Cardiology Department & Cardiovascular Research Centre (S.H., B.A., C.M., G.T.G., A.J.B., F.A., S.K.P., S.V.B.-N., M.A.G., R.H.M., D.J.P., C.I.).
Cardiovascular Magnetic Resonance Unit (S.H., B.A., C.M., G.H., G.T.G., S.K., S.V.B.-N., R.H.M., D.J.P., C.I.).
Circ Cardiovasc Imaging. 2021 Aug;14(8):e012371. doi: 10.1161/CIRCIMAGING.120.012371. Epub 2021 Aug 13.
Partial anomalous venous connections (PAPVC) are associated with left to right shunting and right heart dilatation. Identification of PAPVC has increased with widespread use of cross-sectional imaging modalities. However, management strategies are mostly based on expert opinion given the scarcity of data from large series. We aimed to define types and significance of isolated and atrial septal defect (ASD) associated PAPVC detected by cardiovascular magnetic resonance.
We retrospectively reviewed our cardiovascular magnetic resonance database from 2002 to 2018 to identify isolated or ASD-associated PAPVC cases.
A total of 215 patients (median age 46 years; range, 6-83) with isolated or ASD-associated PAPVC were identified among 102 135 clinical cardiovascular magnetic resonance studies. Of these, 104 were isolated and 111 were associated with an ASD. Anomalous connection of right upper pulmonary vein was the most common single venous anomaly (99/215), but in the isolated PAPVC group there were more anomalous left than right upper pulmonary veins (39 versus 34). The Qp/Qs was significantly higher for isolated anomalous single right upper pulmonary vein than left upper pulmonary vein (1.6 versus 1.4 respectively; =0.01) as were right ventricular end-diastolic volumes (113.7±30.9 versus 90 [57-157] mL/m, =0.004). In the PAPVC with an ASD group, sinus venosus ASDs (82%) were associated with right-sided PAPVCs while both right and left-sided venous anomalies were seen in secundum ASDs (18%). In a substantial number of patients (30 out of 91) with sinus venosus ASDs, PAPVCs were more complex and involved more than a single anomalous right upper pulmonary vein; and in 5 patients with ASD, PAPVC was identified only after the ASD closure.
This large series provides descriptive and hemodynamic features for isolated and ASD-associated PAPVCs. Anomalous isolated right upper pulmonary vein may cause a significant shunt (Qp/Qs >1.5). PAPVC associated with sinus venosus and secundum ASDs might be more complex than a single anomalous pulmonary vein and missed before ASD correction.
部分肺静脉异常连接（PAPVC）与左向右分流和右心扩张有关。随着横断面成像方式的广泛应用，PAPVC 的检出率有所增加。然而，由于缺乏大系列的数据，管理策略主要基于专家意见。我们旨在通过心血管磁共振定义孤立性和房间隔缺损（ASD）相关 PAPVC 的类型和意义。
我们回顾性分析了 2002 年至 2018 年期间心血管磁共振数据库，以确定孤立性或 ASD 相关 PAPVC 病例。
在 102515 例临床心血管磁共振研究中，共发现 215 例孤立性或 ASD 相关 PAPVC 患者（中位年龄 46 岁；范围，6-83 岁）。其中 104 例为孤立性 PAPVC，111 例与 ASD 相关。右上肺静脉异常连接是最常见的单一静脉异常（99/215），但在孤立性 PAPVC 组中，左、右上肺静脉异常的比例分别为 39 比 34。孤立性异常单支右上肺静脉的 Qp/Qs 明显高于左支（分别为 1.6 比 1.4；=0.01），右心室舒张末期容积也明显高于左支（分别为 113.7±30.9 比 90[57-157]ml/m；=0.004）。在 ASD 相关的 PAPVC 组中，窦房静脉 ASD（82%）与右侧 PAPVC 相关，而房间隔缺损 ASD 中同时存在右、左静脉异常（18%）。在相当数量的患者（30/91 例窦房静脉 ASD）中，PAPVC 更为复杂，涉及不止一支异常右上肺静脉；在 5 例 ASD 患者中，只有在 ASD 闭合后才发现 PAPVC。
本大规模系列研究提供了孤立性和 ASD 相关 PAPVC 的描述性和血流动力学特征。孤立性异常右肺静脉可能导致明显分流（Qp/Qs>1.5）。窦房静脉和房间隔缺损 ASD 相关的 PAPVC 可能比单一异常肺静脉更为复杂，在 ASD 矫正前可能会被遗漏。
