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TAVI-in-TAVI in a patient with morquio syndrome: a case report
Diana Xiao Lan Chin1, Gianantonio De Michele2, Davide Cristofani3
1Department of Cardiology, Interventional Cardiology Unit, San Camillo Hospital, Circ.ne Gianicolense 87, Rome 00152, Italy.
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.
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