Shone’s Complex Variant in an Adult: complex mitral repair with neo-chordae and aortic valve replacement with root enlargement

MP059 

Case Video Submission 

Katherine Phillips (1), Les James (2), Aubrey Galloway (1)

(1) NYU Langone Health, New York, NY, (2) NYU Grossman School of Medicine, United States

Katherine Phillips    -  Contact Me
NYU Langone Health

Les James    -  Contact Me
NYU Grossman School of Medicine

*Aubrey Galloway    -  Contact Me
NYU Langone Health

Katherine Phillips    -  Contact Me
N/A

Objective: Variants of Shone's Complex are often unrecognized when presenting in adults, and many cardiac surgeons are unfamiliar with the complexities of surgical correction. Repair of a parachute MV with dysplastic tissue and severe stenosis is challenging and often not feasible, resulting in the need for a more complex double valve replacement with a Konno-Rastan aortoventriculoplasty. The purpose of this report was to increase surgeon awareness of this condition and to demonstrate the feasibility of complex MVr in these patients. A 38 year old woman presented with a parachute MV, with dysplastic and restrictive tissue, and severe tunnel-like mitral stenosis (PG = 27mmHg), a severely stenotic unicuspid aortic valve and a small aortic annulus (18 mm). This video demonstrates the operative techniques used for complex MVr, AVR and posterior aortic root enlargement in this patient.
Case Video Summary: The video shows a dysplastic and restrictive parachute MV with two papillary muscles (PM), restricted leaflet movement and funnel-like stenosis. The PMs are noted to be fused directly to the anterior and posterior MV leaflet edges, and to the left ventricular wall. The posterior leaflet has dysplastic tissue along the free edge with fan-like fusion to the subvalvular apparatus. An initial attempt at splitting the PMs to the ventricular apex and cutting restrictive attachments did not produce adequate leaflet mobility. Therefore, the anterior PM and posterior branch of the posterior PM were transected, and the dysplastic tissue along the free edge of the leaflet was cut, followed by placement of PTFE neochordae for bi-leaflet repair. The unicuspid aortic valve was excised and the aortic annulus enlarged, extending the transannular patch onto the anterior MV leaflet, allowing AVR with a #21 mechanical valve.
Conclusions: Repair of adults with variants of Shone's Complex is complicated, and the pathology may be underappreciate by many adult cardiac surgeons. This video demonstrates a unique approach for complex repair of a parachute MV with dysplastic subvalvular tissue, combined with AVR-aortic root enlargement. This is one of the first reports of repair of a dysplastic parachute MV with restrictive papillary muscle-leaflet fusion in an adult with Shone's complex by PM division and neochordae repair. This approach was an effective option and should be considered for parachute MVs when splitting the fused papillary muscle alone is not adequate.

Congenital Mitral Valve Disease