Introduction
Ehlers-Danlos syndrome (EDS) is a disorder in the metabolism of
fibrillary collagen. Its
vascular type (vEDS) frequently develops arterial rupture and aortic
dissection [1]. In general,
a tear-oriented surgery has been recommended for acute type A aortic
dissection (AAAD).
However, with increased experiences of total arch replacement (TAR), its
prevalence has
recently increased, particularly, in conjunction with frozen elephant
trunk (FET) [2]. In the
proximal site, in cases with rupture or dilatation of the root or with
primary entry in the root,
concomitant aortic root repairs are required. Of them, valve sparing
root replacement (VSRR)
is much more challenging due to prolonged cardiac arrest and
cardiopulmonary bypass
(CPB). We report on successful extended repairs of VSRR and TAR with FET
for massive
AAAD with root rupture and multiple malperfusion in a difficult patient
with vEDS.