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 PVSS13. Long- Term Outcomes after Open and Endovascular Management of the Vasculature and Arterial Patterns Seen in Patients with Marfan, Vascular Ehlers Danlos and Loeys-Dietz Syndromes

​Moqueet A. Qureshi1, Roy K. Greenberg1, Zenia Martin1, Matthew J. Eagleton1, Rocio Moran2
1Cleveland Clinic - Department of Vascular Surgery, Cleveland, OH; 2Cleveland Clinic - Department of Genomic Medicine, Cleveland, OH.

OBJECTIVES: Endovascular means to address arterial problems in patients with connective tissue disorders (CTDs) has been viewed skeptically. We report the long-term outcomes of CTD patients treated for peripheral vascular (PV) problems.

METHODS: A retrospective review was conducted to identify all patients with diagnosed CTDs (1975-2012). Patients with genetic testing who met diagnostic criteria guidelines for Marfan (MS), Vascular Ehlers Danlos (EDS) and Loeys-Dietz Syndromes (LDS) were included. Demographics, genetic data, treatment indication and outcomes were analyzed.
 
RESULTS: A total of 480 patients were identified, with 63 patients (13%) fulfilling the inclusion criteria and presenting with PV rather than cardiac issues. Over a mean follow-up of 10.2±6.6 years, a total of 197 primary (151 open surgical (OS); 46 endovascular procedures (EP)) and 82 secondary procedures (64 OS; 18 EP) were performed. Of the EP, 22 were hybrid (18 aortic and 4 cerebrovascular) with the rest being isolated EP. Seven patients died as a result of vascular complications (5 OS, 2 EP; 4 during the perioperative period). There were 13 additional deaths not related to vascular problems. EDS patients had a higher incidence of infradiaphragmatic aortoiliac, while LDS patients had more supra-aortic trunk aneurysms.
 
CONCLUSIONS: Genetic identification of CTDs is rapidly expanding, allowing for categorization of previously undiagnosed patients. Endovascular options are viable and useful when there is acceptable vascular morphology and the procedure is planned properly, potentially in a hybrid modality.
 
AUTHOR DISCLOSURES: M. J. Eagleton: Cook Medical, Consulting fees or other remuneration (payment); R. K. Greenberg: Cook Medical, Research grants; Cook Medical, Consulting fees or other remuneration (payment); Cook Medical, Honorarium; Z. Martin: Nothing to disclose; R. Moran: Nothing to disclose; M. A. Qureshi: Nothing to disclose.
 
Distribution of Procedures
​Age (y) Thoracic​ ​ Thoracoabdominal​  ​ ​Aortoiliac ​ Subclavian / Carotid / Vertebral ​ ​
​Others
 ​
​Open Endo​ ​Open Endo​ ​Open ​Endo ​Open ​Endo ​Open
Endo
​Marfan (n=42) ​37.4 ± 15.7 ​69 ​11 ​16 ​3 ​9 ​8 ​5 ​1 ​12 ​5
EDS* (n=15) ​ ​42.3 ± 15.9 ​14 ​3 ​3 ​2 ​3 ​4 ​0 ​1 ​2 ​2
LDS (n=6) ​ ​35.8 ± 15.5 ​9 ​0 ​3 ​0 ​1 ​2 ​4 ​3 ​1 ​1
Total​ ​- ​92 ​14 ​22 ​5 ​13 ​14 ​9 ​5 ​15 ​8
 
* One patient had both EDS and LDS and underwent 2 open thoracic and 1 endovascular subclavian intervention.
 
Posted April 2013

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