Mucopolysaccharidoses Causing Valvular Heart Disease: Report and Review of Surgical Management

被引：7

作者：

Encarnacion, Carlos O. ^{[1
]}

Hang, Dustin ^{[1
]}

Earing, Michael ^{[2
]}

Mitchell, Michael E. ^{[3
,4
]}

机构：

[1] Med Coll Wisconsin, Div Cardiothorac Surg, Integrated Cardiothorac Surg Residency Program, Milwaukee, WI 53226 USA

[2] Med Coll Wisconsin, Childrens Hosp Wisconsin, Dept Pediat, Div Pediat Cardiol, Milwaukee, WI 53226 USA

[3] Med Coll Wisconsin, Div Cardiothorac Surg, Milwaukee, WI 53226 USA

[4] Med Coll Wisconsin, Childrens Hosp Wisconsin, Div Pediat Cardiovasc Surg, Milwaukee, WI 53226 USA

来源：

WORLD JOURNAL FOR PEDIATRIC AND CONGENITAL HEART SURGERY | 2020年 / 11卷 / 04期

关键词：

mitral valve replacement; congenital heart disease; congenital heart surgery; adult congenital heart disease; INVOLVEMENT; REPLACEMENT;

D O I：

10.1177/2150135117690105

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Mucopolysaccharidosis type I is a genetic disorder with impaired glycosaminoglycan degradation. Cardiac pathologic involvement in this subset of patients is predominantly valvular heart disease. Valvular heart disease seen in these patients will most likely require surgical intervention in their lifetime. Only a limited amount of reports are dedicated to the cardiac surgical management of mucopolysaccharidoses. We present the case of a 32-year-old female with Hurler-Scheie syndrome who required multiple valve replacements due to progression of valvular dysfunction and decline in the quality of life. Multidisciplinary evaluation and discussion early are crucial for quality of life optimization in this cohort of patients.

引用

页码：NP22 / NP24

页数：3

共 7 条

[1] Usefulness of bone marrow transplantation in the Hurler syndrome [J].