)有记录的心律失常。这4例均在平均36岁时突然意外死亡;生前仅2例怀疑有结节病。其他19例患者(83%)有显微镜下可见的肉芽肿累及。其中,8例(42%)有节律或传导障碍,3例(16%)突然死亡,尽管所有猝死患者均无公认的节律或传导障碍。9例(15%)无心脏结节病的患者有节律或传导障碍,8例(13%)突然死亡。结果表明,虽然至少25%的结节病患者有心肌受累,但最常见的是累及一小部分心肌且临床上无症状。由于61例未发现心肌病变的患者中有些可能仍有微小的显微镜下肉芽肿,心肌结节病的实际发病率可能比这里提示的还要高。节律和传导障碍在心脏结节病组中更常见,但研究结果表明,只有一小部分有严重的、明显的心肌结节病的患者猝死风险增加。

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