Background: Endoscopic procedures are rarely performed in children with congenital heart disease (CHD); therefore, the associated complications are unknown. We report an abrupt change in circulatory and respiratory condition during endoscopic injection sclerotherapy for esophageal varices. Case presentation: A 9-year-old boy with a history of total anomalous pulmonary venous connection (TAPVC) repair and Fontan procedure for asplenia and a single ventricle with TAPVC underwent endoscopic injection sclerotherapy under general anesthesia for esophageal varices. Systolic blood pressure decreased from 70 to 50 mmHg following a sclerosant injection; a second injection reduced his peripheral oxygen saturation from 93 to 79% secondary to ventilation difficulty. Although we suspected anaphylaxis intraoperatively, postoperative imaging suggested that balloon dilation performed to prevent sclerosing agent leakage caused compression of the pulmonary venous chamber and trachea owing to the anomalous intrathoracic organ anatomy. Conclusion: Thorough understanding of the complex anatomy is important before performing endoscopic procedures in children with CHD to preoperatively anticipate possible intraoperative complications and select the optimal therapeutic approach and anesthesia management.
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