Ito Takahiro, Hagino Ikuo, Aoki Mitsuru, Umezu Kentaro, Saito Tomohiro, Suzuki Akiyo
Department of Cardiovascular Surgrey, Chiba Children's Hospital, 579-1 Heta, Midori, Chiba-city, Chiba, 266-0007, Japan.
J Cardiothorac Surg. 2021 Nov 21;16(1):337. doi: 10.1186/s13019-021-01722-5.
Total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome is extremely rare.
We present a case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome in a patient who was diagnosed based on transthoracic echocardiography and computed tomography. We observed complete absence of the lung, the bronchial tree, and vascular structures on the right side, with abnormal drainage of the left pulmonary veins into the innominate vein. The patient showed clear clinical evidence of pulmonary venous obstruction and underwent surgery 3 days after birth. The pulmonary venous chamber containing the vertical vein was anastomosed to the left atrium using 7-0 PDS running sutures via a median sternotomy. Echocardiography and computed tomography performed 1 year postoperatively revealed no pulmonary venous obstruction.
We report a rare case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome, which was successfully repaired 3 days after birth. A median sternotomy is a safe and effective approach for surgical repair of congenital heart disease with unilateral lung agenesis. Repair of the supra cardiac total anomalous pulmonary connection using the vertical vein is feasible in patients with a small pulmonary venous chamber.
完全性肺静脉异位连接合并单侧肺不发育和Goldenhar综合征极为罕见。
我们报告一例完全性肺静脉异位连接合并单侧肺不发育和Goldenhar综合征的病例，该患者通过经胸超声心动图和计算机断层扫描确诊。我们观察到右侧肺、支气管树和血管结构完全缺失，左肺静脉异常引流至无名静脉。该患者有明确的肺静脉梗阻临床证据，并在出生后3天接受了手术。通过正中胸骨切开术，使用7-0 PDS连续缝线将包含垂直静脉的肺静脉腔与左心房吻合。术后1年进行的超声心动图和计算机断层扫描显示无肺静脉梗阻。
我们报告了一例罕见的完全性肺静脉异位连接合并单侧肺不发育和Goldenhar综合征的病例，该病例在出生后3天成功修复。正中胸骨切开术是手术修复合并单侧肺不发育的先天性心脏病的一种安全有效的方法。对于肺静脉腔较小的患者，使用垂直静脉修复心上型完全性肺静脉异位连接是可行的。
