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一例系统性红斑狼疮合并严重肺动脉高压的病例报告,表现为大量心包积液并伴有心脏压塞早期征象:诊断与治疗挑战

A case report of systemic lupus erythematosus with severe pulmonary hypertension presenting as large pericardial effusion with early signs of cardiac tamponade: a diagnostic and therapeutic challenge.

作者信息

Alghamdi Abdullah Ibrahim, Shah Muhammad Azam, Alkhodair Abdullah Mohammed

机构信息

College of Medicine, King Saud University, King Khalid Road, Riyadh, Saudi Arabia.

King Fahad Medical City, Dabab Street, Sulaimaniya, PO Box 221124, 11311 Riyadh, Saudi Arabia.

出版信息

Eur Heart J Case Rep. 2024 Sep 20;8(10):ytae521. doi: 10.1093/ehjcr/ytae521. eCollection 2024 Oct.

DOI:10.1093/ehjcr/ytae521
PMID:39430677
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11487483/
Abstract

BACKGROUND

Pulmonary hypertension is defined as resting arterial pressure >20 mmHg. Cardiac tamponade is a medical emergency where fluids accumulate in the pericardial sac compressing the heart pericardium leading to heart failure. Pericardiocentesis is challenging in patients with cardiac tamponade and severe pulmonary hypertension due to the risk of catastrophic haemodynamic collapse.

CASE SUMMARY

An 18-year-old female who was recently diagnosed to have systemic lupus erythematosus (SLE) presented to the emergency department with shortness of breath, chest pain, fever, and fatigue. The physical examination revealed tachycardia, muffled heart sounds, and distended jugular venous pulse. Chest X-ray showed cardiomegaly, and transthoracic echocardiography showed a large circumferential pericardial effusion with signs of cardiac tamponade. There was severe pulmonary hypertension along with a dilated right ventricle with systolic dysfunction. The right ventricular systolic pressure was around 100 mmHg. The multidisciplinary team of cardiologists and pulmonologists decided to avoid pericardiocentesis due to the high risk of haemodynamic collapse. Aggressive medical therapy targeting pulmonary hypertension and SLE was started, which resulted in complete resolution of the pericardial effusion and normalization of pulmonary artery pressure.

DISCUSSION

A conservative approach can be an alternative strategy to manage patients with large pericardial effusion and impending pericardial tamponade in the presence of severe pulmonary arterial hypertension as pericardiocentesis carries a high risk of haemodynamic collapse.

摘要

背景

肺动脉高压定义为静息动脉压>20 mmHg。心脏压塞是一种医疗急症,即液体在心包腔内积聚,压迫心脏心包,导致心力衰竭。对于患有心脏压塞和严重肺动脉高压的患者,心包穿刺术具有挑战性,因为存在发生灾难性血流动力学崩溃的风险。

病例摘要

一名18岁女性,近期被诊断为系统性红斑狼疮(SLE),因呼吸急促、胸痛、发热和疲劳就诊于急诊科。体格检查发现心动过速、心音减弱和颈静脉怒张。胸部X线显示心脏扩大,经胸超声心动图显示大量心包积液并有心脏压塞的迹象。存在严重肺动脉高压,同时右心室扩张并伴有收缩功能障碍。右心室收缩压约为100 mmHg。心脏病专家和肺病专家的多学科团队决定避免进行心包穿刺术,因为血流动力学崩溃风险高。开始针对肺动脉高压和SLE进行积极的药物治疗,结果心包积液完全消退,肺动脉压恢复正常。

讨论

在存在严重肺动脉高压的情况下,对于有大量心包积液且即将发生心包压塞的患者,保守治疗可以作为一种替代策略,因为心包穿刺术具有较高的血流动力学崩溃风险。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b82/11487483/192523611ccc/ytae521f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b82/11487483/d8f5f0fa7409/ytae521il2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b82/11487483/bb9252adade4/ytae521f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b82/11487483/de2ebcbf2937/ytae521f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b82/11487483/d5a1a6075bd6/ytae521f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b82/11487483/192523611ccc/ytae521f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b82/11487483/d8f5f0fa7409/ytae521il2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b82/11487483/bb9252adade4/ytae521f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b82/11487483/de2ebcbf2937/ytae521f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b82/11487483/d5a1a6075bd6/ytae521f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b82/11487483/192523611ccc/ytae521f4.jpg

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本文引用的文献

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Pericardiocentesis induced right ventricular changes in patients with and without pulmonary hypertension.心包穿刺术可引起肺动脉高压患者和无肺动脉高压患者的右心室变化。
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Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies.系统性红斑狼疮与肺动脉高压:关联、风险及管理策略
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