Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan.
Department of Public Health, Jichi Medical University, Tochigi, Japan.
J Pediatr. 2020 Mar;218:78-84.e2. doi: 10.1016/j.jpeds.2019.11.008.
To clarify the characteristics of valvular lesions after Kawasaki disease with a Japanese nationwide survey.
Among 137 026 patients in the nationwide Japanese surveys between 2007 and 2016, 290 (0.2%) with valvular sequelae were investigated by questionnaires.
Among the 290 patients with valvular sequelae, mitral regurgitation (MR), tricuspid regurgitation, aortic regurgitation, and pulmonary regurgitation were present 1 month after the development of Kawasaki disease in 183 (63%), 112 (39%), 39 (13%), and 49 (17%) patients, respectively. The numbers of patients with MR during the acute phase and 1 year after developing Kawasaki disease were 208 (72%) and 95 (33%), respectively. MR improved significantly during the late period (P < .0001). Although aortic regurgitation and tricuspid regurgitation also improved significantly (P < .001), pulmonary regurgitation did not change. Ruptured mitral valves chordae tendineae occurred in 6 infants by 6 months of age, within 4 months after the onset of Kawasaki disease. Three patients needed mitral valve plasty, and 1 patient died of acute heart failure. Another 4-month-old girl died of an acute myocardial infarction with MR. In the acute phase, there was a significant difference in the MR severity between the intravenous immunoglobulin-responder group and the intravenous immunoglobulin-resistant group (P < .05).
The inflammation caused by acute Kawasaki disease affects the function of the mitral valves. Most cases of MR improve with the alleviation of inflammation. Severe MR may have decreased with the development of treatment for acute vasculitis. However, ruptured mitral valves chordae tendineae rarely occurs in infants younger than 6 months old, within 4 months after Kawasaki disease.
通过日本全国性调查，阐明川崎病后瓣膜病变的特征。
在 2007 年至 2016 年期间进行的全国性日本调查中，对 137026 名患者中的 290 名（0.2%）有瓣膜后遗症的患者进行了问卷调查。
在 290 名有瓣膜后遗症的患者中，183 名（63%）患者在川崎病发病后 1 个月出现二尖瓣反流、112 名（39%）患者出现三尖瓣反流、39 名（13%）患者出现主动脉瓣反流、49 名（17%）患者出现肺动脉瓣反流。在急性期和川崎病发病后 1 年，分别有 208 名（72%）和 95 名（33%）患者存在二尖瓣反流。二尖瓣反流在晚期显著改善（P<.0001）。虽然主动脉瓣反流和三尖瓣反流也显著改善（P<.001），但肺动脉瓣反流没有变化。6 名婴儿在川崎病发病后 6 个月内发生 6 例二尖瓣腱索断裂。3 名患者需要二尖瓣成形术，1 名患者死于急性心力衰竭。另一名 4 个月大的女孩死于伴有二尖瓣反流的急性心肌梗死。在急性期，静脉注射免疫球蛋白反应组和静脉注射免疫球蛋白耐药组之间的二尖瓣反流严重程度存在显著差异（P<.05）。
急性川崎病引起的炎症影响二尖瓣功能。大多数二尖瓣反流病例随着炎症的缓解而改善。严重的二尖瓣反流可能随着急性血管炎治疗的发展而减少。然而，在川崎病发病后 4 个月内，6 个月以下的婴儿很少发生二尖瓣腱索断裂。
