Diagnosis of sinus venosus defects, not infrequently associated with complex anomalous pulmonary venous drainage, may be delayed requiring multimodality imaging.

Retrospective review of all patients from February 2008 to January 2019.

Thirty-seven children were diagnosed at a median age of 4.2 years (range 0.5−15.5 years). In 32 of 37 (86%) patients, diagnosis was achieved on transthoracic echocardiography, but five patients (14%) had complex variants (four had high insertion of anomalous vein into the superior caval vein and three had multiple anomalous veins draining to different sites, two of whom had drainage of one vein into the high superior caval vein). In these five patients, the final diagnosis was achieved by multimodality imaging and intra-operative findings. The median age at surgery was 5.2 years (range 1.6−15.8 years). Thirty-one patients underwent double patch repair, four patients a Warden repair, and two patients a single-patch repair. Of the four Warden repairs, two patients had a high insertion of right-sided anomalous pulmonary vein into the superior caval vein, one patient had bilateral superior caval veins, and one patient had right lower pulmonary vein insertion into the right atrium/superior caval vein junction. There was no post-operative mortality, reoperation, residual shunt or pulmonary venous obstruction. One patient developed superior caval vein obstruction and one patient developed atrial flutter.

Complementary cardiac imaging modalities improve diagnosis of complex sinus venosus defects associated with a wide variation in the pattern of anomalous pulmonary venous connection. Nonetheless, surgical treatment is associated with excellent outcomes.

静脉窦缺损的诊断，通常与复杂的异常肺静脉引流相关，可能会延迟，需要多模态成像。

对 2008 年 2 月至 2019 年 1 月的所有患者进行回顾性分析。

37 名儿童的中位年龄为 4.2 岁（范围 0.5-15.5 岁）。37 名患者中有 32 名 (86%) 经胸超声心动图诊断，但 5 名患者 (14%) 有复杂变异（4 名异常静脉高度插入上腔静脉，3 名有多条异常静脉引流至不同部位，其中两人曾将一根静脉引流至高位上腔静脉）。在这五名患者中，最终诊断是通过多模态成像和术中发现实现的。手术的中位年龄为 5.2 岁（范围 1.6-15.8 岁）。31 名患者接受了双补丁修复，4 名患者接受了 Warden 修复，2 名患者接受了单补丁修复。在四次 Warden 维修中，2例患者右侧异常肺静脉高位插入上腔静脉，1例患者双侧上腔静脉，1例患者右下肺静脉插入右心房/上腔静脉交界处。无术后死亡、再手术、残余分流或肺静脉阻塞。一名患者出现上腔静脉阻塞，一名患者出现心房扑动。

补充心脏成像方式改善了与异常肺静脉连接模式的广泛变化相关的复杂静脉窦缺损的诊断。尽管如此，手术治疗与良好的结果相关。