Top

Published in:

Open Access 01-12-2021 | Aortic Valve Replacement | Case report

Behçet’s disease: successful aortic root reconstruction in severely dilated aortoventricular junction after aortic valve replacement with novel surgical method – case report

Authors: Miklós Pólos, Ádám Koppányi, Kálmán Benke, László Daróczi, Attila Oláh, Krisztina Heltai, Emese Kiss, Attila Fintha, Beáta Nagy, István Hartyánszky, Bálint Lakatos, Attila Kovács, Béla Merkely, Zoltán Szabolcs

Published in: Journal of Cardiothoracic Surgery | Issue 1/2021

Abstract

Background

Behçet’s disease is an auto-inflammatory disorder categorized as a primer systemic vasculitis of unknown aetiology. Genetic factors, infectious agents and the irregularity of T-cell homeostasis are presumed to be responsible for the emergence of Behçet’s disease. Characteristic symptoms are multisystemic. Although cardiovascular involvement is rare, it should be noted due to the difficulty of surgical treatment options.

Case presentation

Our 44-year-old male patient underwent aortic valve replacement due to aortic regurgitation. At the 15-month follow-up, echocardiography showed detachment of the prosthetic valve and in the aortic root, multiple pseudo-aneurysms were identified. We performed an aortic root reconstruction with a Bentall procedure using a special „skirted” conduit to reduce strain in the suture line between the conduit and the extremely dilated left ventricular outflow tract.

Conclusions

The surgical treatment of cardiovascular manifestations of Behçet’s disease remains challenging. This new technique may be beneficial in well-selected cases where the annulus of the aorta is extremely dilated or annular tissue disorder is present.

Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised international Chapel Hill consensus conference nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11. https://doi.org/10.1002/art.37715.CrossRefPubMed

International Team for the Revision of the International Criteria for Behcet's D. The international criteria for Behcet's disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol. 2014;28(3):338–47. https://doi.org/10.1111/jdv.12107.CrossRef

Poppas A, Coady M. Echocardiographic findings and cardiac surgical implications of aortitis and valvulitis in Behcet's disease. J Am Soc Echocardiogr. 2009;22(11):1275–8. https://doi.org/10.1016/j.echo.2009.09.017.CrossRefPubMed

Alpsoy E. Behcet's disease: a comprehensive review with a focus on epidemiology, etiology and clinical features, and management of mucocutaneous lesions. J Dermatol. 2016;43(6):620–32. https://doi.org/10.1111/1346-8138.13381.CrossRefPubMed

Bulur I, Onder M. Behcet disease: new aspects. Clin Dermatol. 2017;35(5):421–34. https://doi.org/10.1016/j.clindermatol.2017.06.004.CrossRefPubMed

Greco A, De Virgilio A, Ralli M, Ciofalo A, Mancini P, Attanasio G, et al. Behcet's disease: new insights into pathophysiology, clinical features and treatment options. Autoimmun Rev. 2018;17(6):567–75. https://doi.org/10.1016/j.autrev.2017.12.006.CrossRefPubMed

Criteria for diagnosis of Behcet's disease. International Study Group for Behcet's Disease. Lancet. 1990;335(8697):1078–80.

Jung Y, Ahn BH, Lee KS, Jeong IS, Kim KH, Na KJ, et al. A novel solution to prosthetic valve dehiscence after aortic valve surgery in Behcet's disease. Interact Cardiovasc Thorac Surg. 2017;24(3):342–7. https://doi.org/10.1093/icvts/ivw361.CrossRefPubMed

Takayama T, Ozawa T, Sanada A, Watanabe T, Ito M, Hirono S, et al. Aortic regurgitation presenting with recurrent detachment of a prosthetic valve, as the first presenting symptom of cardiovascular Behcet's disease. Intern Med. 2018;57(6):823–7. https://doi.org/10.2169/internalmedicine.9603-17.CrossRefPubMed

10.

Reder RF, Dimich I, Steinfeld L, Litwak RS. Left ventricle to aorta valved conduit for relief of diffuse left ventricular outflow tract obstruction. Am J Cardiol. 1977;39(7):1068–72. https://doi.org/10.1016/S0002-9149(77)80223-3.CrossRefPubMed

11.

Ghang B, Kim JB, Jung SH, Chung CH, Lee JW, Song JM, et al. Surgical outcomes in Behcet's disease patients with severe aortic regurgitation. Ann Thorac Surg. 2019;107(4):1188–94. https://doi.org/10.1016/j.athoracsur.2018.08.040.CrossRefPubMed

12.

Jeong DS, Kim KH, Kim JS, Ahn H. Long-term experience of surgical treatment for aortic regurgitation attributable to Behcet's disease. Ann Thorac Surg. 2009;87(6):1775–82. https://doi.org/10.1016/j.athoracsur.2009.03.008.CrossRefPubMed

13.

Azuma T, Yamazaki K, Saito S, Kurosawa H. Aortic valve replacement in Behcet's disease: surgical modification to prevent valve detachment. Eur J Cardiothorac Surg. 2009;36(4):771–2. https://doi.org/10.1016/j.ejcts.2009.05.031.CrossRefPubMed

Title: Behçet’s disease: successful aortic root reconstruction in severely dilated aortoventricular junction after aortic valve replacement with novel surgical method – case report
Authors: Miklós Pólos
Ádám Koppányi
Kálmán Benke
László Daróczi
Attila Oláh
Krisztina Heltai
Emese Kiss
Attila Fintha
Beáta Nagy
István Hartyánszky
Bálint Lakatos
Attila Kovács
Béla Merkely
Zoltán Szabolcs
Publication date: 01-12-2021
Publisher: BioMed Central
Keywords: Aortic Valve Replacement
Echocardiography
Aneurysm
Aneurysm
Echocardiography
Aortic Valve Replacement
Aortic Valve Replacement
Published in: Journal of Cardiothoracic Surgery / Issue 1/2021
Electronic ISSN: 1749-8090
DOI: https://doi.org/10.1186/s13019-021-01467-1

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Behçet’s disease: successful aortic root reconstruction in severely dilated aortoventricular junction after aortic valve replacement with novel surgical method – case report

Abstract

Background

Case presentation

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Case presentation

Conclusions

Please log in to get access to this content

Other articles of this Issue 1/2021

Percutaneous transluminal stenting for superior vena cava syndrome caused by malignant tumors: a single-center retrospective study

Severe hemolytic anemia and acute renal failure after mitral valve repair associated with non-endothelialization of artificial chordae tendinae: case report

Left coronary button aneurysm presenting with progressive enlargement after aortic root replacement

Systematic literature review evaluating evidence and mechanisms of action for platelet-rich plasma as an antibacterial agent

The susceptibility of the aortic root: porcine aortic rupture testing under cardiopulmonary bypass

COVID-19 related mortality in post-operative cardiac surgical patients