Second Transapical Mitral Valve-in-Valve With a Stented Bovine Jugular Venous Valve in Shone's Complex

Severe congenital mitral valve disease is difficult to manage in infants and small children. Off-label use of a Melody valve in the mitral position (MiM) has emerged as an important bridge to definitive valve replacement, with the potential to reintervene.

A 6-year-old boy with Shone's complex and multiple left-sided interventions, including surgical MiM implantation at age 3 months (Melody #1) and transapical MiM valve-in-valve at age 2 years (Melody #2), presented 4 years later with progressive mixed MiM dysfunction. In a hybrid catheterization laboratory, balloon dilation of the existing MiM improved expansion but resulted in worsening regurgitation, necessitating valve replacement. A limited left anterior thoracotomy was performed to facilitate a venoarterial rail, enabling controlled transfemoral advancement and deployment of Melody #3 within the prior MiMs, with good hemodynamic result.

Our case demonstrates the feasibility of repeat MiM valve-in-valve therapy, including a third MiM implantation, using a hybrid transapical approach to facilitate transfemoral delivery.