Patients who have undergone an atrial switch operation for dextro-transposition of the great arteries (dTGA) sometimes suffer from right ventricular dysfunction, tricuspid regurgitation, arrhythmias, or baffle leaks. We report the first case of single-stage arterial switch operation conversion in an adult patient with dTGA and pulmonary arterial hypertension (PAH) due to a baffle leak after a Senning procedure. Perioperative mechanical circulatory support was required for 3 weeks. Although chronic kidney disease persisted, her left ventricular function and pulmonary hypertension improved over the first postoperative year. This is a rare case, and we believe that the patient's PAH might have helped the left ventricle withstand systemic pumping for over two decades.
Learning objective: Patients with dextro-transposition of the great arteries may face problems after an atrial switch operation because their right ventricle is responsible for systemic pumping. Staged arterial switch operation conversion is a strategy that can combat these problems but increases the risk of perioperative mortality. Pulmonary arterial hypertension allows for single-stage arterial switch operation, but the operative risk may be higher.
Keywords: Arterial switch operation; Arterial switch operation conversion; Baffle leak; Congenital heart disease; Dextro-looped transposition of the great arteries; Pulmonary arterial hypertension.
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