Endovascular Thoracoabdominal Aneurysm Repair in Patients with Connective Disease
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1 Endovascular Thoracoabdominal Aneurysm Repair in Patients with Connective Disease TM Mastracci MD FRCSC Royal Free London
2 Pertinent Disclosures CYDAR Medical: Research Collaboration and Consultation Siemens Imaging: Research Collaboration Cultivate Labs: Research Collaboration Cook Medical: Educational Grant and Proctoring The Royal Free Aortic Charity Funds Research and Educational Activities to support the Aortic Team at Royal Free Foundation Trust
3 What Constitutes CTD? What do we know? Are there Any Rules in CTD?
4 What Constitutes CTD?
5
6 **Schematic representation
7 Marfan Syndrome Ectopic Lentis Overgrowth Pectus Scoliosis Aortic Root Ehlers Danlos Uterine Rupture Bowel Rupture Thin Skin Easy Bruising Varicose Veins Arterial Tortuosity Syndrome Familial TAA Aneurysm, proximal Loeys Dietz Overgrowth (milder) Pectus Scoliosis Aortic Root Arterial Tortuoisty Hypertelorism Cleft Palate **Schematic representation
8 Age Family History Morphology
9 What do we know?
10 TEVAR and CTD Author Year Pathology N Endoleak Mortality (IH) Stroke SCI Pacini 2013Dissection 54 22% 2.50% 1.90% Late intervention Eid-Lidt 2013Dissection 10 44% 11% 33% Waterman 2012Dissection 16 Preventza Dissection and 2013 Aneurysm % 1.6% 1.6% 17%
11 Freedom from Secondary Procedures (After any Primary Procedure) Number at Risk Qureshi, Greenberg, Moran et al, SVS 2013
12 The Cleveland Experience Patients 1 Fenestrated Device Roselli et al, Ann Thoracic Surg 2015
13 The Lille Experience 17/427 Patients Mean age 51 years Def n CTD: Met Marfan criteria Family history Extensive aortic disease at a young age Joint hypermobility Chest deformity Characteristic facial features Cleft palate Bifid uvula Hypertelorism Translucent skin 100% technical success Follow up: 3.4 years 6/17: 35% aneurysm regression Clough et al, Ann Vasc Surg 2016
14 Royal Free Experience: Age at Operation CTD Acuity Mortality SCI Reintervention 1 60 Unknown Elective Lost to f/u 2 57 Unknown Elective Lost to f/u 3 60 Unknown Elective Lost to f/u 4 58 Unknown Elective Lost to f/u 5 58 Unknown Elective Alive No endoleak -- onyx 6 58 Unknown Elective Lost to f/u 7 60 Unknown Elective Lost to f/u 8 56 **Infection Urgent Unknown Yes 9 53 Family History Elective Alive No No Unknown Elective Alive No No Takyasu Urgent In Death YesPost open repair Unknown Elective Alive YesDissection, Staged Family History Electiive Alive No Dissection, Staged Loeys Dietz Elective Alive No Iliac Extension
15 Royal Free Experience: Age at CTD Acuity Mortality SCI Reintervention Operation 1 58 Unknown Elective Alive No endoleak -- onyx 2 56 **Infection Urgent Unknown Yes 3 53 Family History Elective Alive No No 4 58 Unknown Elective Alive No No 5 39 Takyasu Urgent In Hosp Death YesPost open repair 6 58 Unknown Elective Alive YesDissection, Staged 7 59 Family History Electiive Alive No Dissection, Staged 8 44 Loeys Dietz Elective Alive No Iliac Extension
16 53 Year Old Woman No formal CTD diagnosis Healthy life to date Family History of Sudden Death Open attempt at repair, aborted at outside hospital Too Scarred to proceed Prolonged ITU stay, 6 months recovery Massive abdominal wall infection with skin grafting, now healed
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20 39 Year Old Woman Takayasu since young age Steroid dependant Morbidly Obese 20m Walking distance d/t SOB Ruptured AAA in Germany at age 32 open repair Renal Failure on Dialysis Presented with Symptomatic Aneurysm
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23 Spinal Cord Ischemia, Death at Day 20
24 44 Year Old Man, 5.8cm JRAA Ureteric atresia requiring Mitrofanoff Ascending and St. Jude AVR 35 year old Renal transplant 36 years old Recurrent Urinary tract infections Morbid obesity Family history of fatal aortic rupture Dx Marfan/Loeys Dietz
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28 Are there Any Rules in CTD? 1. Trust No Artery 2. Access Problems are different 3. Not only aortic sealing zones fail 4. Talk to the Family 5. Rule out Infection 6. Be Creative
29 Trust No Artery
30 Access is Tricky
31 Aortic Sealing Zones are Not the Only Points of Failure
32 Family History is Common Personalized Aortic Care
33 Rule Out Infection
34 Be Creative Pre Op Post Op
35 CTD And Complex Endovascular Repair Not standard of care Fraught with difficulty in the long and short term So much left to be understood
36 The Royal Free Aortic Team: Meryl Davis Debra Chong Nick Schofield Blandine Maurel Teresa Martin Gonzalez Prof George Hamilton Myhrra Gazo Yasmin Uddin Vanessa Swann Ved Ramaini
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