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.