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Frontiers of Medicine

ISSN 2095-0217

ISSN 2095-0225(Online)

CN 11-5983/R

Postal Subscription Code 80-967

2018 Impact Factor: 1.847

Front. Med.    2014, Vol. 8 Issue (4) : 419-426     DOI: 10.1007/s11684-014-0383-6
Aneurysmal dilatation of the aortic sinuses of Valsalva — beyond Marfan syndrome: a single centre experience and review of the literature
Maryanne Caruana1,*(),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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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     
Corresponding Authors: Maryanne Caruana   
Just Accepted Date: 06 November 2014   Online First Date: 01 December 2014    Issue Date: 18 December 2014
URL:     OR
Case No. Sex Age (year) SoV involved Findings on investigation Underlying congenital defects/ conditions Clinical presentation
1 M 29 RC 2 dissection tears above aortic valve+ hemopericardium “Connective tissue disorder” (non-Marfan) Sudden death
2 M 34 RC+ NC Dissection with tear in NC SoV+ hemopericardium - Sudden death
3 M 46 LC+ RC Dissection+ thrombus in LC SVA - Out of hospital cardiac arrest
Aneurysm rupture
4 F 32 RC SVA rupture into RVOT BAV+ AR Heart failure
5 M 39 NC SVA rupture into RA BAV+ AR Heart failure
6 M 36 RC SVA rupture into RVOT Resected SAS Right-sided failure
7 M 46 RC SVA rupture into RV body+ RA - Heart failure
8 F 25 RC SVA rupture into RV body VSD (subaortic perimembranous) Heart failure
9 F 33 NC Ruptured NC SVA with hemopericardium Klippel-Feil syndrome & HT Sudden death
10 F 48 NC Small SVA rupture into RA - Asymptomatic
11 F 27 RC Small SVA rupture into RV body VSD (small muscular VSD) Asymptomatic
12 M 38 RC+ LC+ NC RCA stretched over aneurysm with no occlusion; no histological ventricular changes - Sudden death
Tab.1  Clinical manifestation of sinus of Valsalva aneurysmsa
Fig.1  Postmortem image for case 2 showing aneurysms of right coronary (RC) and non-coronary (NC) sinuses (labeled) with a dissection tear in the non-coronary sinus leading to hemopericardium (asterisk). (Reprinted from Ref. [3] with permission.)
Fig.2  Postmortem image for case 9 showing site of rupture (arrow) of the free wall of non-coronary sinus of Valsalva (NC) (labeled) resulting in a large hemopericardium. (Reprinted from Ref. [3] with permission.)
Fig.3  Two-dimensional transesophageal echocardiogram from case 3 showing aneurysmal dilatation of left coronary sinus with thrombus (arrow) in situ. (Reprinted from Ref. [3]. with permission.)
Fig.4  Computed tomographic angiogram with three-dimensional reconstruction from case 3 showing a large left coronary sinus of Valsalva aneurysm measuring 45mm in its maximum diameter and a smaller right coronary sinus aneurysm measuring 23mm in its maximum diameter (labeled) with stretching of the left coronary artery. (Reprinted from Ref. [3] with permission.)
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