Poster (III-P42)

[III-P42-06] 術中方針決定を要した巨大上大静脈を伴う総肺静脈還流異常症の乳児例

^○片山 雄三¹, 小澤 司¹, 塩野 則次¹, 渡邉 善則¹, 池原 聡², 高月 晋一², 中山 智孝², 松裏 裕行², 佐地 勉², 日根 幸太郎³, 与田 仁志³ (1.東邦大学医療センター大森病院 心臓血管外科, 2.東邦大学医療センター大森病院 小児循環器科, 3.東邦大学医療センター大森病院 新生児科)

Keywords:surgery, total anomalous pulmonary vein connection, pulmonary vein obstruction

We report a case of an 11-month old girl with TAPVC. The patient was referred to our institution for the examination of cyanosis and heart murmur. A grade 2/6 systolic ejection murmur was heard in the upper left parasternal area. A chest X-ray showed the marked protrusion of the right first arch. Cardiac echocardiography revealed the volume overload in right heart, PFO, mild pulmonary valve stenosis without systolic doming, and common chamber drainage to lower SVC. MDCT showed vertical vein was very short, connected to dilated and lower SVC, and arose from the tilted common chamber close to right PVs. Prior to surgery, we were not able to know whether right upper PV was connected to common chamber or singly to SVC.After the median sternotomy, elective CPB was instituted with PA venting. Common chamber was wide enough to anastomose with LA. Right PVs were convergent and connected to common chamber, but vertical vein was too short and wide to ligate. An extended right atriotomy revealed PFO and an opening to common chamber. Through this opening, we were able to find the route to either right PV. On these findings, we had two options; intra-cardiac rerouting or extra-cardiac anastomosis via superior approach. In this case, we chose the latter using the boat-shaped ePTFE patch to close the opening.After the operation, MDCT showed adequate anastomosis between common chamber and LA without PV stenosis. Echocardiography revealed a significant drop in the pulmonary valve gradient, and heart murmur was disappeared.