Department of Cardiovascular Surgery, Numata Neurosurgery Heart-Disease Hospital, 8 Sakae-cho, Numata, 378-0014, Japan.
Department of Cardiovascular Surgery, Chiba Children's Hospital, Chiba, Japan.
J Cardiothorac Surg. 2020 Sep 25;15(1):269. doi: 10.1186/s13019-020-01313-w.
Partial anomalous pulmonary venous connection draining into the right atrium with an intact atrial septum is a very rare clinical entity in the adult population. Partial anomalous pulmonary venous connection must be suspected as a differential diagnosis when the cause of right heart enlargement and pulmonary artery hypertension is unknown.
This study describes the surgical case of an isolated right partial anomalous pulmonary venous connection to the right atrium in a 68-year-old woman, who underwent tricuspid ring annuloplasty and right-sided maze procedure simultaneously. She had complaints of gradually progressing dyspnea on exertion. However, a diagnosis could not be established despite consultations at multiple hospitals for over a year. Right heart catheterization revealed severe pulmonary artery hypertension with a mean pulmonary artery pressure of 46 mmHg, step-up phenomenon of oxygen saturation at the mid-level of the right atrium with a pulmonary-to-systemic blood flow ratio of 2.4, and a pulmonary vascular resistance of 3.1 Wood Units. As medical treatment with pulmonary artery vasodilator therapy did not improve her symptoms, she underwent surgical repair. An atrial septal defect was created surgically with a curvilinear tongue-shaped cut. The right anomalous pulmonary veins were rerouted through the surgically created atrial septal defect into the left atrium with a baffle comprised of the interatrial septum flap, kept in continuity with the anterior margin and sutured while mobilizing the enlarged right atrium. The patient had an uneventful postoperative course and remains asymptomatic.
The described surgical technique could be considered an effective alternative for patients undergoing surgical repair for a partial anomalous pulmonary venous connection isolated to the right atrium. The indication for surgery must be judged on a case-by-case basis in these patients with prevalent systemic-to-pulmonary shunting.
部分肺静脉异常连接并流入右心房，房间隔完整，这种情况在成年人群中非常罕见。当不明原因导致右心增大和肺动脉高压时，必须将部分肺静脉异常连接作为鉴别诊断。
本研究描述了一名 68 岁女性孤立性右部分肺静脉异常连接至右心房的手术病例，该患者同时接受了三尖瓣环成形术和右侧迷宫手术。她逐渐出现劳力性呼吸困难的症状。尽管在多家医院就诊超过一年，但仍无法明确诊断。右心导管检查显示严重肺动脉高压，平均肺动脉压为 46mmHg，中右心房血氧饱和度出现阶梯上升现象，肺血流量与体循环血流量比值为 2.4，肺血管阻力为 3.1 伍德单位。尽管肺动脉血管扩张剂治疗并未改善她的症状，但她仍接受了手术修复。手术中创建了一个房间隔缺损，并用曲线形舌形切口。将右异常肺静脉通过手术创建的房间隔缺损重新引导至左心房，使用由房间隔瓣组成的挡板，与扩大的右心房的前缘保持连续性，并在移动右心房时进行缝合。患者术后恢复顺利，无任何症状。
对于孤立性右心房部分肺静脉异常连接的患者，所描述的手术技术可以被认为是一种有效的替代方法。对于这些存在系统性至肺分流的患者，手术指征必须根据具体情况进行判断。
