全腔隙肺连接后的蛋白质丢失性肠病和可塑性支气管炎。

IF 1.1 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS
Veronika Hammer, Thibault Schaeffer, Helena Staehler, Paul Philipp Heinisch, Melchior Burri, Nicole Piber, Julia Lemmer, Alfred Hager, Peter Ewert, Jürgen Hörer, Masamichi Ono
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引用次数: 0

摘要

背景:我们旨在评估全腔肺动脉连接(TCPC)队列中蛋白质丢失性肠病(PLE)和可塑性支气管炎(PB)的发病率、结果和预测因素。方法:我们纳入了1994年至2021年间连续620名接受TCPC的患者。对PLE/PB的发病率和预测因素进行了评估。检查PLE/PB发作后的死亡和心脏移植情况。结果:共有41例患者出现PLE/PB(31例PLE,15例PB,5例同时发生PLE和PB)。他们在TCPC时的中位年龄为2.2(四分位间距[IQRs],1.7-3.7)年,在TCPC后PLE的发病时间为2.6(IQR:1.0-6.6)年,PB的发病时间则为1.1(IQR:0.3-4.1)年。发生PLE/PB的独立因素是显性右心室(RV,危险比[HR],2.243;95%置信区间[CI],1.129-4.458,P = .021)和TCPC后持续胸腔积液(HR,2.101;95%CI,1.090-4.049,P = .027)。在PLE/PB人群中,5年和10年诊断为PLE/PB后免于死亡或移植的比例分别为88.7%和76.4%。对10名患者进行了11次手术干预,包括房室瓣修复(n = 4) ,Fontan通路修订(n = 2) ,起搏器植入(n = 2) ,二次开窗(n = 1) ,膈襞(n = 1) 和心室辅助装置植入(n = 1) 。在9名患者中,PLE症状得到缓解,蛋白质水平正常,从而实现了PLE的康复。8名患者死亡,其余患者的蛋白质损失仍具有挑战性。结论:蛋白质丢失性肠病和PB仍然是TCPC队列中的严重并发症。具有显性RV和长期胸腔积液的患者有发生PLE/PB的风险。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Protein-Losing Enteropathy and Plastic Bronchitis Following the Total Cavopulmonary Connections.

Background: We aimed to evaluate incidence, outcomes, and predictors of protein-losing enteropathy (PLE) and plastic bronchitis (PB) in a cohort of total cavopulmonary connection (TCPC).

Methods: We included 620 consecutive patients undergoing TCPC between 1994 and 2021. Prevalence and predictors for onset of PLE/PB were evaluated. Death and heart transplantation after onset of PLE/PB were examined.

Results: A total of 41 patients presented with PLE/PB (31 with PLE, 15 with PB, and 5 developed both PLE and PB). Their median age at TCPC was 2.2 (interquartile ranges [IQRs], 1.7-3.7) years, and time period to onset for PLE was 2.6 (IQR: 1.0-6.6) years and for PB was 1.1 (IQR: 0.3-4.1) years after TCPC. Independent factors for developing PLE/PB were dominant right ventricle (RV, hazard ratio [HR], 2.243; 95% confidence interval [CI], 1.129-4.458, P = .021) and prolonged pleural effusion after TCPC (HR, 2.101; 95% CI, 1.090-4.049, P = .027). In PLE/PB population, freedom from death or transplantation after PLE/PB diagnosis at 5 and 10 years were 88.7% and 76.4%, respectively. Eleven surgical interventions were performed in 10 patients, comprising atrioventricular valve repairs (n = 4), Fontan pathway revisions (n = 2), pacemaker implantation (n = 2), secondary fenestration (n = 1), diaphragm plication (n = 1), and ventricular assist device implantation (n = 1). In nine patients, a recovery from PLE with the resolution of PLE symptoms and normal protein levels was achieved. Eight patients died and the remaining continued to have challenging protein loss.

Conclusions: Protein-losing enteropathy and PB remain severe complications in the cohort of TCPC. Patients with dominant RV, and prolonged pleural effusions, were at risk for PLE/PB.

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CiteScore
1.80
自引率
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