Simultaneous “Tirone David–V” Valve-Sparing Aortic Root Replacement and Radical Mitral Valve Repair for the Marfan Syndrome With Barlow Syndrome
Aortic root dilatation and aortic regurgitation (AR) are the most prominent cardiovascular manifestations of the Marfan syndrome (MFS), but mitral valve regurgitation (MR) requiring operative correction develops in 15% to 20%. The mitral pathology usually represents an advanced form of the Barlow syndrome. Aortic root aneurysm and severe mitral regurgitation require simultaneous surgical correction. Historically, replacement of the aortic root and valve with a composite valve graft using a mechanical prosthesis has been the standard operation for patients with the MFS. Recently, valve-sparing aortic root replacement has become popular in selected patients because indefinite anticoagulation is not necessary.
A 26-year-old man with the MFS and progressive fatigue and dyspnea was referred from another state with an enlarging aortic root aneurysm (50 mm), mild AR, and severe MR due to posterior mitral valve leaflet (PMVL) prolapse. He had an aversion to anticoagulation because of his athletic lifestyle and underwent aortic valve-sparing root replacement using the “Tirone David-V” method, involving reimplantation of the aortic valve, creation of Dacron pseudosinuses of Valsalva, and coronary reimplantation (Figures 1A and 2⇓A), in addition to extensive mitral repair involving 7-cm quadrangular resection of the PMVL middle scallop (Figure 1B); Wells advancement-plasty to make the hugely dilated (60 mm) annulus smaller; sliding-plasty of the PMVL anterolateral and posteromedial scallops to lower their heights (Figure 2B); and partial, flexible-ring annuloplasty (Figure 2C). The flail PMVL was secondary to elongated and ruptured chordae tendineae. Postoperative echocardiography showed normal aortic valve function, trace AR, and trace MR (Figure 3A and 3B). He was discharged on postoperative day 6, and 1 year later he is clinically well.
The combined surgical approach described is a valuable option in selected patients with the MFS requiring simultaneous aortic root replacement and correction of MR. It preserves both the mitral and aortic valves with secure stabilization of the aortic and mitral annuli, and indefinite anticoagulation is avoided.
Dr Demers is supported by a Research Fellowship Award of the Heart and Stroke Foundation of Canada and is a Thelma and Henry Doelger Cardiovascular Surgical Research Scholar at Stanford University Medical School.
Dr Miller serves as a consultant to the Medi-Tech Division of Boston Scientific Corp.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
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