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[Ehlers-Danlos disease revealed during pregnancy through the diagnosis of aortic dissection]
G Babatasi1, M Massetti, E Saloux
1Service de chirurgie thoracique et cardiovasculaire, CHU Caen.
Insights
Aortic dissection during pregnancy is rare but serious. This case highlights successful multidisciplinary management of aortic dissection in a patient with Ehlers-Danlos syndrome, ensuring positive outcomes for mother and child.
Area of Science:
- Cardiovascular Surgery
- Obstetrics
- Genetics
Background:
- Aortic dissection is a life-threatening condition, particularly rare during the third trimester of pregnancy.
- Key risk factors include hypertension and connective tissue disorders such as Marfan syndrome and Ehlers-Danlos syndrome.
Observation:
- The authors present a rare case of aortic dissection occurring in the third trimester of pregnancy.
- The patient was diagnosed with Ehlers-Danlos syndrome, a collagen disorder, at the onset of the condition.
Findings:
- A multidisciplinary team involving anesthesiologists, cardiologists, obstetricians, and cardiothoracic surgeons managed the case.
- The management strategy involved staged total replacement of the supra-coronary aorta over a decade, utilizing CT scans and annual echocardiographic follow-ups.
Implications:
- This case demonstrates the feasibility of successful management of aortic dissection in pregnancy with Ehlers-Danlos syndrome.
- A collaborative, long-term approach can lead to favorable outcomes for both the mother and the neonate.
Abstract:
Dissection of the aorta is a serious condition but rare in young women, and occurring during the 3rd trimester of pregnancy. The main risk factors are hypertension and diseases of the connective tissue or of collagen (Marfan's syndrome and Ehlers-Danlos disease). The authors report a case of dissection of the aorta managed in a pluridisciplinary manner by the anaesthetists, cardiologists, obstetricians and cardiothoracic surgeons, which resulted in a favourable outcome for both mother and baby. The diagnosis of Ehlers-Danlos disease was made from the onset and, over a period of 10 years with CT scan and annual echocardiographic follow-up, total replacement of the supra-coronary aorta was performed in several stages.