TAVI-in-TAVI in a patient with morquio syndrome: a case report

TL;DR

This case report describes the first TAVI-in-TAVI procedure in a patient with Morquio syndrome, a rare genetic disorder, highlighting the challenges and successful outcome.

Contribution

The first reported TAVI-in-TAVI procedure in a patient with Morquio syndrome, demonstrating individualized planning for complex redo-TAVI.

Findings

A 65-year-old woman with Morquio syndrome successfully underwent a TAVI-in-TAVI procedure.

The procedure was technically challenging but resulted in favorable haemodynamic outcomes with no major complications.

Abstract

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.

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.

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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