Mucopolysaccharidoses Causing Valvular Heart Disease: Report and Review of Surgical Management. World J Pediatr Congenit Heart Surg 2020 Jul;11(4):NP22-NP24
Date
04/20/2017Pubmed ID
28421916DOI
10.1177/2150135117690105Scopus ID
2-s2.0-85042647531 (requires institutional sign-in at Scopus site) 8 CitationsAbstract
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.
Author List
Encarnacion CO, Hang D, Earing M, Mitchell MEAuthors
Dustin Hang MD Assistant Professor in the Anesthesiology department at Medical College of WisconsinMichael Edward Mitchell MD Chief, Professor in the Surgery department at Medical College of Wisconsin
MESH terms used to index this publication - Major topics in bold
AdultEchocardiography
Female
Heart Valve Diseases
Heart Valve Prosthesis Implantation
Humans
Mitral Valve
Mucopolysaccharidosis I