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

Kazutomo Saito; Yudai Iwasaki; Takahiro Tasaki; Hidehisa Saito; Hiroaki Toyama; Yutaka Ejima; Masanori Yamauchi

doi:10.1186/s40981-023-00674-0

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

JA Clin Rep. 2023 Dec 6;9(1):86. doi: 10.1186/s40981-023-00674-0.

Authors

Kazutomo Saito¹, Yudai Iwasaki², Takahiro Tasaki³, Hidehisa Saito³, Hiroaki Toyama², Yutaka Ejima⁴, Masanori Yamauchi²

Affiliations

¹ Anesthesiology and Perioperative Medicine, Tohoku University Graduate School of Medicine, 2-1 Seiryomachi, Aoba-Ku, Sendai, Miyagi, 980-8575, Japan. kazutomo0815@gmail.com.
² Anesthesiology and Perioperative Medicine, Tohoku University Graduate School of Medicine, 2-1 Seiryomachi, Aoba-Ku, Sendai, Miyagi, 980-8575, Japan.
³ Department of Anesthesiology, Tohoku University Hospital, 1-1 Seiryomachi, Aoba-Ku, Sendai, Miyagi, 980-8575, Japan.
⁴ Division of Surgical Center and Supply, Sterilization, Tohoku University Hospital, 1-1 Seiryomachi, Aoba-Ku, Sendai, Miyagi, 980-8575, Japan.

Abstract

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.

Keywords: Aortic valve replacement; Infectious endocarditis; Major aortopulmonary collateral arteries; Uncorrected tetralogy of Fallot.