Abstract

Introduction and importanceAortic regurgitation and aortic root dilatation are common features in adults with both repaired and unrepaired tetralogy of Fallot (TOF). Valve-sparing aortic root replacement (VSARR) is an effective repair for aortic regurgitation due to progressive aortic root dilatation with TOF after repair. However, the effectiveness of this technique for unrepaired patients has rarely been reported.Case presentationWe reported a case of a 30-year-old man with cyanosis and exertional dyspnea. Echocardiography and computed tomography showed unrepaired TOF with significant aortic regurgitation and massively dilated aortic root. Aortic root replacement was mandatory. He underwent successful concomitant VSARR with TOF repair. At 6-month follow-up, he remains stable with trivial aortic regurgitation on echocardiogram.Clinical discussionIn unrepaired TOF, the absence of sub-annular muscular rim and the unbalance of aortic sinuses make VSARR utmost challenging. If can be done successfully, VSARR preserves the native aortic valve and avoids lifelong anticoagulation therapy.ConclusionConcomitant VSARR with TOF repair can be safely and effectively applied for unrepaired patients presenting with progressive aortic root dilatation and significant aortic regurgitation.

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