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Aortic dissection and Turner's syndrome
C Weytjens1, T Bové, P Van Der Niepen
1Department of Internal Medicine, University Hospital, Vrije Universiteit Brussel, Belgium.
Insights
Turner
Area of Science:
- Cardiovascular Medicine
- Genetics
- Obstetrics
Background:
- Turner's syndrome is frequently associated with cardiovascular anomalies, including aortic coarctation and bicuspid aortic valve.
- Aortic dissection is a rare but serious complication in Turner's syndrome patients, often involving the ascending aorta and necessitating surgical intervention.
Observation:
- A rare case of DeBakey type IIIb aortic dissection occurred in a patient with Turner's syndrome mosaicism.
- The dissection presented without proximal aortic involvement, dilation, or other cardiovascular malformations.
- The event followed a caesarean section for eclampsia.
Findings:
- The patient's distal aortic dissection was managed medically due to its uncomplicated nature and lack of ascending aorta involvement.
- Fifteen-month follow-up showed clinical stability with a stable dissection of the descending and abdominal aorta on CT scan.
Implications:
- This case highlights that aortic dissection in Turner's syndrome can occur distally without pre-existing aortic dilation or malformations.
- It suggests that medical management may be appropriate for specific types of aortic dissections in this patient population.
- Further research is needed to refine management strategies for aortic complications in Turner's syndrome.
Abstract:
Cardiovascular malformations, especially coarctation of the aorta and bicuspid aortic valve, are common in patients with Turner's syndrome. Only 46 patients with aortic dissection and/or rupture have been described. All patients had associated aortic dilation or aneurysm. In all cases except three, involvement of the ascending aorta was present, making surgery often imperative. We describe a rare case of a DeBakey type IIIb aortic dissection (without involvement of the proximal aorta) in a patient with Turner's syndrome mosaicism. The dissection occurred two weeks after a caesarean section because of eclampsia. No aortic dilation or other cardiovascular malformations were found. The distal extension and uncomplicated nature of the dissection indicated medical management. After fifteen months of follow-up, she is clinically doing well and repeated CT scan shows a stable dissection of the descending and abdominal aorta without dilation.