Abstract We present a case of a 36-year-old
male patient with known
arthrogryposis multiplex congenita and an associated unicuspid
aortic valve. The
patient later developed a significant
aneurysm of the
ascending aorta, however refused surgical intervention and missed follow-up
appointments for 5 years. During an urgent,
general practitioner-initiated
transthoracic echocardiography follow-up, a chronic type A
aortic dissection was diagnosed as a result of progressive aortic
dilatation. Due to the stationary
pressure gradients and non-progressive leaflet
fibrosis, a conservative approach for to the unicuspid
aortic valve was chosen, combined with replacement of the
ascending aorta and partial replacement of the
aortic arch.