Ohni S, Goto S, Nakamura H, Yamada T, Taira M, Sakurai I
Second Department of Pathology, Nihon University School of Medicine, Tokyo.
Rinsho Byori. 1998 Feb;46(2):177-81.
Coronary aneurysm in Kawasaki's disease (Acute febril infantile mucocutaneous lymph node syndrome, MCLS) may cause sudden death in childhood and ischemic heart disease in adults. We encountered two adult autopsy cases of Kawasaki's disease with multiple coronary aneurysms. The first case was a 56-year-old man who hospitalized due to recurrent syncope since 51 years of age. At age 55 coronary angiography (CAG) had shown multiple aneurysms in the left and right coronary artery. In September 1991, he developed chest pain and was brought to the hospital, almost dead on arrival (DOA). The patient died later the same day despite cardiopulmonary resuscitation. Autopsy findings showed cardiomegaly (470 g) with multiple coronary aneurysms of three coronary arteries. Microscopically, intimal thickening and medial thinning were found in the aneurysmal wall with calcification and disruption of the internal elastic lamina. The second case, a 28-year-old man had been diagnosed with rheumatic fever and mitral regurgitation at 4 years of age. Coronary aneurysms were noted on CAG at 26 years of age. In April 1992, he developed fever and was admitted to a local hospital where he was diagnosed with infectious endocarditis. After his being transferred to our hospital, disturbance of consciousness suddenly developed and he died in September 1992. Autopsy findings showed cardiomegaly (430 g) with left ventricular hypertrophy and multiple coronary aneurysms in left anterior descending coronary artery and left circumflex coronary artery. The aneurysmal wall showed intimal thickening and medial thinning with multiple recanalizations of occlusive lumina and fibrous intimal thickening. The mitral valve showed mild fibrosis and calcification without valvular deformity. There was no evidence of bacterial endocarditis. Both cases were finally diagnosed as Kawasaki's disease. Ischemic heart disease or lesions related to coronary aneurysm in Kawasaki's disease may show an increased incidence in the near future. Kawasaki's disease should have been followed even in adulthood after treatment in childhood.
川崎病(急性发热性婴儿皮肤黏膜淋巴结综合征,MCLS)中的冠状动脉瘤可能导致儿童期猝死及成人期缺血性心脏病。我们遇到了两例患有多发性冠状动脉瘤的川崎病成人尸检病例。第一例是一名56岁男性,自51岁起因反复晕厥住院。55岁时冠状动脉造影(CAG)显示左、右冠状动脉有多发性动脉瘤。1991年9月,他出现胸痛并被送往医院,入院时几乎濒死(DOA)。尽管进行了心肺复苏,患者仍于同日晚些时候死亡。尸检结果显示心脏增大(470克),三支冠状动脉均有多发性冠状动脉瘤。显微镜下,动脉瘤壁内膜增厚、中膜变薄,伴有钙化及内弹力层破坏。第二例是一名28岁男性,4岁时被诊断为风湿热和二尖瓣反流。26岁时CAG发现冠状动脉瘤。1992年4月,他出现发热,入住当地医院,被诊断为感染性心内膜炎。转至我院后,突然出现意识障碍,于1992年9月死亡。尸检结果显示心脏增大(430克),左心室肥厚,左前降支冠状动脉和左旋支冠状动脉有多发性冠状动脉瘤。动脉瘤壁内膜增厚、中膜变薄,闭塞腔有多处再通及纤维性内膜增厚。二尖瓣有轻度纤维化和钙化,但无瓣膜畸形。无细菌性心内膜炎证据。两例最终均诊断为川崎病。川崎病中的缺血性心脏病或与冠状动脉瘤相关的病变在不久的将来可能会有更高的发病率。即使在儿童期治疗后,川崎病在成年期也应持续随访。