Damus-Kaye-Stansel吻合术伴肺动脉干瓣重建20年。

IF 0.6 Q3 Medicine

ASIAN CARDIOVASCULAR & THORACIC ANNALS Pub Date : 2025-06-01 Epub Date: 2025-07-14 DOI:10.1177/02184923251356574

Kanta Araki, Kanji Ishizu, Takayuki Takehara, Koji Kagisaki, Hideto Ozawa

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引用次数: 0

摘要

我们报告一例24岁男性单心室先天性心脏病合并家族性法布里病，在Fontan手术22年后发展为进行性主动脉下狭窄。尽管心室容量稳定，但由于法布里病相关的心肌肥厚，主动脉下狭窄发展。采用带瓣导管和Damus-Kaye-Stansel （DKS）吻合行肺动脉主干重建。术后流出道通畅，瓣膜功能稳定。本病例强调Fabry病对心室流出道梗阻的影响，显示DKS吻合术治疗主动脉下狭窄的疗效。

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Damus-Kaye-Stansel anastomosis with pulmonary arterial trunk reconstruction using valved conduit 20 years after Fontan procedure.

We report a case of 24-year-old man with single ventricle congenital heart disease and concomitant familial Fabry disease who developed progressive subaortic stenosis 22 years after Fontan procedure. Despite stable ventricular volumes, subaortic stenosis developed due to Fabry disease-related myocardial hypertrophy. He underwent pulmonary arterial trunk reconstruction with valved conduit and Damus-Kaye-Stansel (DKS) anastomosis. Postoperatively, smooth outflow tracts and stable valve function were observed. This case highlights the impact of Fabry disease on ventricular outflow tract obstruction and shows the efficacy of the DKS anastomosis in treating subaortic stenosis.

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来源期刊

ASIAN CARDIOVASCULAR & THORACIC ANNALS Multiple-

CiteScore

1.30

自引率

0.00%

发文量

期刊介绍： The Asian Cardiovascular and Thoracic Annals is an international peer-reviewed journal pertaining to cardiovascular and thoracic medicine. Besides original clinical manuscripts, we welcome research reports, product reviews, reports of new techniques, and findings of special significance to Asia and the Pacific Rim. Case studies that have significant novel original observations, are instructive, include adequate methodological details and provide conclusions. Workshop proceedings, meetings and book reviews, letters to the editor, and meeting announcements are encouraged along with relevant articles from authors.