Case Report
The patient was a 54-year-old gentleman. On arrival, his conditions were critical with blood
pressure of 67/47 mmHg. Echo examinations showed pericardial effusion and dissecting
flaps in the bilateral carotid arteries. Computed tomographic (CT) scans showed AAAD with
malperfusion of the left carotid artery and the right common iliac artery. At surgery, CPB was
immediately established with a right femoro-femoral circuit. A large amount of clot was found
in the intrapericardial space. Central cannulation for CPB into the true channel of the
ascending aorta was added. At 28℃ of the bladder temperature, hypothermic circulatory
arrest (HCA) was induced, and the heart was arrested. A huge primary entry was located
from the transverse arch to the proximal descending aorta at the zone 4 level (Fig 1 ). The
dissection was extended into each arch-vessel. The proximal descending aorta was
transected completely at the zone 4 level, excluding the primary entry. A FET device,
FROZENIX® (Japan Lifeline Co, Tokyo, Japan) of 23 mm in diameter and 60 mm in
length, was inserted into the true lumen. To this aortic stump, a multibranched arch
graft, GelweaveTM (TERUMO VASCUTEK, Glasgow, UK) of 24 mm in diameter, was
connected. After systemic reperfusion, the three arch-vessels were reconstructed,
repairing all dissected arteries. At the proximal site, rupture of the aortic root was found at the
commissure between the right- and the non-Valsalva sinuses (Fig 2A ). The dissection was
extended into the right coronary artery. Aortic valve had no significant deformities, which
allowed us to perform VSRR using a reimplantation technique with Gelweave ValsalvaTM
Ante-Flo (TERUMO VASCUTEK, Glasgow, UK) of 26 mm in diameter. The commissure post
between the right and non-coronary cusps had no adventitial. Only the dissecting flap was
carefully fixed to the graft with a 5-0 polypropylene pledgetted mattress suture. The 2nd-low
sutures for attachment of the aortic wall were also meticulously placed (Fig 2B ). He
developed delay of full awakening. The CT-scans and MRI revealed small cerebral infarction
on the left frontal lobe and the cerebellum, however it was not so critical without any
permanent neurological dysfunctions. The postoperative CT-scans (Fig 3 ) showed no
remarkably abnormal findings. He was finally diagnosed as vEDS by a genetic test, revealing
a novel frame-shift mutation of the COL3A1 gene.