CASE REPORT
A 34-year-old man with Marfan syndrome underwent a David procedure in 2006 for an 8.6 cm ascending aortic aneurysm, complicated by excessive bleeding requiring a Cabrol fistula. One decade later, he presented with worsening dyspnea. Transthoracic echocardiography revealed severe mitral regurgitation (MR) and mild aortic regurgitation requiring repeat full sternotomy for surgical repair.
Intraoperative transesophageal echocardiography (TEE) additionally showed severe aortic regurgitation and mitral valve leaflet prolapse. The patient underwent right axillary arterial cannulation with dual-stage femoral venous cannulation given the sternal proximity of the previous Cabrol fistula. Following complex mitral valve repair, the aortic valve was opened revealing severe adhesions along the aortic root secondary to the previous Cabrol shunt. Given limited access for safe suture placement and already prolonged bypass time, we elected to use the sutureless Edwards Intuity valve (Edwards Life Sciences Corporation, California, United States). After valve leaflet removal, the left ventricular outflow tract was sized and a 27-mm Intuity valve was selected. Guiding sutures were placed in the nadir of each cusp remnant, through the sewing cuff of the valve, and the valve was deployed. The attached balloon was inflated to 5 atmospheres of pressure for 10 seconds. Visual inspection demonstrated proper deployment. The patient was weaned easily off bypass and post-bypass TEE demonstrated mild aortic PVL and minimal MR.
The patient came back to the intensive care unit in a stable condition and was extubated. All vasopressors were weaned within 24 hours. On post-operative day (POD) 2, the patient’s bloodwork revealed ongoing intravascular hemolysis. Transthoracic Echocardiogram showed moderate-to-severe PVL circumferentially halfway around the valve. By POD 3 the patient showed no signs of improvement and re-intervention appeared warranted.
In light of the patient’s complex aorta’s root anatomy, we elected to proceed with the less invasive percutaneous approach as our first-line strategy. The PVL was too large to attempt an Amplatzer Septal Occluder device (St. Jude Medical, Minnesota, United States), therefore off-label BVPD was attempted with a 30-mm Z-MED II balloon (B. Braun Medical, Melsungen, Germany) via the femoral artery. The procedure was performed with appropriate flaring of the valve’s skirt component (Figure 1), but without improvement in PVL.
On POD 4 the patient continued to hemolyze necessitating surgical re-operation. Aortic root inspection revealed a peri-annular gap extending across a third of the valve’s circumference toward the non-coronary sinus area. We replaced the valve with a 27-mm On-X Mechanical Valve (CryoLife, Georgia, United States). Post-bypass TEE showed a well-functioning prosthesis without PVL. His post-operative course was uneventful and he was discharged 12 days after the initial operation.