A 65-year-old woman with Morquio syndrome successfully underwent a TAVI-in-TAVI procedure with favorable hemodynamic outcomes and no major complications.
Is a TAVI-in-TAVI procedure feasible and safe in a patient with Morquio syndrome and structural valve degeneration?
TAVI-in-TAVI is feasible and can be successfully performed with individualized planning in patients with rare congenital disorders like Morquio syndrome.
Abstract Background Morquio syndrome (MPS IV) is a rare multi-systemic disorder with significant cardiovascular implications, including early-onset valvular disease. Due to the improved life expectancy, these patients could require complex interventional solutions such as TAVI-in-TAVI procedure. Case summary We present the first reported case of a 65-year-old woman with Morquio syndrome undergoing a TAVI-in-TAVI procedure for structural degeneration of a prior transcatheter bioprosthetic valve. The procedure was technically challenging due to complex thoracic anatomy, small aortic annulus, and intermediate-risk of coronary obstruction. A self-expanding Evolut FX + valve were successfully implanted with favourable haemodynamic outcomes and no major complications. Conclusion This case highlights the feasibility and importance of individualized planning in complex redo-TAVI interventions in patients with rare congenital disorders.
Chin et al. (2026) studied this question. A 65-year-old woman with Morquio syndrome successfully underwent a TAVI-in-TAVI procedure with favorable hemodynamic outcomes and no major complications.