Pendela Venkata Satish, Tan Bryan E-Xin, Chowdhury Medhat, Chow Ming
Internal Medicine, Rochester General Hospital, Rochester, USA.
Pulmonary and Critical Care Medicine, Rochester General Hospital, Rochester, USA.
Cureus. 2020 Jun 1;12(6):e8388. doi: 10.7759/cureus.8388.
Partial anomalous pulmonary venous return (PAPVR) is a congenital anomaly in which some of the pulmonary veins drain erroneously into the superior vena cava (SVC) or directly into the right atrium (RA). We present four cases of PAPVR presenting in adults. We discussed various presentations, diagnostic approaches and challenges in the management of these patients. Our first case had anomalous drainage from the right upper lobe of lung to SVC and was managed medically with riociguat and ambrisentan. The second patient had an unsuccessful attempt at repair of the anomalous vein. Our other two patients had right upper lobe veins draining into SVC. One of them had a successful surgical repair whereas the other patient declined surgery and is being monitored. In PAPVR patients, the decision for surgical repair depends on symptoms, shunt fraction, recurrent pulmonary infections, and concurrent indication for cardiac surgery.
部分性肺静脉异位回流（PAPVR）是一种先天性异常，其中部分肺静脉错误地引流至上腔静脉（SVC）或直接进入右心房（RA）。我们报告了4例成人PAPVR病例。我们讨论了这些患者的各种临床表现、诊断方法及治疗挑战。我们的首例病例是右肺上叶静脉异常引流至SVC，采用利奥西呱和安立生坦进行药物治疗。第二例患者修复异常静脉的尝试未成功。另外两名患者的右肺上叶静脉均引流至SVC。其中一名患者手术修复成功，而另一名患者拒绝手术，正在接受监测。对于PAPVR患者，手术修复的决策取决于症状、分流分数、反复肺部感染以及心脏手术的并发指征。
