In patients with a congenitally corrected transposition of the great arteries (ccTGA), the regurgitation of the systemic atrioventricular valve (SAVV) increases and anatomical right ventricular (ARV) dysfunction often progressively develops. A low systemic ventricular ejection fraction( SVEF) is a risk factor for mortality. However, in patients with a low ejection fraction of ARV, it is unclear how to best perform valve replacement for patients with regurgitation of SAVV. A 70-year-old female with respirator discomfort was admitted to our hospital and diagnosed to have situs solitus ccTGA, severe SAVV regurgitation, and ARV dysfunction. Her ARV ejection fraction was 25% and she was therefore dependent on inotropic agents. We successfully performed a tricuspid valve replacement while preserving the leaflets, the chorda tendineae's, and papillary muscles, and placing the lead for cardiac resynchronization therapy on the ARV. Her postoperative course was uneventful. Thereafter, she was discharged 6 weeks after surgery.

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