背景:α-gal综合征是一种过敏性疾病,其中个体在食用红肉及其衍生产品时产生免疫介导的超敏反应。它的诊断对接受心脏手术的人很重要,由于患者经常需要大剂量的普通肝素或插入外科植入物,两者都是猪或牛的来源。目前尚无α-gal患者的肝素给药指南,在接受生物瓣膜置换或其他假体后,对这些患者的长期临床意义的了解甚至更少。
方法:我们介绍了一例31岁男性,因巨大的房间隔缺损(ASD)和二尖瓣脱垂(MVP)在α-gal综合征的情况下接受了心脏手术。患者在接受二尖瓣修复手术一年后继续表现良好,使用牛心包进行三尖瓣修复和ASD闭合。他对使用肝素产品或牛心包贴片没有不良反应。这个罕见的病例是由一个由心胸外科组成的多学科小组管理的,心脏麻醉学,和过敏/免疫学导致最佳结果,尽管患者有相关的过敏史。
结论:该案例强调,如果没有其他选择,可以考虑使用牛心包和猪肝素来关闭轻度形式的α-gal过敏患者的间隔缺损。需要进一步的研究来研究这种潜在的含α-gal的假体和肝素暴露的长期结果,并建立最佳的决策算法和预防方案。
BACKGROUND: Alpha-gal syndrome is an allergic condition in which individuals develop an immune-mediated hypersensitivity response when consuming red meat and its derived products. Its diagnosis is important in individuals undergoing cardiac surgery, as patients frequently require large doses of unfractionated heparin or the insertion of surgical implants, both of which are porcine or bovine in origin. There are currently no guidelines for heparin administration in alpha-gal patients, with even less knowledge regarding the long-term clinical implications of these patients after receiving bioprosthetic valve replacements or other prostheses.
METHODS: We present the
case of a 31-year-old male who underwent cardiac surgery in the setting of alpha-gal syndrome for a large atrial septal defect (ASD) and mitral valve prolapse (MVP). The patient continues to do well one year after undergoing a mitral valve repair, tricuspid valve repair and an ASD closure using bovine
pericardium. He sustained no adverse reaction to the use of heparin products or the presence of a bovine pericardial patch. This rare
case was managed by a multidisciplinary team consisting of cardiothoracic surgery, cardiac anesthesiology, and allergy/immunology that led to an optimal outcome despite the patient\'s pertinent allergic history.
CONCLUSIONS: This
case highlights that the use of bovine
pericardium and porcine heparin to close septal defects in patients with milder forms of alpha-gal allergy can be considered if other options are not available. Further studies are warranted to investigate the long-term outcomes of such potential alpha-gal containing prostheses and heparin exposure and establish the optimal decision making algorithm and prophylactic regimen.