Abstract

BackgroundQuadricuspid aortic valve (QAV) is a rare congenital heart defect usually accompanied with different hemodynamic abnormalities. Due to the rarity of QAV, treatment and prognosis of QAV patients with aortic regurgitation still remain challenging. We here present the first case of a patient with severe QAV regurgitation who underwent successful treatment and performed favorable prognosis with transapical aortic valve implantation (TAVI) using J-Valve system.Case presentationA 62-year-old man experienced intermittent palpitation, shortness of breath and chest pain. Echocardiography revealed congenital QAV with massive aortic regurgitation and mild aortic stenosis, left ventricular enlargement. Aortic valve replacement was successfully performed with TAVI using J-Valve system. The postoperation and follow-up was uneventful.ConclusionTAVI using J-Valve system has emerged as a new high success rate method for treatment of patients with simple non-calcified aortic valve insufficiency.

Highlights

  • Quadricuspid aortic valve (QAV) is a rare congenital heart defect with an estimated incidence between 0.003 and 0.013%

  • transapical aortic valve implantation (TAVI) using J-Valve system has emerged as a new high success rate method for treatment of patients with simple non-calcified aortic valve insufficiency

  • Echocardiography revealed congenital QAV (Supplemental video 1) with massive aortic regurgitation and mild aortic stenosis, left ventricular enlargement (Fig. 1)

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Summary

Introduction

Quadricuspid aortic valve (QAV) is a rare congenital heart defect with an estimated incidence between 0.003 and 0.013%. Echocardiography revealed congenital QAV (Supplemental video 1) with massive aortic regurgitation (the regurgitant jet area was measured as 12.4 cm2) and mild aortic stenosis, left ventricular enlargement (Fig. 1). A 27-mm J-Valve (Jie-cheng Medical Technology, Suzhou, Fig. 1 Echocardiography showed left ventricular enlargement (Panel A, red star indicated dilated ventricle), quadricuspid aortic valve (Panel B, red arrow indicated four aortic valves, which have been marked numerically), mild aortic stenosis (Panel C, red arrow indicated the hole formed by aortic insufficiency, the area of which is about 0.124cm2) and massive aortic regurgitation (Panel D) of the QAV patient. Our patient was followed up at the outpatient, he made a successful recovery, the NT-ProBNP was down to 104 ng/L and enchocardiography revealed that the artificial aortic valve functioned normally, the left ventricular diameter and ventricular function were normal with the left ventricular ejection fraction of 64% (Fig. 3)

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