Valvular heart disease in four patients with Maroteaux-Lamy syndrome.
BACKGROUND
Maroteaux-Lamy syndrome is a lysosomal storage disease of mucopolysaccharide metabolism (MPS type VI) that may involve the mitral and aortic valves. Affected patients have other skeletal and oropharyngeal malformations that complicate anesthetic and surgical management.


RESULTS
The present report describes the clinical, echocardiographic, and pathological findings in four patients with Maroteaux-Lamy syndrome. Two of three siblings underwent successful double-valve replacement for aortic and mitral valve stenoses. The third sibling, whose aortic and mitral valves were thick and fibrotic, died from septicemia after hip surgery. A fourth, unrelated patient also had successful double-valve replacement.


CONCLUSIONS
Our experience emphasizes the potential difficulties in preoperative assessment and surgical treatment as well as the unique problems related to airway management in patients with this syndrome.
Tan
C T
CT
Section of Thoracic and Cardiovascular Surgery, Mayo Clinic, Rochester, MN 55905.
Schaff
H V
HV
Miller
F A
FA
Edwards
W D
WD
Karnes
P S
PS
D000328:Adult; D001024:Aortic Valve Stenosis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D004452:Echocardiography; D015150:Echocardiography, Doppler; D005260:Female; D006350:Heart Valve Prosthesis; D006801:Humans; D008297:Male; D008946:Mitral Valve Stenosis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D009087:Mucopolysaccharidosis VI / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology