Tsuda Etsuko, Yamada Osam
Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, 6-1 Kishibe-shinmachi, Suita, Osaka, Japan.
Department of Pathology, National Cerebral and Cardiovascular Center, 6-1 Kishibe-shinmachi, Suita, Osaka, Japan.
Pediatr Cardiol. 2023 Jan;44(1):187-195. doi: 10.1007/s00246-022-02971-4. Epub 2022 Jul 21.
Long-term outcomes of patients with left ventricular dysfunction after Kawasaki disease (KD) remain unknown. The clinical course of 37 patients (29 males 8 females) with left ventricular ejection function (LVEF) less than 45% after KD since 1978 was retrospectively investigated. Cardiac events in this study were defined as admissions because of heart failure, fatal ventricular arrhythmias, heart transplantation, and death. Their ages when evaluated ranged from 1 to 70 years (median 35 years). The onset of KD ranged from 2 months to 20 years (median 21 months). All patients had bilateral coronary artery lesions, and multi-vessel occlusion occurred in 31 patients (84%). Previous myocardial infarction (MI) was found in 28 patients (76%). The interval from the onset of acute KD to the initial MI ranged from 15 days to 25 years (median 3 months). Reperfusion therapy was successful in 6 patients (30%), and coronary artery bypass grafting was performed in 23 patients (62%). Non-sustained ventricular tachycardia and fatal ventricular arrhythmias were detected in 11 patients and 22 patients, respectively. There were 15 deaths (41%). The 20-year and 50-year survival rates after KD were 84% (95% CI 67-92) and 54% (34-73), respectively (n = 37). The 30-year cardiac event-free rate after the detection of low LVEF was 42% (95% CI 27-59). The cutoff point of the left ventricular end-diastolic dimension for cardiac events was 65 mm. Patients with low LVEF had fatal ventricular arrhythmias and a worsening of their ischemic cardiomyopathy after 30 years of age and their outcomes were poor.
川崎病（KD）后左心室功能不全患者的长期预后尚不清楚。对自1978年以来37例KD后左心室射血功能（LVEF）低于45%的患者（29例男性，8例女性）的临床病程进行了回顾性研究。本研究中的心脏事件定义为因心力衰竭、致命性室性心律失常、心脏移植和死亡而入院。评估时他们的年龄为1至70岁（中位数35岁）。KD的发病时间为2个月至20年（中位数21个月）。所有患者均有双侧冠状动脉病变，31例（84%）发生多支血管闭塞。28例（76%）患者曾发生过心肌梗死（MI）。从急性KD发作到首次MI的间隔时间为15天至25年（中位数3个月）。6例患者（30%）再灌注治疗成功，23例患者（62%）接受了冠状动脉旁路移植术。分别在11例和22例患者中检测到非持续性室性心动过速和致命性室性心律失常。有15例死亡（41%）。KD后的20年和50年生存率分别为84%（95%CI 67 - 92）和54%（34 - 73）（n = 37）。检测到低LVEF后的30年无心脏事件发生率为42%（95%CI 27 - 59）。发生心脏事件的左心室舒张末期内径的截断点为65mm。LVEF低的患者在30岁后发生致命性室性心律失常且缺血性心肌病恶化，其预后较差。
