Okada Seigo, Muneuchi Jun, Nagatomo Yusaku, Nonaka Kaori, Iida Chiaki, Shirouzu Hiromitsu, Matsuoka Ryohei, Watanabe Mamie, Joo Kunitaka
Department of Pediatrics, Japan Community Healthcare Organization, Kyushu Hospital.
Department of Rehabilitation, Japan Community Healthcare Organization, Kyushu Hospital.
Int Heart J. 2018 Jul 31;59(4):873-876. doi: 10.1536/ihj.17-292. Epub 2018 May 23.
We present a 16-year-old male patient with hypoplastic left heart syndrome who developed protein-losing enteropathy (PLE) and plastic bronchitis (PB) after a Fontan operation. He received medical therapies, including albumin infusion, unfractionated heparin, and high-dose anti-aldosterone therapy but could not obtain clinical relief. Biphasic cuirass ventilation (BCV) led to expectoration of bronchial casts and prompt resolution of PB. Notably, clinical symptoms related to PLE were dramatically improved after starting BCV. A brief period of BCV increased stroke volume from 26±1.4 to 39±4.0 mL. This case suggests that BCV could be an effective treatment for PLE in patients with failing Fontan circulation.
我们报告一名16岁患有左心发育不全综合征的男性患者，其在Fontan手术后出现蛋白丢失性肠病（PLE）和塑形性支气管炎（PB）。他接受了包括输注白蛋白、普通肝素和高剂量抗醛固酮治疗在内的药物治疗，但未获得临床缓解。双相胸甲通气（BCV）促使咳出支气管铸型并使PB迅速缓解。值得注意的是，开始BCV治疗后，与PLE相关的临床症状显著改善。短时间的BCV使每搏输出量从26±1.4 mL增加至39±4.0 mL。该病例表明，BCV可能是治疗Fontan循环衰竭患者PLE的有效方法。
