部分肺静脉异常回流:进行性肺动脉高压患者的诊断挑战和成功纠正。

IF 0.6 Q3 MEDICINE, GENERAL & INTERNAL
Miguel Antonio Belaunzaran, Samuel Koenigsberg, Akash Mathavan, Akshay Mathavan
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引用次数: 0

摘要

部分肺静脉回流异常(PAPVR)是一种罕见的先天性异常,其中一条或多条肺静脉流入全身静脉系统,造成左向右分流并可能导致右侧容量过载。PAPVR常与房间隔缺损相关,可表现为非特异性症状,如用力性呼吸困难,常被误诊为其他原因的肺动脉高压。我们描述了一个妇女进行性用力呼吸困难和下肢水肿在新确定的毛细血管前肺动脉高压的设置。影像学显示右心房严重扩大,右上肺静脉从左向右分流流入上腔静脉,符合PAPVR。她接受了成功的手术矫正,功能恢复。本病例强调了识别原因不明的右心房扩大作为PAPVR线索的重要性,并证明了及时手术干预后症状和生理解决的可能性。
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 return: diagnostic challenges and successful correction in a patient with progressive pulmonary hypertension.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more pulmonary veins drain into the systemic venous system, creating a left-to-right shunt and potentially causing right-sided volume overload. Frequently associated with atrial septal defects, PAPVR may present with non-specific symptoms such as exertional dyspnoea and is often misdiagnosed as pulmonary hypertension of other origins. We describe a woman with progressive exertional dyspnoea and lower extremity oedema in the setting of newly identified precapillary pulmonary hypertension. Imaging revealed severe right atrial enlargement and a left-to-right shunt from the right superior pulmonary vein draining into the superior vena cava, consistent with PAPVR. She underwent successful surgical correction with the Warden procedure resulting in functional recovery. This case highlights the importance of recognising unexplained right atrial enlargement as a clue to PAPVR and demonstrates the potential for symptomatic and physiological resolution following timely surgical intervention.

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来源期刊
BMJ Case Reports
BMJ Case Reports Medicine-Medicine (all)
CiteScore
1.40
自引率
0.00%
发文量
1588
期刊介绍: BMJ Case Reports is an important educational resource offering a high volume of cases in all disciplines so that healthcare professionals, researchers and others can easily find clinically important information on common and rare conditions. All articles are peer reviewed and copy edited before publication. BMJ Case Reports is not an edition or supplement of the BMJ.
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