European Journal of Echocardiography 2008 9(2):311-313; doi:10.1016/j.euje.2006.11.009
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2007. For permissions please email: journals.permissions@oxfordjournals.org.
Ruptured sinus of Valsalva aneurysm associated with noncompaction of the ventricular myocardium
Murat Unlu1,*,
Ozcan Ozeke1,
Muammer Kara2 and
Sait Yesillik2
1 Diyarbakir Military Hospital, Cardiology, 21100 Diyarbakir, Turkey
2 Diyarbakir Military Hospital, Internal Medicine, Diyarbakir, Turkey
Received 4 September 2006; accepted after revision 26 November 2006.
* Corresponding author. Tel: +90 505 526 65 31. E-mail address: drmuratunlu{at}gmail.com.
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Abstract
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Rupture of a sinus of Valsalva aneurysm is a rare, but life-threatening
cardiac abnormality that requires surgical correction when diagnosed,
and is frequently associated with other congenital defects,
particularly with ventricular septal defect, aortic valve regurgitation,
and bicuspid aortic valve. We present the case of a 21-year-old
man who had a ruptured aneurysm of the noncoronary sinus into
the right atrium, a ventricular septal defect, a persistent
left superior vena cava and a noncompaction of the ventricular
myocardium diagnosed by two-dimensional echocardiography. Surgical
repair was carried out and the patient made an uneventful recovery.
Keywords: Ruptured sinus of Valsalva aneurysm; Noncompaction of the ventricular myocardium
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Case report
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A 20-year-old man, under evaluation by the internal medicine
clinic for anemia, was referred to echocardiography because
of his continuous murmur. He was well until 1 year earlier when,
exertional dyspnea and generalized fatigue developed after a
fall from a height. On examination, the patient was thin and
appeared pallor. His blood pressure was 110/65 mmHg, and his
pulse rate was 84 bpm. A loud, harsh, continuous grade 4/6 murmur
was audible near the right sternal border. On transthoracic
echocardiography, the left and right ventricular dimensions
and functions were normal. A ruptured sinus of Valsalva aneurysm
(SVA) into the right atrium with a typical ‘windsock’
appearance was detected at the parasternal short axis (
Figure 1A,
arrow) and modified apical four-chamber views (
Figure 2B,
arrow). Doppler echocardiography showed an abnormal flow consistent
with rupture of a noncoronary SVA into the right atrium and
detected a 100-mmHg peak gradient between the aorta and the
right atrium. The ventricular septal defect (VSD) was also seen
at the modified parasternal short axis (
Figure 1B, immediately
below the aortic valve) and the apical four-chamber views (
Figure 2A,
arrow). The coronary sinus was dilated (
Figures 2B and 3A, black arrow). In order to reveal the cause of coronary sinus
dilation an agitated saline injection was given into the left
antecubital vein. The contrast entered first into the coronary
sinus and subsequently appeared in the right atrium. The typical
prominent trabeculations and intertrabecular recesses, which
are pathognomonic for ventricular noncompaction were also seen
on echocardiogram (
Figure 3A,B). In view of the aforementioned
echocardiographic findings, the diagnoses of the ruptured SVA,
the VSD, the persistent left superior vena cava and the noncompaction
of the ventricular myocardium were made, and the patient was
referred to cardiovascular surgery for corrective surgery. The
thin-walled aneurysmal portion was resected, and communication
between the aorta and the right atrium was closed by a Dacron
patch. The VSD was also closed with a patch. The patient made
an uneventful recovery and was discharged from the hospital
5 days after surgery.
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Figure 1 The standart (A) and modified (B, immediately below the aortic valve) parasternal short axis view echocardiogram showing the ruptured SVA with typical windsock appearance (arrow in A) and the VSD (arrow in B). LA, left atrium; RA, right atrium; RVOT, right ventricular outflow tract; PA, pulmonary artery; and Ao, Aort.
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Figure 2 The standart (A) and modified (B, with inferior angulation) apical four-chamber view echocardiogram showing the VSD (arrow in A) and the ruptured SVA (arrow in B). LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle; and Cs, coronary sinus.
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Figure 3 The modified apical long axis (A) and parasternal short axis at the apical level (B) echocardiogram showing prominent trabeculations and intertrabecular recesses (white arrows) and dilated coronary sinus (black arrow). LA, left atrium; LV, left ventricle; and Cs, coronary sinus.
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Discussion
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Ruptured SVA is a rare, but life-threatening cardiac abnormality
that requires surgical correction when diagnosed, and is frequently
associated with other congenital defects, particularly with
VSD, aortic valve regurgitation, bicuspid aortic valve and less
commonly pulmonary stenosis, coarctation, left superior vena
cava and atrial septal defect.
1 The clinical presentations vary
from asymptomatic to progressive heart failure following rupture
of the aneurysm into an adjacent cardiac chamber depending mainly
upon the rapidity of the rupture, the size of the ruptured aneurysm
and the chamber into which it ruptures.
2
Noncompaction of the ventricular myocardium is a rare congenital cardiac disorder characterised by an excessively prominent trabeculations in ventricular wall segments and deep intratrabecular recesses.3 Currently, transthoracic echocardiography is the most beneficial noninvasive diagnostic modality of choice for both ruptured SVA and noncompaction of the ventricular myocardium. The aetiology of SVA is usually a congenital defect in aortic media tissue and abnormal development of bulbous cordis.4 Noncompaction of the ventricular myocardium is also thought to be caused by arrest of normal embryogenesis of the myocardium.3 In presented case, their coexistence may be coincidental, but, it may reflect the common congenital pathway in origin.
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References
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- Sakakibara S, Konno S. Congenital aneurysm of the sinus of Valsalva. Anatomy and classification. Am Heart J (1962) 63:405–24.[CrossRef][Web of Science][Medline]
- Ozeke O, Tufekcioglu O, Geyik B, Yildiz A, Yetim M. Asymptomatic ruptured sinus of Valsalva aneurysm into the right ventricle. Eur J Echocardiogr (2005) 6:64.[Medline]
- Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R. Isolated noncompaction of left ventricular myocardium: a study of eight cases. Circulation (1990) 82:507–13.[Abstract/Free Full Text]
- Edwards JE, Burchell HB. The pathological anatomy of deficiencies between the aortic root and the heart, including aortic sinus aneurysms. Thorax (1957) 12:125–39.[Free Full Text]
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Abstract
Case report