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Interact CardioVasc Thorac Surg 2009;9:888-890. doi:10.1510/icvts.2009.215590
© 2009 European Association of Cardio-Thoracic Surgery

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Case report - Aortic and aneurysmal

Complicated ruptured sinus of Valsalva: cardiac computed tomographic angiography (64 slice) predicts surgical appearance and obviates need for invasive cardiac catheterization

Thomas K. Roa, Bruno R. Cottera, Sinan A. Simsirb and Ronald P. Karlsbergc,*

a University of San Diego School of Medicine, San Diego, CA, USA
b Cedars Sinai Medical Center, Los Angeles, CA, USA
c Cardiovascular Research Foundation of Southern California, 414 North Camden Drive, Beverly Hills, CA, USA

Received 1 July 2009; received in revised form 10 August 2009; accepted 12 August 2009

*Corresponding author. Tel.: +1 310 278 3400.

E-mail address: Karlsberg{at}cvmg.com (R.P. Karlsberg).


    Abstract
 Top
 Abstract
 1. Introduction
 2. Case report
 3. Discussion
 References
 
We present a case report of a ruptured sinus of Valsalva aneurysm (SVA) that presented as aortic insufficiency following bacterial endocarditits in a cardiac transplant patient. Cardiac computed tomographic angiography (CCTA) including volume rendered images predicted the appearance of the fistula entrance and defined spatial relationships facilitating the surgical approach. CCTA ability to define the coronary anatomy obviated the need for invasive coronary angiography. The use of this imaging modality especially with three-dimensional spatial visualization, and multiphase cine angiography can add significant value to the care of a patient with ruptured sinus of Valsalva.

Key Words: Cardiac computed tomographic angiography; CT angiography; Rupture sinus of Valsalva; Aorta; Aortic valve


    1. Introduction
 Top
 Abstract
 1. Introduction
 2. Case report
 3. Discussion
 References
 
A sinus of Valsalva aneurysm (SVA) is a rare entity [1]. The majority are congenital in origin secondary to incomplete fusion of the aortic media to the annulus fibrosis [2]. Less frequently, they may be acquired as sequelae of endocarditis, atherosclerosis, cystic medial necrosis, and trauma [3, 4]. The presentation may range from asymptomatic to acute, and rupture usually necessitates surgical repair. The diagnosis is generally made by transthoracic echocardiography [5] with confirmation by transesophageal echocardiography (TEE) or cardiac magnetic resonance imaging (MRI). More recently, cardiac computed tomographic angiography (CCTA) [6] in the evaluation of aortic valve disease has been a promising new modality.


    2. Case report
 Top
 Abstract
 1. Introduction
 2. Case report
 3. Discussion
 References
 
A 64-year-old man with a history of idiopathic dilated cardiomyopathy underwent orthotopic heart transplantation in 1998. One year later, he developed aortic valve bacterial endocarditis. A transesophageal echocardiogram revealed normal systolic function, mild aortic regurgitation, and aortic valve thickening consistent with vegetations. In addition, a 2.1x1.5 cm fluid collection adjacent to the right and non-coronary cusps thought to represent a perivalvular abscess was identified. Having refused surgical intervention, he was treated with intravenous antibiotics with subsequent resolution of his illness. In 2005, an outside surveillance echocardiogram, while remaining asymptomatic, revealed moderate aortic regurgitation and an ill-defined cavity coming off the base of the aortic root between the right and non-coronary cusps. This was attributed to chronic changes consistent with healed endocarditis.

In 2007, a screening chest radiograph resulted in an incidental finding of a radiodensity along the right heart border. Further evaluation by non-contrast helical computed tomography (CT) did not result in a diagnosis. A few months later, the patient was seen by us with complaints of increasing fatigue and generalized weakness. His dyspnea was exertional with moderate activity; however, he denied any orthopnea, paroxysmal nocturnal dyspnea, or chest pain. An echocardiogram revealed normal left ventricular function and size with severe aortic regurgitation. CCTA was performed for evaluation of the coronary arteries and previously reported ill-defined structure near the right heart border. This clearly revealed a ruptured sinus of Valsalva above the right and non-coronary cusps contained by a pseudoaneurysm (2x3 cm) and aorto-left ventricular outflow tract fistula. An intraoperative TEE showed central and perivalvular aortic regurgitation. Surgical findings confirmed the 20 mm diameter fistula tract between the left ventricular outflow just right of the right and non-coronary commissure tunneling under the non-coronary leaflet into the wall of the aorta. The non-coronary leaflet was prolapsed and untethered. An aortic bioprosthetic valve replacement and repair of the fistulous tract with a bovine pericardial patch was successfully performed.


    3. Discussion
 Top
 Abstract
 1. Introduction
 2. Case report
 3. Discussion
 References
 
A ruptured SVA requires a timely and accurate diagnosis, as a delay in intervention can have an impact on morbidity and mortality [1, 5, 7]. Most series have shown predominance of the right sinus of Valsalva involvement with rupture into the right ventricular outflow tract, right ventricle, and right atrium in diminishing incidence, respectively. Left SVA rupture is rare [5, 8, 9]. Echocardiography is the first tool in evaluation of cardiac structure and function. It is safe and readily available in nearly all centers; however, technical difficulties do arise in certain patient populations making complete evaluation impossible.

The emergence of CCTA is becoming an invaluable tool for both diagnoses and management decisions of cardiovascular problems [6, 10]. It provides a unique advantage by allowing detailed structural, functional, and coronary evaluation in one scan. Our case report is an example of a diagnosis made by cardiac CCTA leading to appropriate management that had failed to be clearly identified by other modalities.

Axial and coronal views depict the location of the ruptured coronary sinus and contained pseudoaneurysm. By multiphase cine it was apparent that the enclosed vascular mass connected inferior to the origin of the right coronary artery inferiorly along the aorta into a fistulous tract to the left ventricular outflow (Videos 1 and 2). The contained rupture had calcium and low-density material consistent with clot, suggesting some chronicity of the rupture and perhaps noting cautious clinical stability. The aortic valves appeared trileafet with abnormality of the non-coronary leaflet. A volume rendered image depicted the actual external appearance of the pseudoaneurysm in relative space to the right coronary artery and sinuses of Valsalva (Fig. 1). The CCTA volume rendered images accurately reflected the shape of the entrance of the fistula as seen from the aorta. The abnormality of the non-coronary leaflet at the time of surgery was found to be ‘floating’ similar to the volume rendered images and represent a new opportunity for predicting and staging surgical approaches (Fig. 2).


Figure 3
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Video 1. Axial oblique multiphase cine loop illustrates a contained ruptured sinus of Valsalva of the non-coronary leaflet with abnormal opening of the leaflets and aortic flow into the rupture tract.

 

Figure 4
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Video 2. Coronal multiphase cine loop of the contained pseudoaneurysm demonstrating a fistulous tract into the left ventricular outflow. Left ventricular function was preserved with normal EF and no regional wall motion abnormalities.

 

Figure 1
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Fig. 1. A volume rendered view of the rupture shows the course of the right coronary artery adjacent to the sinus Valsalva rupture and explains previous findings on chest X-ray of a right side cardiac mass.

 

Figure 2
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Fig. 2. A virtual endoscopic view reveals an accurate reconstruction compared to surgical pathology. The non-coronary leaflet is ‘floating in the breeze’, while the size, shape, and location of the fistula entrance is near identical to the pathologic photographed at the time of surgery.

 
The right and left coronary arteries were free of any stenosis, calcified or non-calcified plaque in this post-transplant patient and clarity and details of the coronary images precluded the need for invasive coronary angiography. The specificity and sensitivity of CCTA compared to invasive coronary angiography in the absence of artifact or calcium has the potential to be a replacement for invasive coronary angiography in select cases such as this one [10]. CCTA is safer than invasive coronary angiography by avoiding catheter manipulation of an aortic root containing a ruptured sinus and pseudoaneurysm. With this information at hand, both the cardiologist and the cardiac surgeon were able to conceive a precise course of action before the patient entered the doors of the operating room and even visualize the shape of the fistula and subtle abnormalities of the valve as seen from the aorta. The use of CCTA in this manner, especially with volume reconstruction, three-dimensional spatial visualization, and multiphase cine angiography can add significant value to the future of patient care with ruptured sinus of Valsalva.


    References
 Top
 Abstract
 1. Introduction
 2. Case report
 3. Discussion
 References
 

  1. 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 Cardiol 2007;99:1159–1164.[CrossRef][Medline]
  2. Edwards JE, Burchell HB. The pathological anatomy of deficiencies between aortic root and the heart including aortic sinus aneurysms. Thorax 1957;12:125–139.[Free Full Text]
  3. Conde CA, Meller J, Donoso E, Dack S. Bacterial endocarditis with ruptured sinus of Valsalva and aorticocardiac fistula. Am J Cardiol 1975;35:912–917.[CrossRef][Medline]
  4. Hurley J, McGovern E. Rupture of a sinus of Valsalva aneurysm due to Aspergillus endocarditis. J Cardiovasc Surg (Torino) 1994;35:75–77.[Medline]
  5. Shah RP, Ding ZP, Ng A, Quek A. Ten-year review of ruptured sinus of Valsalva: clinico-pathological and Echo-Doppler features. Singapore Med J 2001;42:473–476.[Medline]
  6. Gilkeson R, Markowitz A, Balgude A, Sachs P. CCTA evaluation of aortic valve disease. Am J Roentgenol 2006;186:350–360.[Abstract/Free Full Text]
  7. Brabram KR, Roberts WC. Fatal intrapericardial rupture of sinus of Valsalva aneurysm. Am Heart J 1990;120(6 Pt 1):1455–1456.[CrossRef][Medline]
  8. Guenther F, von Zur Muhlen C, Lohrmann J, Bode C, Geibel A. Rupture of an aneurysm of the noncoronary sinus of Valsalva into the right atrium. Eur J Echocardiogr 2008;9:186–187.[Abstract/Free Full Text]
  9. Park SH, Jung HS, Yu M, Min SK, Ahn JH, Kim Y. Left Valsalva sinus aneurysm rupture into left atrium and aortic valve prolapse confirmed with transesophageal echocardiography. J Am Soc Echocardiogr 2007;20:1010.e3–e6; Epub 2007 Jun 6.[CrossRef][Medline]
  10. Raff GL, Abidov A, Achenbach S, Berman DS, Boxt LM, Budoff MJ, Cheng V, DeFrance T, Hellinger JC, Karlsberg RP. Society of Cardiovascular Computed Tomography. SCCT guidelines for the interpretation and reporting of coronary computed tomographic angiography. J Cardiovasc Comput Tomogr 2009;3:138–139.[CrossRef][Medline]




This Article
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Sinan A. Simsir
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Right arrow Articles by Ro, T. K.
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Right arrow Cardiac - other
Right arrow Valve disease


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