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伴有心肌受累和心源性休克的系统性渗漏性毛细血管综合征:一例报告

Systemic leak capillary syndrome with myocardial involvement and cardiogenic shock: a case report.

作者信息

Garatti Laura, Wu Maddalena Alessandra, Ammirati Enrico, Sacco Alice

机构信息

Cardiology Department, 'De Gasperis' Cardio Center, ASST Grande Ospedale Metropolitano Niguarda, Milan 20126, Italy.

Department of Internal Medicine, ASST Fatebenefratelli Sacco, 'Luigi Sacco' Hospital, Polo Universitario, University of Milan, Milan, Italy.

出版信息

Eur Heart J Case Rep. 2022 Jun 28;6(7):ytac262. doi: 10.1093/ehjcr/ytac262. eCollection 2022 Jul.

Abstract

BACKGROUND

Systemic capillary leak syndrome (SCLS) is a potentially fatal disorder characterized by relapses of hypovolemic shock episodes.

CASE SUMMARY

We present a case of a 58-year-old man who presented to the Emergency Department with a history of recurrent episodes of syncope in the last hours. A few days before medical contact the patient complained of sore throat, fever, and flu-like symptoms. He was initially admitted with a diagnosis of suspected myopericarditis. Forty-eight hours later, the haemodynamic status suddenly deteriorated to a mixed cardiogenic and shock; an endomyocardial biopsy showed localized inflammatory infiltrates and areas of necrosis of cardiomyocytes with positive viral search for parvovirus B19 (PVB19), therefore the patient was treated with methylprednisolone pulses. Based on the concurrent presence of the typical triad of hypotension, hypoalbuminaemia, and haemoconcentration we suspected systemic leak capillary syndrome potentially triggered by the PVB19 infection with acute myocarditis. The clinical conditions further deteriorated with rhabdomyolysis and acute kidney injury: we started continuous veno-venous haemofiltration adding a cytokines adsorber. In the following hours, we observed a significant clinical improvement. The patient was discharged 1 month later and 5 months after discharge he experienced a new attack of SCLS, this time without myocardial involvement and with prompt symptoms resolution.

CONCLUSION

Systemic capillary leak syndrome is a potentially fatal disorder: early recognition of this entity and prompt initiation of supportive therapy are warranted, therefore, it is paramount that an emergency physician thinks of SCLS in patients with signs of cardiogenic shock and the classical triad of hypotension, hypoalbuminia, and haemoconcentration.

摘要

背景

系统性毛细血管渗漏综合征(SCLS)是一种潜在致命性疾病,其特征为反复出现低血容量性休克发作。

病例摘要

我们报告一例58岁男性患者,该患者因过去数小时内反复出现晕厥发作而就诊于急诊科。在就医前几天,患者主诉咽痛、发热及流感样症状。他最初因疑似心肌炎入院。48小时后,血流动力学状态突然恶化为混合性心源性休克;心内膜心肌活检显示局部炎性浸润及心肌细胞坏死区域,病毒检测发现细小病毒B19(PVB19)呈阳性,因此患者接受了甲泼尼龙冲击治疗。基于低血压、低白蛋白血症和血液浓缩这一典型三联征的同时存在,我们怀疑系统性毛细血管渗漏综合征可能由PVB19感染伴急性心肌炎引发。临床病情因横纹肌溶解和急性肾损伤而进一步恶化:我们开始进行持续静静脉血液滤过,并添加细胞因子吸附器。在接下来的数小时内,我们观察到患者临床症状显著改善。患者1个月后出院,出院5个月后再次发作SCLS,此次无心肌受累且症状迅速缓解。

结论

系统性毛细血管渗漏综合征是一种潜在致命性疾病:因此有必要早期识别该疾病并及时启动支持性治疗,对于出现心源性休克体征以及低血压、低白蛋白血症和血液浓缩这一经典三联征的患者,急诊医生考虑到SCLS至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4176/9258032/fb0d14b672a0/ytac262ga1.jpg

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