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Background Limited research has been conducted on the surgical management of the aortic valve in congenitally corrected transposition of great arteries (ccTGA) and to our knowledge there have been no reports on the treatment of bicuspid aortic regurgitation (AR) in ccTGA. We report on a ccTGA patient with bicuspid AR and systemic right ventricule (SRV) dysfunction who underwent transcatheter aortic valve replacement (TAVR). Case summary A 17-year-old male with a history of ccTGA and cerebral palsy diagnosed at birth presented with heart failure. During childhood, he did not experience any heart failure symptoms, however, secondary to progressive bicuspid AR he experienced worsening SRV dysfunction beginning at 15-year-old. Echocardiography showed reduced SRV ejection fraction and severe bicuspid AR. The heart team, including a cardiac surgeon and paediatric cardiologist, discussed the treatment strategies and decided to proceed with TAVR as surgical aortic valve replacement was deemed high risk. TAVR was performed with the 34 mm Evolut R (Medtronic, Minneapolis, MN, USA). Post-operative echocardiography showed severe paravalvular leak (PVL). Therefore, valve-in-valve TAVR using a 29 mm Edwards SAPIEN 3 (Edwards Lifesciences, Irvine, CA, USA) was performed on post-operative Day 2 for PVL reduction. Following second procedure, PVL was significantly improved. The patient was discharged in stable condition. Discussion This is the first case wherein TAVR was performed for bicuspid AR in a patient with ccTGA. With appropriate preparation and planning and a collaborative multi-disciplinary team approach, TAVR can be a treatment option for severe AR in patients with ccTGA at high risk for surgery.

european heart journal. case reports

Transcatheter aortic valve replacement for bicuspid aortic valve regurgitation in a 17-year-old patient with congenitally corrected transposition of great arteries: a case report