Daimon Masahiro, Yamagishi Masaaki
Department of Pediatric Cardiovascular Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Japan.
Cardiol Young. 2005 Oct;15(5):517-9. doi: 10.1017/S104795110500140X.
I-cell disease is a rare, hereditary, and fatal disease, which is often complicated with disease of the cardiac valves. We have experienced a case of severe mitral valvar insufficiency in a 9-month-old male infant with I-cell disease. Mitral valvoplasty was difficult, because the leaflets were remarkably hypertrophic, and the tendinous cords were elongated and adherent. Despite these difficulties, surgery proved effective in the treatment of the congestive heart failure, allowing for further treatment of the I-cell disease.
I型细胞病是一种罕见的遗传性致命疾病，常并发心脏瓣膜疾病。我们曾遇到一例患有I型细胞病的9个月大男婴，出现严重二尖瓣关闭不全。二尖瓣成形术很困难，因为瓣叶明显肥厚，腱索延长并粘连。尽管存在这些困难，但手术被证明对治疗充血性心力衰竭有效，从而可以对I型细胞病进行进一步治疗。
