Background: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.Methods:We retrospectively reviewed our cardiovascular magnetic resonance database from 2002 to 2018 to identify isolated or ASD-associated PAPVC cases.Results: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; P=0.01) as were right ventricular end-diastolic volumes (113.7±30.9 versus 90 [57–157] mL/m², P=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.Conclusions: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.

中文翻译：

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心血管磁共振确定的部分异常肺静脉连接（孤立性和房间隔缺损相关）的临床意义


背景：部分异常静脉连接（PAPVC）与左向右分流和右心扩张有关。随着横截面成像方式的广泛使用，PAPVC 的识别率有所增加。然而，由于缺乏大规模系列数据，管理策略大多基于专家意见。我们的目的是定义心血管磁共振检测到的孤立性和房间隔缺损（ASD）相关的 PAPVC 的类型和意义。方法：我们回顾性回顾了 2002 年至 2018 年的心血管磁共振数据库，以识别孤立性或 ASD 相关的 PAPVC 病例。结果：在 102 135 项临床心血管磁共振研究中，共鉴定出 215 名患有孤立性或 ASD 相关 PAPVC 的患者（中位年龄 46 岁；范围为 6-83 岁）。其中，104 例被隔离，111 例与 ASD 相关。右上肺静脉异常连接是最常见的单静脉异常（99/215），但在孤立的 PAPVC 组中，左上肺静脉异常多于右上肺静脉（39 比 34）。孤立性异常单右上肺静脉的 Qp/Qs 显着高于左上肺静脉（分别为 1.6 与 1.4； P = 0.01），右心室舒张末期容积也是如此（113.7±30.9 与 90 [57–157] mL） /m ² ， P =0.004）。在伴有 ASD 的 PAPVC 组中，窦静脉 ASD（82%）与右侧 PAPVC 相关，而继发孔 ASD 中可见右侧和左侧静脉异常（18%）。 在大量患有静脉窦 ASD 的患者（91 名中的 30 名）中，PAPVC 更为复杂，并且涉及多个异常右上肺静脉；在 5 名 ASD 患者中，仅在 ASD 闭合后才识别出 PAPVC。结论：这个大系列提供了孤立的和 ASD 相关的 PAPVC 的描述性和血流动力学特征。异常孤立的右​​上肺静脉可能导致显着分流（Qp/Qs >1.5）。与静脉窦和继发孔 ASD 相关的 PAPVC 可能比单个异常肺静脉更复杂，并且在 ASD 校正之前被遗漏。