CARDIAC SARCOID - CLINICOPATHOLOGIC STUDY OF 84 UNSELECTED PATIENTS WITH SYSTEMIC SARCOIDOSIS

CARDIAC SARCOID - CLINICOPATHOLOGIC STUDY OF 84 UNSELECTED PATIENTS WITH SYSTEMIC SARCOIDOSIS
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DOI:
10.1161/01.cir.58.6.1204
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发表时间:
1978-01-01
期刊:
影响因子:
37.8
通讯作者:
BULKLEY, BH
BULKLEY, BH
中科院分区:
医学1区
文献类型:
--
作者:
SILVERMAN, KJ;HUTCHINS, GM;BULKLEY, BH

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虽然结节病可能累及心肌,但关于其发病率和意义的资料很少。对84例结节病尸检病例进行了研究。患者年龄18-80岁(平均46岁),61%为女性,其中23例(27%)有心肌肉芽肿。8例(35%)患者临床上无症状,15例(65%)患者有心力衰竭和/或心律失常和传导缺陷病史。在23例患者中,只有4例(17%)有明显的、广泛的心肌病变:3例(75%)有记录的心律失常。所有4例患者均在平均36岁时突然意外死亡,只有2例患者在生前怀疑患有结节病。其他19例患者(83%)在显微镜下有明显的肉芽肿累及。其中,8例(42%)有节律或传导障碍,3例(16%)猝死,尽管猝死者中没有一人有公认的节律或传导障碍。在没有心脏结节病的患者中,9例(15%)有心律或传导障碍,8例(13%)猝死。虽然至少25%的结节病患者发生心肌受累,但最常累及一小部分心肌,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肉芽肿,心肌结节病的真实发生率可能比本文所建议的更高。心律和传导紊乱在心脏类肉瘤组中更常见,但研究结果表明,只有一小部分严重的、明显的心肌类肉瘤患者猝死风险增加。
Although sarcoid may involve the myocardium, there is little information on its incidence or significance. Autopsied patients (84) with sarcoidosis were studied. The patients ranged in age from 18-80 yr (average 46 yr) and 61% were women; 23 (27%) of them had myocardial granulomas. In 8 (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 4 (17%) had grossly evident, widespread myocardial lesions: 3 (75%) had documented arrhythmias. All 4 had sudden, unexpected death at an average age of 36 yr; in only 2 had sarcoid been suspected during life. The other 19 patients (83%) had microscopically evident granulomatous involvement. Of these, 8 (42%) had a rhythm or conduction disturbance and 3 (16%) sudden death, although none of those who suffered sudden death had a recognized rhythm or conduction disturbance. Of those without cardiac sarcoidosis 9 (15%) had a rhythm or conduction disturbance and 8 (13%) suffered a sudden death. 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.