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一期杂交姑息治疗对左心发育不全综合征的影响:组织病理学研究结果

Impact of Hybrid Stage 1 Palliation for Hypoplastic Left Heart Syndrome: Histopathological Findings.

作者信息

Kido Takashi, Hoashi Takaya, Kitano Masataka, Shimada Masatoshi, Kurosaki Kenichi, Ishibashi-Ueda Hatsue, Ichikawa Hajime

机构信息

Department of Pediatric Cardiovascular Surgery, National Cardiovascular and Cerebral Center, 5-7-1, Fujishiro-dai, Suita, Osaka, 565-8565, Japan.

Department of Pediatric Cardiology, National Cardiovascular and Cerebral Center, 5-7-1, Fujishiro-dai, Suita, Osaka, 565-8565, Japan.

出版信息

Pediatr Cardiol. 2018 Jun;39(5):1001-1008. doi: 10.1007/s00246-018-1851-6. Epub 2018 Mar 9.

Abstract

The purpose of the study is to analyze the impact of hybrid stage 1 palliation on right ventricular myocardial pathology in hypoplastic left heart syndrome. Sufficient amount of right ventricular biopsies could be obtained from 16 of 32 patients who underwent Norwood operation between 2007 and 2013. Histopathological findings of right ventricle in patients who underwent primary Norwood operation (primary group, n = 5), patients with aortic atresia (HS1P AA group, n = 6) or aortic stenosis (HS1P AS group, n = 5) who underwent staged Norwood palliation following hybrid stage 1 palliation were compared. To eliminate the influence of right ventricular pressure afterload, right ventricular biopsies were obtained from patients with truncus arteriosus communis (TAC group, n = 6) at total correction. The percentage of myocardial fibrosis was significantly higher in both HS1P groups than in TAC group; moreover, it was significantly higher in HS1P AA group than in primary group. Capillary vascular density was significantly lower in all hypoplastic left heart syndrome groups than in TAC group. At the sub-endocardial layer, collagen type I/III ratios were higher in HS1P AA group than in other hypoplastic left heart syndrome groups. The proportions of N-cadherin immunolocalized to myocyte termini were lower in all hypoplastic left heart syndrome groups than in TAC group. Right ventricle in hypoplastic left heart syndrome showed more significant ischemic change and myocardial immaturity than that in truncus arteriosus communis. Hybrid stage 1 palliation for aortic atresia would be a risk factor for further right ventricular myocardial ischemia.

摘要

本研究的目的是分析一期杂交姑息治疗对左心发育不全综合征右心室心肌病理的影响。在2007年至2013年期间接受诺伍德手术的32例患者中,有16例获得了足够数量的右心室活检样本。比较了接受初次诺伍德手术的患者(初次组,n = 5)、在一期杂交姑息治疗后接受分期诺伍德姑息治疗的主动脉闭锁患者(HS1P AA组,n = 6)或主动脉狭窄患者(HS1P AS组,n = 5)右心室的组织病理学结果。为消除右心室压力后负荷的影响,在完全矫正时从共同动脉干患者(TAC组,n = 6)获取右心室活检样本。两个HS1P组的心肌纤维化百分比均显著高于TAC组;此外,HS1P AA组显著高于初次组。所有左心发育不全综合征组的毛细血管血管密度均显著低于TAC组。在心内膜下层,HS1P AA组的I/III型胶原比率高于其他左心发育不全综合征组。所有左心发育不全综合征组中N-钙黏蛋白免疫定位到心肌细胞末端的比例均低于TAC组。左心发育不全综合征的右心室比共同动脉干的右心室表现出更显著的缺血变化和心肌不成熟。主动脉闭锁的一期杂交姑息治疗将是右心室心肌进一步缺血的危险因素。

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