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.