European Journal of Echocardiography 2008 9(1):173-174; doi:10.1016/j.euje.2007.07.004
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2007. For permissions please email: journals.permissions@oxfordjournals.org
Giant ruptured sinus of Valsalva aneurysm: diagnosis on echocardiography
Suneil Kumar Aggarwal*,
Ramachandra Barik,,
Jai Kumar Agarwal,,
Vijay Sai and
V. Ramnath Iyer
Department of Cardiology, Sri Sathya Sai Institute of Higher Medical Sciences, Prashantigram, AP 515134, India
Received 14 June 2007; accepted after revision 16 July 2007; online publish-ahead-of-print 10 September 2007.
* Corresponding author. Tel: +91 9440 699299; fax: +91 8555 287181. E-mail address: suneilaggarwal{at}doctors.net.uk
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Abstract
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A giant ruptured sinus of Valsalva aneurysm was diagnosed on
transthoracic and subsequent transesophageal echocardiography,
in a 45-year-old man who presented with gradual onset shortness
of breath. Although the initial presentation was insidious,
he later rapidly deteriorated. We discuss the unusual clinical
course in a patient with such a large aneurysm and discuss the
likely reasons.
Keywords: Congenital heart disease; Rupture; Aneurysm
A 45-year-old man presented with a three-month history of atypical
chest pain and exertional dyspnea. He was noted to have a grade
four continuous murmur, loudest over the right 4th intercostal
space. A chest X-ray showed cardiomegaly, widening of the mediastinum
and pulmonary plethora. Transthoracic echocardiography appeared
to show a very large aneurysm of the non-coronary sinus of Valsalva,
which protruded into the right atrial cavity. (
Figure 1/Movie
clip 1). There was a small rupture of the aneurysm into the
right atrium. These findings were clarified on transesophageal
echocardiography (
Figure 2, Movie clips 2 and 3). Cardiac
catheterization was performed, which showed a step-up in oxygen
saturations from 76% (mixed venous) to 90% in the right atrium
(left-to-right shunt of 2.4:1) and an elevated right atrial
pressure of 20 mmHg. There was mild pulmonary arterial hypertension
(mean pulmonary arterial pressure 27 mmHg). Angiography further
demonstrated a giant aneurysm of the non-coronary sinus of Valsalva
(
Figure 3, Movie clip 3).
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Figure 1 Transthoracic echocardiograms showing: (A) parasternal short-axis view of the giant sinus of Valsalva aneurysm (ANEU) as it bulges into the right atrium (RA) and (B) modified apical 5-chamber view showing colour Doppler flow from the aneurysm into the right atrium, with the jet hitting the atrial free wall (black arrow). AO, aorta; LV, left ventricle; RV, right ventricle.
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Figure 2 Transesophageal echocardiogram showing the large aneurysm (ANEU) of the non-coronary sinus, with rupture into the right atrium (RA): (A) without colour Doppler and (B) with colour Doppler. LA, left atrium.
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Unfortunately the patient developed a chest infection and so
his operation was postponed for a few days. He then suddenly
went into gross right sided heart failure, although repeat echocardiography
failed to demonstrate worsening of the rupture. He needed stabilization
with fluid, inotropes and cautious use of diuretics. Although
he became haemodynamically stable once again, the risk of operation
had considerably increased, with echocardiography showing severe
right ventricular dysfunction. After consultation with the cardiologists
and surgeons, the patient opted for medical therapy alone and
was discharged to the care of his local physician.
Aneurysms of the sinuses of Valsalva comprise about 1% of congenital heart disease.1 They most commonly arise from the right coronary sinus (65–85%), also from the non-coronary sinus (10–30%) and rarely the left coronary sinus (<5%).2 The primary concern is of rupture and this is most commonly into the right ventricle (90%), occasionally into the right atrium (10%) and rarely into the left heart chambers or pericardium. However, the aneurysm and rupture are usually small and take on the appearance of a ‘windsock.’ Previous cases of giant aneurysms which have ruptured have required immediate surgical intervention.3 Our case demonstrates that even with a giant aneurysm of one of the sinuses of Valsalva, the clinical course of rupture may be insidious and survival may be possible without emergency intervention. However, the further developments in this case exhibit the importance of not delaying surgery once a diagnosis has been made, due to the risk of developing further complications.
Echocardiography, particularly transesophageal, appears to be the imaging modality of choice in detailing the anatomy of this rare condition.
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Supplementary material
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Supplementary material associated with this article can be found in the online version.
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Acknowledgment
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Thank you to Dr Sanjay K Prasad, Royal Brompton Hospital, London
for his helpful advice during preparation of this image.
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References
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- Perloff JK. Congenital aneurysms of the sinuses of Valsalva. In: Clinical recognition of congenital heart disease (2003) 5th ed. Philadelphia: Saunders. 457.
- Fishbein MC, Obma R, Roberts WC. Unruptured sinus of Valsalva aneurysm. Am J Cardiol (1975) 35:918–22.[CrossRef][Web of Science][Medline]
- Ritter M, Oechslin E, Jenni R. Giant congenital aneurysm of the non-coronary sinus of Valsalva. Heart (2002) 88:243.[Free Full Text]
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