日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Mixed-type total anomalous pulmonary venous connection (TAPVC) with univentricular circulation has a wide variation of pulmonary venous anatomy and drainage. Surgical intervention for mixed-type TAPVC, including management of the vertical vein, remains controversial. Herein, we report a rare case of symptomatic portosystemic shunt via the unligated vertical vein following repair of the mixed-type TAPVC. An 18-day-old male neonate with a mixed-type TAPVC and asplenia underwent surgical repair of the TAPVC. The right vertical vein draining to the superior vena cava, and the left vertical vein draining to the portal vein were left patent. Elevated liver enzymes, coagulation abnormalities, hyperammonemia, and hypoglycemia were detected following surgery. Echocardiography revealed a portosystemic shunt draining from the portal vein to the left-sided atrium via the left unligated vertical vein. Elevated atrial pressure was detected on cardiac catheterization, and as a result, we performed banding of the left unligated vertical vein. This case highlights the need to discuss the portosystemic shunt as a postoperative complications in mixed-type TAPVC with univentricular circulation when considering both the surgical procedure and postoperative management.