Chlebowski Meghan M, Heese Bryce A, Malloy-Walton Lindsey E
1Department of Pediatric Cardiology,Children's Mercy,Kansas City,MO,USA.
2Department of Pediatrics,University of Missouri-Kansas City School of Medicine,Kansas City,MO,USA.
Cardiol Young. 2018 May;28(5):786-787. doi: 10.1017/S1047951118000215. Epub 2018 Mar 6.
Cardiac involvement has been reported in various mucopolysaccharidoses syndromes. Cardiac valve pathology is the most prominent cardiac manifestation of patients with these syndromes. To date, there have been no reports of early childhood onset of high-grade atrioventricular block in patients with Hunter syndrome. We present a case of a 3-year-old boy with Hunter syndrome who was found to have various degrees of atrioventricular block. This case highlights the importance of early routine cardiac screening for conduction abnormalities and close follow-up in patients with mucopolysaccharidoses syndromes.
已有报道称多种黏多糖贮积症综合征会累及心脏。心脏瓣膜病变是这些综合征患者最突出的心脏表现。迄今为止，尚无亨特综合征患者在幼儿期出现高度房室传导阻滞的报道。我们报告一例3岁亨特综合征男孩，发现其存在不同程度的房室传导阻滞。该病例强调了对黏多糖贮积症综合征患者进行早期常规心脏传导异常筛查及密切随访的重要性。
