Endovascular Treatment of Aortic Pathologies in Patients With Marfan Syndrome: Single-Center Experience

Abstract

Objectives:

To study the outcome of endovascular treatment of aortic pathologies in patients with Marfan syndrome (MFS) at a single institution.

Methods:

Consecutive MFS patients who underwent endovascular repair or hybrid procedures for aortic pathologies from January 2010 to May 2020 were identified. Several endovascular and hybrid strategies have been used. Technical success, short- and mid-term survival, complications, and re-interventions were retrospectively analyzed.

Results:

During the study period, 24 patients with MFS (median age, 48 [13–78] years; 58% males) were treated. Indications for intervention were chronic aortic dissection with aneurysm degeneration in 16 patients (67%), acute type B aortic dissection in 4 patients (17%), aortic aneurysm without any dissection in 3 patients (13%), and aortic intramural hematoma in 1 patient (4%). Most patients were asymptomatic (83%), three (13%) were symptomatic and one (4%) had a contained rupture. The median aneurysm diameter was 56 (35–86) mm. Hybrid procedures were performed in 7 (29%) patients. Thoracic endovascular repair was performed in 12 (50%) patients, a fenestrated or branched endovascular aortic repair in 4 (17%) patients, and placement of an iliac artery stent-graft in 1 (4%) patient. Procedures were staged in 12 (50%) patients. Technical success was achieved in all patients. The median intensive care unit stay was 6 (range, 1–30) days, and the median hospital stay was 23 (range, 3–112) days. Early mortality was reported in 1 (4%) patient. Wound infection was seen in 7 (29%) patients and gastrointestinal complications in 3 (13%) patients. The median follow-up was 42 (range, 1–127) months. The cumulative survival rate was 87% at 24 months. The cumulative freedom from re-intervention was 77% at 12 months.

Conclusions:

Endovascular treatment of aortic pathologies in patients with MFS appears feasible with acceptable early and mid-term outcomes in terms of mortality and re-intervention rates. Endovascular therapy plays an increasing role in MFS patients with aortic pathology.

Keywords

aortic dissection endovascular repair EVAR Marfan syndrome TEVAR thoracoabdominal aortic aneurysm

Get full access to this article

View all access options for this article.

References

Marsalese

Moodie

Vacante

, et al. Marfan’s syndrome: natural history and long-term follow-up of cardiovascular involvement. J Am Coll Cardiol. 1989;14(2):422–428; discussion 429.

Gott

Greene

Alejo

, et al. Replacement of the aortic root in patients with Marfan’s syndrome. N Engl J Med. 1999;340:1307–1313.

Gagné-Loranger

Voisine

Dagenais

Should endovascular therapy be considered for patients with connective tissue disorder. Can J Cardiol. 2016;32(1):1–3.

Dake

Miller

Semba

, et al. Transluminal placement of endovascular stent grafts for the treatment of descending thoracic aortic aneurysms. N Engl J Med. 1994;331:1729–1734.

Oderich

Mendes

Gloviczki

, et al. Current role and future directions of hybrid repair of thoracoabdominal aortic aneurysms. Perspect Vasc Surg Endovasc Ther. 2012;24(1):14–22.

Moulakakis

Mylonas

Avgerinos

, et al. Hybrid open endovascular technique for aortic thoracoabdominal pathologies. Circulation. 2011;124:2670–2680.

Spanos

Antoniou

GΑ

Giannoukas

, et al. Durability of fenestrated endovascular aortic repair for juxta-renal abdominal aortic aneurysm repair. J Cardiovasc Surg (Torino). 2018;59(2):213–224.

Georgiadis

van Herwaarden

Saengprakai

, et al. Endovascular treatment of complex abdominal and thoracoabdominal type IV aortic aneurysms with fenestrated technology. J Cardiovasc Surg (Torino). 2017;58(4):574–590.

Geisbüsch

Kotelis

von Tengg-Kobligk

, et al. Thoracic aortic endografting in patients with connective tissue diseases. J Endovasc Ther. 2008;15(2):144–149.

10.

Glebova

Cameron

Black

III . Treatment of thoracoabdominal aortic disease in patients with connective tissue disorders. J Vasc Surg. 2018;68(4):1257–1267.

11.

Fleck

Hutschala

Tschernich

, et al. Stent graft placement of the thoracoabdominal aorta in a patient with Marfan syndrome. J Thorac Cardiovasc Surg. 2003;125(6):1541–1543.

12.

Botta

Russo

Grigioni

, et al. Unusual rapid evolution of type B aortic dissection in a Marfan patient following heart transplantation: successful endovascular treatment. Eur J Vasc Endovasc Surg. 2006;32(4):358–360.

13.

Beregi

Haulon

Otal

, et al. Endovascular treatment of acute complications associated with aortic dissection: midterm results from a multicenter study. J Endovasc Ther. 2003;10(3):486–493.

14.

Ince

Rehders

Petzsch

, et al. Stent grafts in patients with Marfan syndrome. J Endovasc Ther. 2005;12(1):82–88.

15.

Marcheix

Rousseau

Bongard

, et al. Stent grafting of dissected descending aorta in patients with Marfan syndrome. J Am Coll Cardiol Intervent. 2008;1:673–680.

16.

Böckler

Meisenbacher

Peters

, et al. Endovascular treatment of genetically linked aortic diseases. Gefasschirurgie. 2017;22(suppl 1):1–7.

17.

Hiratzka

Bakris

Beckman

, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation. 2010;121:e266–e369.

18.

Riambau

Böckler

Brunkwall

, et al. Editor’s choice—Management of descending thoracic aorta diseases: clinical practice guidelines of the European Society for Vascular Surgery (ESVS). Eur J Vasc Endovasc Surg. 2017;53(1):4–52.

19.

Dean

Marfan syndrome: clinical diagnosis and management. Eur J Hum Genet. 2007;15:724–733.

20.

Lombardi

Hughes

Appoo

, 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(3):723–747.

21.

Rohlffs

Tsilimparis

Fiorucci

, et al. The candy-plug technique: technical aspects and early results of a new endovascular method for false lumen occlusion in chronic aortic dissection. J Endovasc Ther. 2017;24(4):549–555.

22.

Kölbel

Tsilimparis

Mani

, 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(4):456–463.

23.

Tsilimparis

Debus

Biehl

, et al. Fenestrated-branched endografts and visceral debranching plus stenting (hybrid) for complex aortic aneurysm repair. J Vasc Surg. 2018;67(6):1684–1689.

24.

Spanos

Kölbel

Kubitz

, et al. Risk of spinal cord ischemia after fenestrated or branched endovascular repair of complex aortic aneurysms. J Vasc Surg. 2019;69(2):357–366.

25.

Tsilimparis

Fiorucci

Debus

, et al. Technical aspects of implanting the t-branch off-the-shelf multibranched stent-graft for thoracoabdominal aneurysms. J Endovasc Ther. 2017;24(3):397–404.

26.

Clough

Martin-Gonzalez

Van Calster

, 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.

27.

Waterman

Feezor

Lee

, et al. Endovascular treatment of acute and chronic aortic pathology in patients with Marfan syndrome. J Vasc Surg. 2012;55(5):1234–1240; Discussion 1240–1241.

28.

Pacini

Parolari

Berretta

, et al. Endovascular treatment for type B dissection in Marfan syndrome: is it worthwhile. Ann Thorac Surg. 2013;95(2):737–749.

29.

Wipper

Kölbel

Sandhu

, et al. Impact of hybrid thoracoabdominal aortic repair on visceral and spinal cord perfusion: the new and improved SPIDER-graft. J Thorac Cardiovasc Surg. 2019;158(3):692–701.

30.

Cooper

Walsh

Sadat

, et al. Treating the thoracic aorta in Marfan syndrome: surgery or TEVAR? J Endovasc Ther. 2009;16(1): 60–70.

31.

Qato

Conway

, et al. Outcomes of Thoracic Endovascular Aneurysm Repair (TEVAR) in patients with connective tissue disorders. Vasc Endovascular Surg. 2020;54(8):676–680.

32.

Shalhub

Eagle

Asch

, et al. Endovascular thoracic aortic repair in confirmed or suspected genetically triggered thoracic aortic dissection. J Vasc Surg. 2018;68(2):364–371.

33.

Harky

Hussain

SMA

MacCarthy-Ofosu

, et al. The Role of Thoracic Endovascular Aortic Repair (TEVAR) of thoracic aortic diseases in patients with connective tissue disorders—a literature review. Braz J Cardiovasc Surg. 2020;35(6):977–985.