Tan C T, Schaff H V, Miller F A, Edwards W D, Karnes P S
Section of Thoracic and Cardiovascular Surgery, Mayo Clinic, Rochester, MN 55905.
Circulation. 1992 Jan;85(1):188-95. doi: 10.1161/01.cir.85.1.188.
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.
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.
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.
马罗-拉米综合征是一种黏多糖代谢的溶酶体贮积病（Ⅵ型黏多糖贮积症），可能累及二尖瓣和主动脉瓣。受影响的患者还存在其他骨骼和口咽畸形，这使麻醉和手术管理变得复杂。
本报告描述了4例马罗-拉米综合征患者的临床、超声心动图和病理检查结果。三兄妹中的两人因主动脉瓣和二尖瓣狭窄成功接受了双瓣置换术。第三个兄妹的主动脉瓣和二尖瓣增厚且纤维化，在髋关节手术后死于败血症。第四例非亲属患者也成功接受了双瓣置换术。
我们的经验强调了该综合征患者术前评估和手术治疗的潜在困难，以及与气道管理相关的独特问题。
