Aneurysmal dilatation of the aortic sinuses of Valsalva — beyond Marfan syndrome: a single centre experience and review of the literature

doi:10.1007/s11684-014-0383-6

Frontiers of Medicine

2014, Vol. 8

Issue (4): 419-426 https://doi.org/10.1007/s11684-014-0383-6

本期目录

Aneurysmal dilatation of the aortic sinuses of Valsalva — beyond Marfan syndrome: a single centre experience and review of the literature

Maryanne Caruana^1,^*(

),Mary N. Sheppard²,Wei Li¹

1. Department of Cardiology, Mater Dei Hospital, Birkirkara Bypass, Birkirkara MSD 2090, Malta
2. Imperial Cardiac Pathology Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK
3. Adult Congenital Heart Disease Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK

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Abstract：

Aneurysmal dilatation of the aortic sinuses of Valsalva has been most extensively documented in the setting of aortopathies, particularly Marfan syndrome. On the other hand, there is limited data in the literature about congenital sinus of Valsalva aneurysms outside this context. For the purpose of this review, we carried out a literature search on aneurysmal dilatation of the sinuses of Valsalva in Marfan syndrome, and compared this with congenital sinus of Valsalva aneurysms, also including data from a case series from our institution. In conclusion, there are differences in management of aortic dilatation in Marfan syndrome and congenital sinus of Valsalva aneurysms. Though less well-recognised, congenital aneurysms are often associated with significant morbidity and mortality and timely intervention is necessary.

Key words： sinus of Valsalva aneurysms Marfan syndrome aortic dissection

收稿日期: 2014-08-09 出版日期: 2014-12-18

Corresponding Author(s): Maryanne Caruana

引用本文:

. [J]. Frontiers of Medicine, 2014, 8(4): 419-426.
Maryanne Caruana,Mary N. Sheppard,Wei Li. Aneurysmal dilatation of the aortic sinuses of Valsalva — beyond Marfan syndrome: a single centre experience and review of the literature. Front. Med., 2014, 8(4): 419-426.

链接本文:

https://academic.hep.com.cn/fmd/CN/10.1007/s11684-014-0383-6
https://academic.hep.com.cn/fmd/CN/Y2014/V8/I4/419

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1	Ho SY. Structure and anatomy of the aortic root. Eur J Echocardiogr2009; 10(1): i3–i10 https://doi.org/10.1093/ejechocard/jen243 pmid: 19131496
2	Bricker AO, Avutu B, Mohammed TLH, Williamson EE, Syed IS, Julsrud PR, Schoenhagen P, Kirsch J. Valsalva sinus aneurysms: findings at CT and MR imaging. Radiographics2010; 30(1): 99– 110 https://doi.org/10.1148/rg.301095719 pmid: 20083588
3	Caruana M, Sheppard M, Li W. Sinus of Valsalva aneurysms in non-Marfan patients: an underestimated entity? A case series. Br J Med Med Res2014; 4(7): 1448–1459 https://doi.org/10.9734/BJMMR/2014/7057
4	Loeys BL, Dietz HC, Braverman AC, Callewaert BL, De Backer J, Devereux RB, Hilhorst-Hofstee Y, Jondeau G, Faivre L, Milewicz DM, Pyeritz RE, Sponseller PD, Wordsworth P, De Paepe AM. The revised Ghent nosology for the Marfan syndrome. J Med Genet2010; 47(7): 476–485 https://doi.org/10.1136/jmg.2009.072785 pmid: 20591885
5	Hope J. A Treatise on the Diseases of the Heart and Great Vessels, and on the Affections which May be Mistaken for Them. 3rd ed. Churchill. 1839
6	Mayer ED, Ruffmann K, Saggau W, Butzmann B, Bernhardt-Mayer K, Schatton N, Schmitz W. Ruptured aneurysms of the sinus of Valsalva. Ann Thorac Surg1986; 42(1): 81–85 https://doi.org/10.1016/S0003-4975(10)61842-5 pmid: 3729620
7	Dong C, Wu QY, Tang Y. Ruptured sinus of valsalva aneurysm: a Beijing experience. Ann Thorac Surg2002; 74(5): 1621–1624 https://doi.org/10.1016/S0003-4975(02)03987-5 pmid: 12440619
8	Takach TJ, Reul GJ, Duncan JM, Cooley DA, Livesay JJ, Ott DA, Frazier OH. Sinus of Valsalva aneurysm or fistula: management and outcome. Ann Thorac Surg1999; 68(5): 1573–1577 https://doi.org/10.1016/S0003-4975(99)01045-0 pmid: 10585023
9	Moustafa S, Mookadam F, Cooper L, Adam G, Zehr K, Stulak J, Holmes D. Sinus of Valsalva aneurysms—47 years of a single center experience and systematic overview of published reports. Am J Cardiol2007; 99(8): 1159–1164 https://doi.org/10.1016/j.amjcard.2006.11.047 pmid: 17437748
10	Nenci I, Malaguti R. Congenital aneurysm of Valsalva’s sinus in the Klippel-Feil syndrome. Riv Patol Clin Sper1967; 8(4): 493–517 (in Italian) pmid: 5605968
11	Kawano Y, Tamura A, Kadota J. Klippel-Feil syndrome accompanied by an aneurysm of the non-coronary sinus of Valsalva. Intern Med2006; 45(20): 1191–1192 https://doi.org/10.2169/internalmedicine.45.6135 pmid: 17106170
12	Roussin I, Sheppard MN, Rubens M, Kaddoura S, Pepper J, Mohiaddin RH. Cardiovascular complications of cutis laxa syndrome: successful diagnosis and surgical management. Circulation2011; 124(1): 100–102 https://doi.org/10.1161/CIRCULATIONAHA.111.025056 pmid: 21730323
13	Dobrilovic N, Fernandez AB, Lin A, Singh AK. Treacher Collins syndrome: sinus of Valsalva aneurysm. Circulation2013; 128(2): e12–e13 https://doi.org/10.1161/CIRCULATIONAHA.113.001588 pmid: 23836833
14	Dias R, Camurca F, Leite Filho, Stolf N. Right sinus of Valsalva aneurysm causing extrinsic coronary compression. Arg Bras Cardiol2009; 92(6): e47–50, e74–7
15	Schlatmann TJ, Becker AE. Pathogenesis of dissecting aneurysm of aorta. Comparative histopathologic study of significance of medial changes. Am J Cardiol1977; 39(1): 21–26 https://doi.org/10.1016/S0002-9149(77)80005-2 pmid: 831424
16	Erdheim J. Medionecrosis aortae idiopathica. Virchows Arch1929; 273(2): 454–479 (in German) https://doi.org/10.1007/BF02158989
17	Davies RR, Gallo A, Coady MA, Tellides G, Botta DM, Burke B, Coe MP, Kopf GS, Elefteriades JA. Novel measurement of relative aortic size predicts rupture of thoracic aortic aneurysms. Ann Thorac Surg2006; 81(1): 169–177 https://doi.org/10.1016/j.athoracsur.2005.06.026 pmid: 16368358
18	Meijboom LJ, Timmermans J, Zwinderman AH, Engelfriet PM, Mulder BJM. Aortic root growth in men and women with the Marfan’s syndrome. Am J Cardiol2005; 96(10): 1441–1444 https://doi.org/10.1016/j.amjcard.2005.06.094 pmid: 16275195
19	Nienaber C, Haverich A, Erbel R. Diseases of the aorta and trauma to the aorta and the heart. In: Camm J, Luscher T, Serruys P. The ESC Textbook of Cardiovascular Medicine. Oxford: Blackwell Publishing Ltd, 2006: 993–1031
20	Lijoi A, Parodi E, Passerone GC, Scarano F, Caruso D, Iannetti MV. Unruptured aneurysm of the left sinus of valsalva causing coronary insufficiency: case report and review of the literature. Tex Heart Inst J2002; 29(1): 40–44 pmid: 11995849
21	Pepper C, Munsch C, Sivananthan UM, Pye M. Unruptured aneurysm of the left sinus of Valsalva extending into the left ventricular outflow tract: presentation and imaging. Heart1998; 80(2): 190–193 https://doi.org/10.1136/hrt.80.2.190 pmid: 9813568
22	Thankachen R, Gnanamuthu R, Doshi H, Shukla V, Korula RJ. Unruptured aneurysm of the sinus of Valsalva presenting with right ventricular outflow obstruction. Tex Heart Inst J2003; 30(2): 152–154 pmid: 12809262
23	Haraphongse M, Ayudhya RK, Jugdutt B, Rossall RE. Isolated unruptured sinus of Valsalva aneurysm producing right ventricular outflow obstruction. Cathet Cardiovasc Diagn1990; 19(2): 98–102 https://doi.org/10.1002/ccd.1810190208 pmid: 2407367
24	Pasteuning WH, Roukema JA, van Straten AHM, van der MA, Wonnink-de Jonge WF. Rapid hemodynamic deterioration because of acute rupture of an aneurysm of the sinus of Valsalva: the importance of echocardiography in early diagnosis. J Am Soc Echocardiogr2002; 15(10): 1108–1110 https://doi.org/10.1067/mje.2002.122964 pmid: 12373255
25	Devereux RB, de Simone G, Arnett DK, Best LG, Boerwinkle E, Howard BV, Kitzman D, Lee ET, Mosley TH Jr, Weder A, Roman MJ. Normal limits in relation to age, body size and gender of two-dimensional echocardiographic aortic root dimensions in persons≥15 years of age. Am J Cardiol2012; 110(8): 1189–1194 https://doi.org/10.1016/j.amjcard.2012.05.063 pmid: 22770936
26	Baumgartner H, Bonhoeffer P, De Groot NMS, de Haan F, Deanfield JE, Galie N, Gatzoulis MA, Gohlke-Baerwolf C, Kaemmerer H, Kilner P, Meijboom F, Mulder BJ, Oechslin E, Oliver JM, Serraf A, Szatmari A, Thaulow E, Vouhe PR, Walma E, Vahanian A, Auricchio A, Bax J, Ceconi C, Dean V, Filippatos G, Funck-Brentano C, Hobbs R, Kearney P, McDonagh T, Popescu BA, Reiner Z, Sechtem U, Sirnes PA, Tendera M, Vardas P, Widimsky P, McDonagh T, Swan L, Andreotti F, Beghetti M, Borggrefe M, Bozio A, Brecker S, Budts W, Hess J, Hirsch R, Jondeau G, Kokkonen J, Kozelj M, Kucukoglu S, Laan M, Lionis C, Metreveli I, Moons P, Pieper PG, Pilossoff V, Popelova J, Price S, Roos-Hesselink J, Uva MS, Tornos P, Trindade PT, Ukkonen H, Walker H, Webb GD, Westby J; Task Force on the Management of Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC); Association for European Paediatric Cardiology (AEPC); ESC Committee for Practice Guidelines (CPG). ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J2010; 31(23): 2915–2957 https://doi.org/10.1093/eurheartj/ehq249 pmid: 20801927
27	Milewicz DM, Dietz HC, Miller DC. Treatment of aortic disease in patients with Marfan syndrome. Circulation2005; 111(11): e150–e157 https://doi.org/10.1161/01.CIR.0000155243.70456.F4 pmid: 15781745
28	Rossi-Foulkes R, Roman MJ, Rosen SE, Kramer-Fox R, Ehlers KH, O’Loughlin JE, Davis JG, Devereux RB. Phenotypic features and impact of beta blocker or calcium antagonist therapy on aortic lumen size in the Marfan syndrome. Am J Cardiol1999; 83(9): 1364–1368 https://doi.org/10.1016/S0002-9149(99)00101-0 pmid: 10235096
29	Shores J, Berger KR, Murphy EA, Pyeritz RE. Progression of aortic dilatation and the benefit of long-term beta-adrenergic blockade in Marfan’s syndrome. N Engl J Med1994; 330(19): 1335–1341 https://doi.org/10.1056/NEJM199405123301902 pmid: 8152445
30	Gersony DR, McClaughlin MA, Jin Z, Gersony WM. The effect of beta-blocker therapy on clinical outcome in patients with Marfan’s syndrome: a meta-analysis. Int J Cardiol2007; 114(3): 303–308 https://doi.org/10.1016/j.ijcard.2005.11.116 pmid: 16831475
31	Brooke BS, Habashi JP, Judge DP, Patel N, Loeys B, Dietz HC 3rd. Angiotensin II blockade and aortic-root dilation in Marfan’s syndrome. N Engl J Med2008; 358(26): 2787–2795 https://doi.org/10.1056/NEJMoa0706585 pmid: 18579813
32	Groenink M, den Hartog AW, Franken R, Radonic T, de Waard V, Timmermans J, Scholte AJ, van den Berg MP, Spijkerboer AM, Marquering HA, Zwinderman AH, Mulder BJ. Losartan reduces aortic dilatation rate in adults with Marfan syndrome: a randomized controlled trial. Eur Heart J2013; 34(45): 3491–3500 https://doi.org/10.1093/eurheartj/eht334 pmid: 23999449
33	Mullen MJ, Flather MD, Jin XY, Newman WG, Erdem G, Gaze D, Valencia O, Banya W, Foley CE, Child A. A prospective, randomized, placebo-controlled, double-blind, multicenter study of the effects of irbesartan on aortic dilatation in Marfan syndrome (AIMS trial): study protocol. Trials2013; 14(1): 408 https://doi.org/10.1186/1745-6215-14-408 pmid: 24289736

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