Quadruple Valve Replacement for Behçet's Syndrome: A Case Report.

BACKGROUND

Behcet's, a systemic autoimmune vasculitis, often leads to cardiovascular complications sunch as valvular damage and aortic root pseudoaneurysms. Concurrent involvement of all four cardiac valves is exceptionally rare, posing significant surgical challenges due to tissue fraagility, inflammatory destruction, and high reoperation risks.

OBJECTIVE

To describe a tailored surgical strategy for quadruple replacement in a Behcet's syndrome patient with extensive valvular lesions, aortic root pseudoaneurysm, and prior mechanical valve failure, emphasizing techniques to address anatomical complexity and enable future transcatheter interventions.

METHODS

A 51-year-old female with Behcet's syndrome and prior aortic/pulmonary valve replacements underwent reoperation for aortic root pseudoaneurysm, paravalvular leaks, severe mitral/tricuspid regurgitation, and pulmonary valve thrombosis. Key techniques include three parts, firstly, radical debridement of necrotic tissue and annular enlargement (aortic: 20-25mm; mitral: 25-29mm). Second, modified "chimney" Commando-Bentall hybrid procedure with a tongue-shaped vascular path to reconstruct the aorto-mitral curtain and reinforce the aortic root. Third, "Sandwich" technique for aortic root reconstruction to minimize paravalvular leakage.

RESULTS

The surgery was successful, with uneventful recovery and discharge on postoperative day 30. Six-month follow-up confirmed patient survival, stable valve function, and no recurrent leaks. Dual annular enlargement facilitated larger protheses, reducing patient-prothesis mismatch and preserving options for future transcatheter valve im plantation (TAVI).

CONCLUSIONS

This cases highlights the feasibility of combining annular enlargement, root reinforcement, and bioprosthetic valve replacement in Behcet's syndrome with pan-valvular involvement. The hybrid chimney-Bentall technique and meticulous root reconstruction address inflammatory tissue vulnerability while enabling future minimally invasive interventions. This approach optimizes immediate outcomes and long-term durability in complex autoimmune-associated valvulopathies.