黏多糖贮积症（Hurler综合征）成年患者二尖瓣和主动脉瓣联合置换术的结果

Outcome of Combined Mitral and Aortic Valve Replacement in Adults With Mucopolysaccharidosis (the Hurler Syndrome).

作者信息

Robinson Clark R, Roberts William C

机构信息

Baylor Heart and Vascular Institute, Departments of Pathology and Internal Medicine (Division of Cardiology), Baylor University Medical Center at Dallas, Baylor Scott & White Health, Dallas, Texas.

出版信息

Am J Cardiol. 2017 Dec 1;120(11):2113-2118. doi: 10.1016/j.amjcard.2017.08.001. Epub 2017 Aug 12.

DOI:10.1016/j.amjcard.2017.08.001

PMID:28964381

Abstract

We describe 2 adult sisters with type I mucopolysaccharidosis (MPS) who underwent combined mitral and aortic valve replacement for mitral and aortic valve stenosis. One died early postoperatively and the other survived but had a repeat double-valve replacement 1 month after the first. We analyzed previously reported patients with MPS and valve replacement to learn of their outcomes. The study of our 2 patients and those previously reported suggests that valve replacement in patients with MPS should be viewed with extreme caution.

摘要

我们描述了2例患有I型黏多糖贮积症（MPS）的成年姐妹，她们因二尖瓣和主动脉瓣狭窄接受了二尖瓣和主动脉瓣联合置换术。其中1例在术后早期死亡，另1例存活，但在首次手术后1个月进行了再次双瓣膜置换。我们分析了先前报道的接受瓣膜置换术的MPS患者，以了解他们的治疗结果。对我们这2例患者以及先前报道患者的研究表明，对于MPS患者进行瓣膜置换术应极其谨慎。