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

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.

Keywords

sinus of Valsalva aneurysms / Marfan syndrome / aortic dissection

References

[1] Ho SY. Structure and anatomy of the aortic root. Eur J Echocardiogr2009; 10(1): i3–i10

[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

[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

[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

[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

[7] Dong C, Wu QY, Tang Y. Ruptured sinus of valsalva aneurysm: a Beijing experience. Ann Thorac Surg2002; 74(5): 1621–1624

[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

[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

[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)

[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

[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

[13] Dobrilovic N, Fernandez AB, Lin A, Singh AK. Treacher Collins syndrome: sinus of Valsalva aneurysm. Circulation2013; 128(2): e12–e13

[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

[16] Erdheim J. Medionecrosis aortae idiopathica. Virchows Arch1929; 273(2): 454–479 (in German)

[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

[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

[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

[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

[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

[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

[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

[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

[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

[27] Milewicz DM, Dietz HC, Miller DC. Treatment of aortic disease in patients with Marfan syndrome. Circulation2005; 111(11): e150–e157

[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

[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

[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

[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

[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

[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

RIGHTS & PERMISSIONS

Higher Education Press and Springer-Verlag Berlin Heidelberg