部分肺静脉连接异常:诊断、处理和结果

Fatma Sengul, Perver Arslan, Ensar Duras, Pelin Ayyildiz, Hacer Kamali, Okan Yildiz, smihan Onan, Alper Guzeltas
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引用次数: 0

摘要

目的:部分肺静脉连接异常(PAPVC)是一种罕见的先天性心血管异常,其特征是一条或多条肺静脉流入右心房,但不是全部。这种情况可以单独发生,也可以与其他心脏异常(如房间隔缺损(ASD))一起发生。在这项研究中,我们旨在探讨PAPVC患者的临床表现、诊断方法、管理选择和结果。材料和方法:本回顾性研究纳入了2010年1月至2022年12月期间诊断为孤立性或asd相关PAPVC的140例患者。结果:患者的中位年龄为5岁(1个月至39岁)。孤立性房间隔缺损36例(25.7%),房间隔缺损104例(74.3%)。在我们的患者队列中,119例患者(85%)表现为部分受影响的异常pv,而其余21例患者(15%)表现为累及整个肺的异常肺静脉。右侧pv 116例(82.8%),左侧pv 20例(14.3%),右侧和左侧pv均有4例(2.9%)。原隔错位8例(5.8%),出现弯刀综合征7例(5%)。71.4% (n=100)的患者行手术治疗,2例双引流患者行导管血管造影合并垂直静脉闭塞。结论:尽管PAPVC罕见且复杂,但及时识别和定制手术干预有助于避免严重并发症的发生。先进的影像技术在诊断和手术计划中的应用是至关重要的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Partial anomalous pulmonary venous connection: diagnosis, management, and outcomes
Aim: Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital cardiovascular anomaly characterized by one or more, but not all, pulmonary veins draining into the right atrium. This condition can occur in isolation or in conjunction with other cardiac abnormalities such as an atrial septal defect (ASD). In this study we aimed to investigate the clinical presentation, diagnostic approaches, management options, and outcomes of patients with PAPVC. Materials and methods: This retrospective study included 140 patients diagnosed with isolated or ASD-associated PAPVC between January 2010 and December 2022. Results: The median age at presentation was 5 years (1 month to 39 years). Isolated PAPVC was observed in 36 patients (25.7%), while 104 patients (74.3%) had an atrial septal defect (ASD). In our patient cohort, 119 patients (85%) exhibited partially affected anomalous PVs, while the remaining 21 patients (15%) presented with anomalous pulmonary veins involving the entire lung. Right-sided PVs were involved in 116 patients (82.8%), left-sided PVs in 20 patients (14.3%), and both right and left PVs in 4 patients (2.9%). Malposition of primum septum was detected in 8 patients (5.8%), and scimitar syndrome was present in 7 patients (5%). Surgical treatment was performed in 71.4% (n=100) of the patients, while catheter angiography with vertical vein occlusion was performed in 2 patients with dual drainage. Conclusion: Despite the infrequency and complexity of PAPVC, timely identification and customized surgical intervention can help avert serious complications. The application of advanced imaging technologies in diagnosis and surgical planning is crucial.
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