de Blok K, Alberts C, Düren D R, David G K
Afd. Interne Geneeskunde, Academisch Medisch Centrum, Amsterdam.
Ned Tijdschr Geneeskd. 1991 Mar 16;135(11):475-8.
In 2 patients arrhythmias and conduction disorders were the first symptoms at presentation of cardiac sarcoidosis. One patient with severe conduction disorders showed normalisation of the EKG during steroid treatment. The other patient developed an overall cardiomyopathy and the arrhythmia was treated with conventional drugs. The frequency of cardiac involvement in sarcoidosis is much higher than that of clinical symptoms of sarcoid heart disease. Cardiac sarcoidosis is increasingly diagnosed in the last few decades. In most cases, it presents with sudden death, arrhythmia, conduction disorders or cardiomyopathy. The main diagnostic pathological feature is evidence of noncaseating granulomas, but mononuclear cell infiltration and focal interstitial fibrosis have also been found. The basal part of the interventricular septum is particularly prone to involvement. If conventional therapy in clinically important cases proves inadequate, steroid therapy should be added. It is recommended to make an EKG in every patient presenting with sarcoidosis even in the absence of cardiac symptoms. Sarcoid heart disease should be considered in every patient with diagnostic sarcoidosis and heart disease but also in every case of difficult heart disease without an obvious aetiology. Because of the relatively early age at onset, life expectancy is shortened.
在2例患者中,心律失常和传导障碍是心脏结节病初发时的首发症状。1例患有严重传导障碍的患者在类固醇治疗期间心电图恢复正常。另1例患者发展为全心心肌病,心律失常采用传统药物治疗。结节病中心脏受累的频率远高于结节性心脏病的临床症状出现频率。在过去几十年中,心脏结节病的诊断越来越多。在大多数情况下,它表现为猝死、心律失常、传导障碍或心肌病。主要的诊断病理特征是非干酪样肉芽肿,但也发现了单核细胞浸润和局灶性间质纤维化。室间隔基部特别容易受累。如果在临床上重要的病例中常规治疗被证明不足,应加用类固醇治疗。建议对每例结节病患者即使在没有心脏症状时也进行心电图检查。每例诊断为结节病且患有心脏病的患者,以及每例病因不明的疑难心脏病患者,都应考虑结节性心脏病。由于发病年龄相对较早,预期寿命缩短。