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

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.

Multiple Major Aortopulmonary Collateral Arteries With Tetralogy of Fallot and Other Congenital Cardiac Disorders Detected in the Fourth Decade: A Report of a Rare Case

“…Tetralogy of Fallot (TOF) is a common cyanotic type of congenital heart disease (CHD) that consists of ventricular septal defect (VSD), right ventricular hypertrophy, and right ventricular outflow tract obstruction, overriding of the aorta [1]. Pulmonary atresia (PA) is a rare variant of TOF, and major aortopulmonary collateral arteries (MAPCAs) are the main dependable circulatory adjuvants for pulmonary circulation in patients with PA and TOF.…”

“…Pulmonary atresia (PA) is a rare variant of TOF, and major aortopulmonary collateral arteries (MAPCAs) are the main dependable circulatory adjuvants for pulmonary circulation in patients with PA and TOF. The combination of MAPCAs with TOF and PA is the most extreme type of TOF [1]. The 22q11.2 deletion (del22q11), also known as DiGeorge syndrome, occurs in approximately 13% of cases with TOF.…”