Related Experiment Videos
The Bentall procedure: a surgical option in Ehlers-Danlos syndrome
J Raman1, R F Saldanha, D S Esmore
1Cardiothoracic Surgical Unit, St. Vincent's Hospital, Sydney, Australia.
Insights
Ehlers-Danlos syndrome (EDS) patients with aortic aneurysms can safely undergo the Bentall procedure. This cardiovascular surgery offers a promising option to reduce risks of dissection and rupture in these complex cases.
Area of Science:
- Cardiovascular Surgery
- Connective Tissue Disorders
- Vascular Medicine
Background:
- Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders.
- Arterial-ecchymotic type IV EDS presents significant risks for large vessel involvement and bleeding.
- Cardiovascular surgical outcomes for EDS patients are typically poor.
Observation:
- Two patients with Ehlers-Danlos syndrome and ascending aortic aneurysmal dilatation were treated.
- One patient had an aortic dissection from the ascending aorta to the iliac arteries.
- The second patient had asymptomatic, progressive aortic root aneurysmal dilatation.
Findings:
- Both patients successfully underwent the Bentall procedure using a composite valved conduit.
- Despite vascular friability, patients tolerated surgery without complications.
- Early follow-up shows positive outcomes for both individuals.
Implications:
- The Bentall procedure appears to be a viable and safe option for managing aortic aneurysms in EDS patients.
- This surgical approach may mitigate the risks of catastrophic aortic events in this population.
- Further research is warranted to confirm long-term efficacy and safety in Ehlers-Danlos syndrome patients.
Abstract:
The Ehlers-Danlos syndrome (EDs) is one of the most frequently encountered inherited disorders of connective tissue. The arterial-ecchymotic type IV is notorious for large vessel involvement associated with spontaneous catastrophic bleeding. Most of these patients who require cardiovascular surgical procedures have a poor prognosis. Our experience of two patients, with aneurysmal dilatation of the ascending aorta is presented. The first patient presented with an aortic dissection extending from the ascending aorta to the common iliac arteries. The second patient presented with asymptomatic but progressive aneurysmal dilatation of the aortic root. Both patients underwent the Bentall procedure using a 25 mm St. Jude composite valved conduit. Despite increased vascular friability, both of these patients tolerated the operative procedure without complication and are doing well on early follow-up. This experience suggests that the Bentall procedure may be used to decrease the risks of dissection and rupture of the ascending aorta in patients with Ehlers-Danlos syndrome.