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Congenital Aortic Valve Fenestration Repair & Root Remodeling in Heterotaxy Syndrome & Interrupted Inferior Vena Cava

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posted on 28.09.2021, 18:41 by Aditya Sengupta, Elbert E. Williams, Ismail El-Hamamsy, Paul E Stelzer

Fenestrations of the aortic valve have been described in patients with Down syndrome, Marfan syndrome, in those with bicuspid or quadricuspid valves, and in patient with myxomatous valvular degeneration. While rare and an infrequent cause of valve disease, congenital fenestrations can also occur (1). When indicated, such fenestrations can be repaired using a variety of strategies (2).

We herein present the case of a 24-year-old woman with pathologic aortic root enlargement (6.2 cm in greatest diameter) and an incidentally discovered large aortic valve fenestration that was successfully repaired as part of a valve-sparing root replacement procedure. The patient also had a history of heterotaxy syndrome and interrupted inferior vena cava (IVC).

At operation, the transverse arch and right atrium were cannulated, and arrest was achieved via antegrade cardioplegia. Valve inspection revealed the left leaflet to be a little longer than the other two with a large fenestration at the right-left commissural end. Leaflet height was measured with special aortic calipers and found to be adequate for repair. After closure of a large, complex atrial septal defect, the aortic root was separated from the surrounding tissues. The noncoronary sinus wall was resected and excess tissue in the other two sinuses was trimmed down. A 6-0 Goretex suture was placed from the outside of the aorta through the top of the left leaflet at the left-non commissure. This was woven along just below the free edge in loose, running fashion, staying below the fenestration on the other end and then out the top of that commissure. The other end of the Goretex was brought in the same way and placed running parallel to the first until the fenestration area, which was then included in the suture with an over-and-over looping technique without shortening the leaflet. This was also brought to the outside at the top of the left-right commissure and the two ends were gently tied. A small clip was placed on the Goretex above the knot.

Following this, an external ring annuloplasty was performed, and the valve-sparing root remodeling was completed using a 28-mm Hemashield graft. The cross-clamp was removed after a total ischemic time of 178 minutes, and the patient separated from bypass uneventfully. Following an unremarkable postoperative course, she was discharged home a week later. Her discharge echocardiogram showed no aortic insufficiency.


(1) Kaplan J, Farb A, Carliner NH, et al. Large aortic valve fenestrations producing chronic aortic regurgitation. Am Heart J.1991;122:1475–1477.

(2) Schäfers HJ, Langer F, Glombitza P, Kunihara T, Fries R, Aicher D. Aortic valve reconstruction in myxomatous degeneration of aortic valves: are fenestrations a risk factor for repair failure? J Thorac Cardiovasc Surg. 2010;139(3):660-664.


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