Aortic valve replacement in a 41-year-old woman with uncorrected tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries: a case report

• Published in: JA clinical reports Vol. 9; no. 1; p. 86
• Main Authors: Saito, Kazutomo, Iwasaki, Yudai, Tasaki, Takahiro, Saito, Hidehisa, Toyama, Hiroaki, Ejima, Yutaka, Yamauchi, Masanori
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 06.12.2023; Springer Nature B.V; SpringerOpen
• Subjects: Anesthesiology; Antibiotics; Aortic valve replacement; Blood; Cardiovascular disease; Case Report; Case reports; Catheters; Congenital diseases; Coronary vessels; Critical Care Medicine; Emergency Medicine; Endocarditis; General anesthesia; Heart rate; Heart surgery; Hemoglobin; Hospitals; Infectious endocarditis; Intensive; Major aortopulmonary collateral arteries; Medicine; Medicine & Public Health; Oxygen saturation; Pain Medicine; Patients; Pulmonary arteries; Tomography; Uncorrected tetralogy of Fallot; Vegetation; Veins & arteries; Weaning; Uncorrected tetralogy of Fallot; Aortic valve replacement; Infectious endocarditis; Major aortopulmonary collateral arteries
• ISSN: 2363-9024; 2363-9024
• DOI: 10.1186/s40981-023-00674-0

Background Tetralogy of Fallot (TOF) is a complex cyanotic congenital heart disease. As most patients with TOF undergo palliative or radical surgical repair during childhood, cardiac surgery under cardiopulmonary bypass (CPB) for adult survivors with unrepaired TOF is exceedingly rare. Case presentation A 41-year-old woman with unrepaired TOF, pulmonary atresia (PA), and major aortopulmonary collateral arteries (MAPCAs) developed acute infectious endocarditis (IE). As vegetation gradually increased despite intravenous antibiotic administration, she was scheduled for urgent aortic valve replacement under CPB. Pulmonary blood flow was primarily provided by the MAPCAs originating from the descending aorta. Intra-aortic balloon occlusion for MAPCAs was performed to ensure a bloodless surgical field. Aortic valve replacement was successful. Conclusion An adult with uncorrected TOF developed acute IE and subsequently had successful cardiac surgery under CPB. Understanding TOF physiology with PA and MAPCAs, particularly pulmonary blood flow through MAPCAs, is crucial.