Tavora Fabio, Cresswell Nathaniel, Li Ling, Ripple Mary, Solomon Carol, Burke Allen
Department of Cardiovascular Pathology, Armed Forces Institute of Pathology, Washington, DC, USA.
Am J Cardiol. 2009 Aug 15;104(4):571-7. doi: 10.1016/j.amjcard.2009.03.068. Epub 2009 Jun 18.
The clinical diagnosis of cardiac sarcoidosis can be difficult and is largely dependent on newer imaging modalities. A retrospective search of sudden cardiac deaths was performed from a reference laboratory and statewide medical examiner system for a 12-year period. Planimetry was performed on gross photographs of transverse short-axis sections, and the phase of the lesion and the portion of myocardium extent was estimated histologically. Lesions were classified histologically as early (primarily lymphocytic), intermediate (primarily granulomatous), and late (primarily scar). A total of 41 cases were found, including 25 in which the death was ascribed to sarcoidosis of the heart (group 1) and 16 in which sudden death was due to other findings (group 2). No significant differences were found in age or activity at death, although gross scars and epicardial nodules were more frequent in group 1 (p <0.0001). In the hearts with gross scars, the ventricular septum had the largest percentage of involvement (32%) followed by the posterior wall (25%). Histologically, the intermediate phase predominated in group 1, and the late phase predominated in group 2. Approximately 50% of the cases in group 1 had involvement in the right ventricular apex and septum, suggesting a positive yield by biopsy. In conclusion, cardiac sarcoidosis causing sudden death is characterized by extensive active granulomas with a predilection for the subepicardium and ventricular septum.
心脏结节病的临床诊断可能具有挑战性,并且在很大程度上依赖于更新的成像方式。我们从一个参考实验室和全州范围内的法医系统对12年期间的心源性猝死病例进行了回顾性检索。对横向短轴切片的大体照片进行了面积测量,并通过组织学方法估计病变的阶段和心肌受累范围。病变在组织学上分为早期(主要为淋巴细胞性)、中期(主要为肉芽肿性)和晚期(主要为瘢痕性)。共发现41例病例,其中25例死亡归因于心脏结节病(第1组),16例猝死归因于其他原因(第2组)。尽管第1组中大体瘢痕和心外膜结节更为常见(p<0.0001),但两组在年龄或死亡时的活动状态方面未发现显著差异。在有大体瘢痕的心脏中,室间隔受累比例最高(32%),其次是后壁(25%)。组织学上,第1组以中期为主,第2组以晚期为主。第1组中约50%的病例右心室心尖和室间隔受累,提示活检有阳性结果。总之,导致猝死的心脏结节病的特征是广泛的活动性肉芽肿,好发于心外膜下和室间隔。