Wang Hong, Guan Hanxiong, Wang Dao Wen
Division of Cardiology and Department of Internal Medicine, Tongji Hospital, Tongji Medical College of Huazhong University of Science and Technology, 1905 Jiefang Dadao, Wuhan 430030, PR China.
BMC Cardiovasc Disord. 2014 Oct 25;14:149. doi: 10.1186/1471-2261-14-149.
Partial anomalous venous connection (PAPVC) is a rare congenital heart disease where the blood flow from one or more pulmonary veins (but not all) returns to the right atrium or systemic venous circulation and is often associated with a sinus venosus atrial defect (SVD). Transthoracic echocardiography (TTE) can provide limited information for this anomaly and the diagnosis of this congenital defect has been a clinical challenge.
We report here a case of a 75-year-old female with adult-onset pulmonary arterial hypertension (PAH), hypoxemia and right-sided chamber dilatation. The diagnosis of PAPVC was made incidentally by multidetector computed tomographic angiography (MCTA) that was performed to exclude pulmonary embolism. In this type of PAPVC, the atrial septum is intact, the right upper pulmonary vein (RUPV) connects to the superior vena cava (SVC), and the SVC overrides across the atrial septum and has bi-atrial connection, all of which are clearly manifested by MCTA.
This case indicates the need to exclude a PAPVC and SVD in unexplained pulmonary hypertension, and MCTA is a reliable non-invasive imaging technique with high resolution and wide anatomic coverage. The case also demonstrates that the coexisting SVD with PAPVC is an anomalous venous connection instead of atrial septal defect (ASD) and its key feature is the overriding of SVC or IVC across the intact atrial septum.
部分性肺静脉异位连接(PAPVC)是一种罕见的先天性心脏病,其中一条或多条(而非全部)肺静脉的血流回流至右心房或体静脉循环,且常与静脉窦型房间隔缺损(SVD)相关。经胸超声心动图(TTE)对这种异常情况提供的信息有限,而诊断这种先天性缺陷一直是一项临床挑战。
我们在此报告一例75岁女性患者,患有成人起病的肺动脉高压(PAH)、低氧血症和右心腔扩张。PAPVC的诊断是在进行多排螺旋计算机断层血管造影(MCTA)以排除肺栓塞时偶然发现的。在这种类型的PAPVC中,房间隔完整,右上肺静脉(RUPV)连接至上腔静脉(SVC),且SVC跨越房间隔并具有双心房连接,所有这些在MCTA上均清晰显示。
该病例表明,在不明原因的肺动脉高压中需要排除PAPVC和SVD,且MCTA是一种可靠的非侵入性成像技术,具有高分辨率和广泛的解剖覆盖范围。该病例还表明,PAPVC合并存在的SVD是一种肺静脉异位连接而非房间隔缺损(ASD),其关键特征是SVC或下腔静脉(IVC)跨越完整的房间隔。