Endovascular and Hybrid Repair in Patients with Heritable Thoracic Aortic Disease


      • Largest single-center series examining endovascular repair in patients with HTAD.
      • Endovascular repair is relatively safe and may be lifesaving in emergent situations.
      • Endoleaks and reintervention rates are high after endovascular repair in HTAD.
      • Endovascular repair is appropriate as a bridging technique to open surgical repair.
      • Hybrid repair with surgically created proximal landing zones may improve durability.


      In individuals with heritable thoracic aortic disease (HTAD), endovascular repair for treatment of aortic aneurysm and dissection may be lifesaving, but is associated with increased risk of failure of endovascular repair and adverse outcomes. This study reports our experience with early and late outcomes of endovascular aortic and branch vessel repair in patients with HTAD.


      A retrospective case series was performed by chart review of individuals with HTAD followed at Washington University School of Medicine/Barnes-Jewish Hospital who underwent endovascular aortic and/or branch vessel repair. Clinical features, imaging characteristics, and short- and long-term outcomes were collected.


      Twenty-nine patients with HTAD (20 male; mean age 45 ± 13 years) underwent 37 endovascular procedures between 2006 and 2020 with mean follow up of 54 ± 41 months. Seven patients underwent two or more separate endovascular procedures. Each procedure was considered separate for data collection and analysis. Underlying conditions included Marfan syndrome (n = 16 procedures), Loeys-Dietz syndrome (n = 14 procedures), vascular Ehlers-Danlos syndrome (n = 3 procedures), and nonsyndromic HTAD (n = 4 procedures). Twenty patients (69%) had prior open surgical aortic repair. Indications for endovascular aortic repair (n = 31) included urgent repairs of acute complications of aortic dissection (n = 10) or aneurysm rupture (n = 3), and elective aortic repair (n = 18; 10 chronic dissections and eight chronic aneurysms). Six patients underwent elective endovascular repair of six branch vessel aneurysms or dissections. Six patients underwent hybrid open surgical and endovascular repair. Of the 37 procedures, 25 (68%) proximal landing zones were in the native aorta or branch vessel, 11 (30%) were in a surgical graft or elephant trunk and one was in a previously placed endograft. Thirty-six (97%) procedures were technically successful, and none required emergency surgical conversion. Two patients died: one from sepsis and one from presumed late pseudoaneurysm rupture, for a 5% per-procedure mortality rate. Two procedures were complicated by stroke and one patient developed paraparesis. Of the 31 aortic procedures, seven aortic endografts (23%) developed a stent-induced new entry (SINE) discovered with imaging at 20 ± 15 days post-procedure. Seven endografts (23%) developed a Type I endoleak and eight (26%) developed a Type II endoleak. No Type III endoleaks were seen. Within 30 days, two endografts (of 37, 5%) required reintervention. After 30 days, fifteen additional endografts (of 37, 41%) required reintervention. Two patients (of 6, 33%) who underwent hybrid repair required reintervention.


      This study is the largest single-center case series examining outcomes of HTAD patients following endovascular repair. Urgent and elective endovascular repairs in patients with HTAD can manage acute and chronic complications of aortic aneurysm and dissection with relatively low risk. However, risk of early and late endoleaks and SINE is high. Close post-procedural surveillance is required, and many individuals will require additional interventions. Hybrid repair shows promise and requires further investigation.
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        • Isselbacher E.M.
        • Lino Cardenas C.L.
        • Lindsay M.E.
        Hereditary influence in thoracic aortic aneurysm and dissection.
        Circulation. 2016; 133: 2516-2528
        • Pellenc Q.
        • Girault A.
        • Roussel A.
        • et al.
        Optimising aortic endovascular repair in patients with marfan syndrome.
        Eur J Vasc Endovasc Surg. 2020; 59: 577-585
        • Kölbel T.
        • Tsilimparis N.
        • Mani K.
        • et al.
        Physician-modified thoracic stent-graft with low distal radial force to prevent distal stent-graft–induced new entry tears in patients with genetic aortic syndromes and aortic dissection.
        J Endovasc Ther. 2018; 25: 456-463
        • Svensson L.G.
        • Kouchoukos N.T.
        • Miller D.C.
        • et al.
        Expert consensus document on the treatment of descending thoracic aortic disease using endovascular stent-grafts.
        Ann Thorac Surg. 2008; 85: S1-S41
        • Shalhub S.
        • Eagle K.A.
        • Asch F.M.
        • et al.
        Endovascular thoracic aortic repair in confirmed or suspected genetically triggered thoracic aortic dissection.
        J Vasc Surg. 2018; 68: 364-371
        • Parisi R.
        • Secco G.G.
        • Di Eusanio M.
        • et al.
        Endovascular repair of aortic dissection in marfan syndrome: current status and future perspectives.
        Diseases. 2015; 3: 159-166
        • Geisbüsch P.
        • Kotelis D.
        • von Tengg-Kobligk H.
        • et al.
        Thoracic aortic endografting in patients with connective tissue diseases.
        J Endovasc Ther. 2008; 15: 144-149
        • Waterman A.L.
        • Feezor R.J.
        • Lee W.A.
        • et al.
        Endovascular treatment of acute and chronic aortic pathology in patients with Marfan syndrome.
        J Vasc Surg. 2012; 55 (discussion 1240-1): 1234-1240
        • Orozco-Sevilla V.
        • Weldon S.A.
        • Coselli J.S.
        Hybrid thoracoabdominal aortic aneurysm repair: is the future here?.
        J Vis Surg. 2018; 4: 61
        • Moulakakis Konstantinos G.
        • Mylonas Spyridon N.
        • Avgerinos Efthimios D.
        • et al.
        Hybrid open endovascular technique for aortic thoracoabdominal pathologies.
        Circulation. 2011; 124: 2670-2680
        • Loeys B.L.
        • Dietz H.C.
        • Braverman A.C.
        • et al.
        The revised Ghent nosology for the Marfan syndrome.
        J Med Genet. 2010; 47: 476
        • Lombardi J.V.
        • Hughes G.C.
        • Appoo J.J.
        • et al.
        Society for vascular surgery (SVS) and Society of thoracic Surgeons (STS) reporting standards for type B aortic dissections.
        J Vasc Surg. 2020; 71: 723-747
        • Braverman A.C.
        • Moon M.R.
        • Geraghty P.
        • et al.
        Pregnancy after aortic root replacement in Loeys-Dietz syndrome: high risk of aortic dissection.
        Am J Med Genet A. 2016; 170: 2177-2180
        • Andersen N.D.
        • Keenan J.E.
        • Ganapathi A.M.
        • et al.
        Current management and outcome of chronic type B aortic dissection: results with open and endovascular repair since the advent of thoracic endografting.
        Ann Cardiothorac Surg. 2014; 3: 264-274
        • Glebova N.O.
        • Cameron D.E.
        • Black J.H.
        Treatment of thoracoabdominal aortic disease in patients with connective tissue disorders.
        J Vasc Surg. 2018; 68: 1257-1267
        • Dua A.
        • Kuy S.
        • Lee C.J.
        • et al.
        Epidemiology of aortic aneurysm repair in the United States from 2000 to 2010.
        J Vasc Surg. 2014; 59: 1512-1517
        • Roselli E.E.
        • Idrees J.J.
        • Lowry A.M.
        • et al.
        Beyond the aortic root: staged open and endovascular repair of arch and descending aorta in patients with connective tissue disorders.
        Ann Thorac Surg. 2016; 101: 906-912
        • Bozso S.J.
        • White A.
        • Nagendran J.
        • et al.
        Hybrid aortic arch and frozen elephant trunk reconstruction: bridging the gap between conventional and total endovascular arch repair.
        Expert Rev Cardiovasc Ther. 2018; 16: 209-217
        • Rudarakanchana N.
        • Jenkins M.P.
        Hybrid and total endovascular repair of the aortic arch.
        Br J Surg. 2018; 105: 315-327
        • Cowan J.A.
        • Dimick J.B.
        • Henke P.K.
        • et al.
        Surgical treatment of intact thoracoabdominal aortic aneurysms in the United States: hospital and surgeon volume-related outcomes.
        J Vasc Surg. 2003; 37: 1169-1174
        • Coselli J.S.
        • LeMaire S.A.
        • Preventza O.
        • et al.
        Outcomes of 3309 thoracoabdominal aortic aneurysm repairs.
        J Thorac Cardiovasc Surg. 2016; 151: 1323-1338
        • Frankel W.C.
        • Song H.K.
        • Milewski R.K.
        • et al.
        Open thoracoabdominal aortic repair in patients with heritable aortic disease in the GenTAC Registry.
        Ann Thorac Surg. 2020; 109: 1378-1384
        • Marcheix B.
        • Rousseau H.
        • Bongard V.
        • et al.
        Stent grafting of dissected descending aorta in patients with Marfan's syndrome: mid-term results.
        JACC Cardiovasc Interv. 2008; 1: 673-680
        • Nordon I.M.
        • Hinchliffe R.J.
        • Holt P.J.
        • et al.
        Endovascular management of chronic aortic dissection in patients with Marfan syndrome.
        J Vasc Surg. 2009; 50: 987-991
        • Botta L.
        • Russo V.
        • La Palombara C.
        • et al.
        Stent graft repair of descending aortic dissection in patients with Marfan syndrome: an effective alternative to open reoperation?.
        J Thorac Cardiovasc Surg. 2009; 138: 1108-1114
        • Eid-Lidt G.
        • Gaspar J.
        • Meléndez-Ramírez G.
        • et al.
        Endovascular treatment of type B dissection in patients with marfan syndrome: mid-term outcomes and aortic remodeling.
        Catheter Cardiovasc Interv. 2013; 82: E898-E905
        • Ince H.
        • Rehders T.C.
        • Petzsch M.
        • et al.
        Stent-grafts in patients with Marfan syndrome.
        J Endovasc Ther. 2005; 12: 82-88
        • Clough R.E.
        • Martin-Gonzalez T.
        • Van Calster K.
        • et al.
        Endovascular repair of thoracoabdominal and arch aneurysms in patients with connective tissue disease using branched and Fenestrated devices.
        Ann Vasc Surg. 2017; 44: 158-163
        • Jiang X.
        • Chen B.
        • Jiang J.
        • et al.
        Outcomes of endovascular therapy for Stanford type B aortic dissection in patients with Marfan syndrome.
        J Thorac Cardiovasc Surg. 2021;
        • Eleshra A.
        • Panuccio G.
        • Spanos K.
        • et al.
        Safety and effectiveness of TEVAR in native proximal landing zone 2 for chronic type B aortic dissection in patients with genetic aortic syndrome.
        J Endovasc Ther. 2021; (15266028211061276)
        • Kölbel T.
        • Eleshra A.
        • Aldag M.
        • et al.
        Endovascular treatment of aortic Pathologies in patients with marfan syndrome: single-center experience.
        J Endovasc Ther. 2021; (15266028211067733)
        • Pantaleo A.
        • Jafrancesco G.
        • Buia F.
        • et al.
        Distal stent graft-induced new entry: an emerging complication of endovascular treatment in aortic dissection.
        Ann Thorac Surg. 2016; 102: 527-532
        • Dong Z.
        • Fu W.
        • Wang Y.
        • et al.
        Stent graft-induced new entry after endovascular repair for stanford type B aortic dissection.
        J Vasc Surg. 2010; 52: 1450-1457
        • Loeys B.L.
        • Chen J.
        • Neptune E.R.
        • et al.
        A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2.
        Nat Genet. 2005; 37: 275-281
        • Bellamkonda K.S.
        • Dardik A.
        • Nassiri N.
        Endovascular repair of a dissecting pararenal abdominal aortic aneurysm in a patient with type III Loeys-Dietz syndrome.
        J Vasc Surg Cases Innov Tech. 2021; 1: 10-15
        • Casey K.
        • Zayed M.
        • Greenberg J.I.
        • et al.
        Endovascular repair of bilateral iliac artery aneurysms in a patient with Loeys-Dietz syndrome.
        Ann Vasc Surg. 2012; 26: 107.e5-10
        • Wang S.
        • Kernodle A.
        • Hicks C.W.
        • et al.
        Endovascular repair of tortuous recurrent femoral-popliteal aneurysm in a patient with Loeys-Dietz syndrome.
        J Vasc Surg Cases Innov Tech. 2018; 2: 156-159
        • Tenorio E.R.
        • Tallarita T.
        • Mirza A.K.
        • et al.
        Endovascular repair of large intercostal artery patch aneurysm using branch stent-graft in a patient with Loeys-Dietz syndrome.
        J Thorac Cardiovasc Surg. 2020; 159: e95-e99
        • Ohman J.W.
        • Charlton-Ouw K.M.
        • Azizzadeh A.
        Endovascular repair of an internal mammary artery aneurysm in a patient with Loeys-Dietz syndrome.
        J Vasc Surg. 2012; 55: 837-840
        • Kalra A.
        • Harris K.M.
        • Kische S.
        • et al.
        Endovascular repair of descending thoracic aorta in Loeys-Dietz II syndrome.
        Ann Vasc Surg. 2015; 29: 1452.e17-1452.e21
        • Agrawal Y.
        • Gupta V.
        A rare cause of recurrent aortic dissection.
        J Saudi Heart Assoc. 2016; 28: 176-179
        • Preventza O.
        • Mohammed S.
        • Cheong B.Y.
        • et al.
        Endovascular therapy in patients with genetically triggered thoracic aortic disease: applications and short- and mid-term outcomes.
        Eur J Cardiothoracic Surg. 2014; 46: 248-253
        • Hashizume K.
        • Shimizu H.
        • Honda M.
        • et al.
        Stepwise total aortic repairs with Fenestrated endografts in a patient with Loeys-Dietz syndrome.
        Ann Thorac Surg. 2017; 104: e39-e42
        • Clare R.
        • Jorgensen J.
        • Brar S.S.
        Open versus endovascular or hybrid thoracic aortic aneurysm repair.
        Curr Atheroscler Rep. 2016; 18: 60