European Journal of Echocardiography 2008 9(2):301-302; doi:10.1016/j.euje.2006.10.008
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2007. For permissions please email: journals.permissions@oxfordjournals.org
A multi-perforated man: Asymptomatic ruptured sinus of Valsalva aneurysm associated with an atrial and ventricular septal defect
David Attias1,
David Messika-Zeitoun1,*,
Agnès Cachier1,
Eric Brochet1,
Jean-Michel Serfaty2,
Jean-Pierre Laissy2,
Ulrik Hvass3 and
Alec Vahanian1
1 Cardiology Department, AP-HP, Bichat Hospital, 75018 Paris, France
2 Radiology Department, AP-HP, Bichat Hospital, Paris, France
3 Cardiac Surgery Department, AP-HP, Bichat Hospital, Paris, France
Received 10 August 2006; accepted after revision 30 October 2006; online publish-ahead-of-print 2 January 2007.
* Corresponding author. Tel: +33 01 40 25 66 01; fax: +33 0140 25 67 32. E-mail address: david.messika-zeitoun{at}bch.ap-hop-paris.fr
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Abstract
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We report the case of an exceptional association of a right
coronary sinus of Valsalva aneurysm (SVA) ruptured into the
right ventricle, a supracristal ventricular septal defect (VSD)
and an atrial septal defect (ASD). Our patient was totally asymptomatic
and the diagnosis was established by echocardiography. The patient
underwent prompt surgery that consisted in closing the aneurysm
and the VSD with a pericardium patch.
Keywords: Sinus of Valsalva aneurysm; Atrial septal defect; Ventricular septal defect; Echocardiography
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Case report
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A 26-year-old asymptomatic man with a known small supracristal
VSD and mild aortic regurgitation (AR) was referred to our institution
for progressive left ventricular (LV) enlargement. Transthoracic
and transesophageal echocardiography confirmed the LV enlargement
with normal LV systolic function. A large right SVA ruptured
into the right ventricular outflow tract was observed (
Figure 1).
There was also a mild eccentric AR directed toward the anterior
leaflet of the mitral valve due to a retraction of the right
cusp (
Figure 2). In addition to the known small VSD, a
small ASD was also found (
Figure 3). Computed tomography
confirmed the echocardiographic findings and showed that the
right coronary artery ostium was not involved by the aneurysm
(
Figure 4).
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Figure 1 Transesophageal echocardiography (long-axis view). Two-dimensional and colour view of the ruptured right sinus of Valsalva aneurysm (arrowhead). Ao: aorta, LA: left atrium, LV: left ventricle, RV: right ventricle.
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Figure 2 Transesophageal echocardiography (long-axis view). In this view both the mild eccentric aortic regurgitation directed to the anterior mitral valve (arrow) due to the retraction of the right cusp and the associated supracristal VSD (dashed arrow) are well visualized (arrowhead: right ruptured sinus of Valsalva aneurysm).
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The patient was promptly referred to surgery. Surgical findings
were a tricuspid aortic valve with thin cusps and a retraction
of the right cusp; a right sinus of Valsalva aneurysm with multiple
ruptures into the right ventricular outflow tract and a 5-mm
supracristal VSD. We decided not to operate on the aortic valve
and the small ASD. The septal ventricular defect was first closed.
A repair of the ruptured SVA with an autolog patch of pericardium
was performed.
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Discussion
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Sinus of Valsalva aneurysms are rare mostly congenital anomalies,
and predominates in young males.
1 Right sinus is the most common
localization of SVA (approximately 75% of cases).
1 SVA usually
enlarges over time and remains silent until they rupture. However,
unruptured SVA can cause complications such as right ventricular
outflow tract obstruction; coronary artery occlusion or compression
and aortic regurgitation. Due to thrombus formation inside the
aneurysm, embolic events have been reported.
The rupture of SVA may occur in all cardiac chambers preferentially into the right ventricle but also into the right atrium or into the pericardium. Clinical presentation varies according to the rupture location but rupture can remain silent, like in our patient. Echocardiography plays a key role in the diagnosis of ruptured SVA. Transthoracic and transoesophageal echocardiography determines the location and size of the rupture and can identify associated lesions.
There is a frequent association between SVA and VSD. Right SVA is most likely to be associated with supracristal VSD.1 A mild AR is often associated with SVA and VSD. Association of SVA with congenital aortic stenosis, aortic coarctation, coronary anomalies, bicuspid aortic valve, patent ductus arteriosus, mitral regurgitation, tricuspid regurgitation, and pulmonary artery stenosis have also been described. To the best of our knowledge, the association of ruptured SVA, ASD and VSD has only been reported once.2 Whether this is an incidental finding or a real association is unclear.
Early diagnosis of SVA is important because surgical cure is easier when performed promptly3 and provides better long-term results.4 Surgery consists of excision of the aneurysm and direct closure of the aneurismal base with a Gore-Tex or a pericardium patch. Recurrence of SVA after surgery is exceptional. Aortic valve replacement may be required in case of severe AR or impossibility of direct closure of the aneurismal base. In children, a percutaneous transcatheter closure of the aneurysm has been reported.5
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References
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- Babacan KM, Tasdemir O, Zengin M, Karagoz HY, Zorlutuna YI, Ozer C, et al. Fistulous communication of aortic sinuses into the cardiac chambers. Jpn Heart J (1986) 27:865–70. Fifteen years surgical experience and a report of 23 patients.[Medline]
- Grellner W, Karsch KR, Bultmann B. [Fatal outcome of a congenital aneurysm of the right sinus valsalvae ruptured into the right atrium]. Z Kardiol (1995) 84:553–9.[Web of Science][Medline]
- Au WK, Chiu SW, Mok CK, Lee WT, Cheung D, He GW. Repair of ruptured sinus of valsalva aneurysm: determinants of long-term survival. Ann Thorac Surg (1998) 66:1604–10.[Abstract/Free Full Text]
- Arora R, Trehan V, Rangasetty UM, Mukhopadhyay S, Thakur AK, Kalra GS. Transcatheter closure of ruptured sinus of valsalva aneurysm. J Interv Cardiol (2004) 17:53–8.[CrossRef][Medline]
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