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Valve-sparing Aortic Root Replacement In 18 Month-old With Loeys-dietz And Arterial Tortuosity Syndrome
Alan O'Donnell, Awais Ashfaq, David Morales.
Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
We present a case of an 18m/o, 9.6kg male with Loeys-Dietz (Type II - TGFBR2 Mutation - c.1583G>A - p.Arg528His) and Arterial Tortuosity Syndrome presenting for a valve-sparing aortic root replacement due to his aggressive form of Loeys-Dietz Syndrome. Patient was followed as an outpatient that had significant change in his aortic root dimensions on serial echocardiograms. Patient had a chest CT completed with his aortic measurements below. Aortic Valve: 10x21mm (Z-Score: 8.4x11.6)Aortic Root: 33x34x38mm (Z-Score: 15.4x16.5x19.8)STJ: 26x27mm (Z-Score: 13.5x14.4)Ascending Aorta: 18.7x17.3mm (Z-Score: 5.4x4.3) The patient was placed on single venous cardiopulmonary bypass and the heart is arrested. The ascending aorta was transected, and commissural pledget-supported sutures were placed. Right and left coronary buttons were excised and mobilized. The remaining aortic root was mobilized at the ventriculoarterial junction and the noncoronary sinus excised. Pledget-supported sutures were placed at the nadir of each sinus and three sutures placed at the base of the commissures. A 20mm Hemashield Platinum graft was chosen. The subannular and commissural sutures were passed through the graft and tied. Starting at the nadir of each sinus, the leaflet sinuses were sutured to the graft and tied. Valve appeared to coapt well. The left (posterior) and right (anterior) coronary buttons were secured to the graft using a pericardial buttress. The graft was sutured to the distal ascending aorta with a bovine pericardial buttress. Patient is now one-year post-op with stable measurements of his aortic root and ascending aorta.
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