Center for the Diagnosis and Treatment of Pericardial Diseases,Section of Cardiovascular Imaging,Department of Cardiovascular Medicine, Heart Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio, USA.
Department of Cardiovascuar Medicine, MetroHealth Medical Center, Cleveland, Ohio, USA.
Heart. 2023 Aug 11;109(17):1281-1285. doi: 10.1136/heartjnl-2022-321798.
Chylopericardium (CPE) is a rare condition associated with accumulation of triglyceride-rich chylous fluid in the pericardial cavity. Due to minimal information on CPE within the literature, we conducted a systematic review of all published CPE cases to understand its clinical characteristics, management and outcomes.
We performed a literature search and identified cases of patients with CPE from 1946 until May 2021 in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. We identified relevant articles for pooled analyses of clinical, diagnostic and outcome data.
A total of 95 articles with 98 patients were identified. Patient demographics demonstrated male predominance (55%), with a mean age of 37±15 years. Time from symptom onset to diagnosis was 5 (Q1 4.5, Q3 14) days, with 74% of patients symptomatic on presentation. Idiopathic CPE (60%) was the most common aetiology. Cardiac tamponade secondary to CPE was seen in 38% of cases. Pericardial fluid analysis was required in 94% of cases. Lymphangiography identified the leakage site in 59% of patients. Medical therapy (total parenteral nutrition, medium-chain triglycerides or octreotide) was undertaken in 63% of cases. In our cohort, 32% progressed towards surgical intervention. During a median follow-up of 180 (Q1 180, Q3 377) days, CPE recurred in 16% of cases. Of the patients with recurrence, 10% were rehospitalised.
CPE tends to develop in younger patients and may cause serious complications. Many patients fail medical therapy, thereby requiring surgical intervention. Although overall mortality is low, associated morbidities warrant close follow-up and possible reintervention and hospitalisations.
乳糜性心包积液(CPE)是一种罕见的疾病,其特征是在心包腔内积聚富含甘油三酯的乳糜液。由于文献中关于 CPE 的信息很少,我们对所有已发表的 CPE 病例进行了系统回顾,以了解其临床特征、处理方法和结局。
我们按照系统评价和荟萃分析的首选报告项目的指南进行了文献检索,并确定了 1946 年至 2021 年 5 月期间患有 CPE 的患者病例。我们确定了相关文章,以便对临床、诊断和结局数据进行汇总分析。
共确定了 95 篇文章,其中有 98 例患者。患者的人口统计学特征显示男性居多(55%),平均年龄为 37±15 岁。从症状出现到诊断的时间为 5 天(Q14.5,Q314),74%的患者在就诊时出现症状。特发性 CPE(60%)是最常见的病因。38%的病例发生 CPE 导致的心包填塞。94%的病例需要进行心包液分析。59%的患者行淋巴管造影术以确定渗漏部位。63%的病例接受了药物治疗(全胃肠外营养、中链甘油三酯或奥曲肽)。在我们的队列中,32%的患者进展为手术干预。在中位随访 180 天(Q1180,Q3377)期间,16%的病例 CPE 复发。在复发的患者中,有 10%需要再次住院。
CPE 倾向于在年轻患者中发生,并且可能导致严重的并发症。许多患者药物治疗无效,因此需要手术干预。尽管总体死亡率较低,但相关的发病率需要密切随访,可能需要再次干预和住院治疗。