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一例患有常见可变免疫缺陷的患者因巨细胞性心肌炎接受心脏移植的病例报告。

Case report of heart transplantation for giant cell myocarditis in a patient with common variable immunodeficiency.

作者信息

Franzon Thomas A, Kovalszki Anna, Rabah Raja, Nicklas John M

机构信息

Cardiovascular Medicine, University of Michigan, 2381 CVC SPC 5853, 1500 E. Medical Center Drive, Ann Arbor, MI 48109-5853, USA.

Allergy and Immunology, University of Michigan, Ann Arbor, MI, 380 Parkland Plaza, Ann Arbor, MI 48103, USA.

出版信息

Eur Heart J Case Rep. 2021 Nov 18;5(12):ytab447. doi: 10.1093/ehjcr/ytab447. eCollection 2021 Dec.

Abstract

BACKGROUND

Solid-organ transplantation in patients with common variable immunodeficiency (CVID) is controversial due to the risk for severe and recurrent infections. Determining transplantation candidacy in CVID patients is further complicated by the presence of CVID-related non-infectious complications that can reduce overall survival and also recur in the transplanted organ. Data regarding solid organ transplantation in patients with CVID are limited, particularly in heart transplantation.

CASE SUMMARY

A 32-year-old female with CVID presented with new heart failure after 3 months of dyspnoea on exertion. Her echocardiogram showed severe global systolic dysfunction with an ejection fraction of approximately 10%, and her right heart catheterization revealed severe biventricular pressure overload and severely reduced cardiac output. Endomyocardial biopsy revealed giant cells and mononuclear infiltrate consistent with giant cell myocarditis (GCM). Despite medical management, she developed progressive cardiogenic shock and underwent uncomplicated orthotopic heart transplantation on hospital Day 38. After 2 years of follow-up, she has had no major infectious complications and continues to have normal graft function with no recurrence of GCM.

CONCLUSION

We report a case of successful heart transplantation for GCM in a patient with CVID, with no major infectious complications after 2 years of follow-up. CVID should not be considered an absolute contraindication for heart transplantation.

摘要

背景

由于存在严重和反复感染的风险,普通可变免疫缺陷(CVID)患者的实体器官移植存在争议。CVID相关的非感染性并发症会降低总体生存率,且在移植器官中也会复发,这使得确定CVID患者的移植候选资格变得更加复杂。关于CVID患者实体器官移植的数据有限,尤其是在心脏移植方面。

病例摘要

一名32岁的CVID女性患者,在出现劳力性呼吸困难3个月后出现了新的心力衰竭。她的超声心动图显示严重的全心收缩功能障碍,射血分数约为10%,右心导管检查显示严重的双心室压力过载和心输出量严重降低。心内膜心肌活检显示有巨细胞和单核细胞浸润,符合巨细胞心肌炎(GCM)。尽管进行了药物治疗,但她仍发展为进行性心源性休克,并于住院第38天接受了无并发症的原位心脏移植。经过2年的随访,她没有出现重大感染并发症,移植心脏功能正常,GCM也未复发。

结论

我们报告了一例CVID患者因GCM成功进行心脏移植的病例,随访2年后无重大感染并发症。CVID不应被视为心脏移植的绝对禁忌证。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e8ec/8664757/5ca7b8fb401c/ytab447f3.jpg

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