Ann Thorac Surg 2006;82:1525-1527
© 2006 The Society of Thoracic Surgeons
Case Reports
Austrian Triad with Sinus of Valsalva Aneurysm and Rupture
John McLean Trotter, MDa,
Giorgio Aru, MDb,
Ervin R. Fox, MD, MPHa,*
a Department of Medicine, Jackson, Mississippi
b Department of Surgery, University of Mississippi Medical Center, Jackson, Mississippi
Accepted for publication January 11, 2006.
* Address correspondence to Dr Fox, Jackson Heart Study, University of MS Medical Center, 2500 North State Street, Jackson, MS 39216 (Email: efox{at}medicine.umsmed.edu).
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Abstract
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An interesting case of a young patient presenting with Austrian's Triad: pneumococcal pneumonia, pneumococcal meningitis, and pneumococcal endocarditis. On echocardiogram the patient was noted to have a noncoronary sinus of Valsalva aneurysm and vegetations on the aortic cusps resulting in disruption of valvular integrity and severe aortic insufficiency. Color Doppler also revealed rupture of the aneurysm into the right atrium. The patient was taken to surgery where the noncoronary cusp was noted to be completely replaced with vegetative lesions. The aortic valve was replaced with a No. 21 Carpentier-Edwards bioprosthetic valve (Edwards Lifesciences, Irvine, CA), and the noncoronary sinus of Valsalva was repaired with concomitant closure of the fistula using glutaraldehyde autologous pericardium.
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Introduction
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The triad of coexisting pneumococcal pneumonia, meningitis, and endocarditis is known as Austrian's triad. It often carries a very poor prognosis despite aggressive medical and surgical management. Sinus of Valsalva aneurysms are rare and mostly congenital structural abnormalities that may be associated with rupture, significant aortic insufficiency, conduction abnormalities, and encroachment on coronary arteries or on the right ventricular outflow tract. Different techniques are used to correct ruptured sinus of Valsalva aneurysms including resection and patch closure, excision, and direct suturing of the defect and suture ligation at the base of the aneurysm.
A 43-year-old African American man presented to the emergency department with complaints of weakness, cough, and altered mental status. The patient was febrile on physical examination with evidence of nuchal rigidity. In addition, he had a loud blowing diastolic murmur along the left sternal border. Auscultation of the lungs revealed decreased breath sounds and egophony in the right upper lung field. His chest roentgenogram confirmed right upper lobe pneumonia with a right-sided pleural effusion. Both cerebrospinal fluid and blood cultures grew heavy streptococcus pneumonia. The patient underwent transesophageal echocardiogram for endocarditis evaluation. On echocardiogram he was noted to have vegetations on the aortic cusps resulting in disruption of valvular integrity and severe aortic insufficiency (Figs 1A,
1B). Two-dimensional images of the aortic root also revealed a noncoronary sinus of Valsalva aneurysm with evidence of rupture into the right atrium on color Doppler (Figs 2A,
2B). Aggressive treatment with intravenous antibiotics was initiated and the patient was taken for urgent surgical valve replacement with concurrent repair of the aortic-atrial fistula. At surgery the noncoronary cusp was noted to be completely replaced with vegetative lesions; there was also noted to be a sinus of Valsalva aneurysm with an aortic-atrial fistula. The aortic valve was replaced with a No. 21 Carpentier-Edwards bioprosthetic valve (Edwards Lifesciences, Irvine, CA), and the noncoronary sinus was repaired with concomitant closure of the fistula using glutaraldehyde autologous pericardium.
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Fig 1. (A) Long-axis of the aortic root on transesophageal echocardiogram showing large vegetations on the aortic cusps. There is disruption of valvular integrity with mal-coaptation of the cusps in diastole. (B) Color Doppler across the aortic valve in diastole showing severe aortic insufficiency. On further imaging aortic insufficiency jets appeared to be both tranvalvular as well as through apparent rupture(s) in one or more of the cusps.
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Fig 2. (A) Short-axis of the aortic root at the level of the sinuses on transesophageal echocardiogram showing an aneurysm of the noncoronary sinus of Valsalva protruding into the right atrium. (B) Color Doppler across the noncoronary sinus of Valsalva aneurysm showing flow communication from the aorta into the right atrium through the aneurysm consistent with rupture.
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Comment
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Robert Austrian [1] first described the triad of pneumococcal pneumonia, meningitis, and endocarditis. Despite aggressive medical and surgical management, the Austrian triad continues to carry a high mortality rate. In a review of pneumococcal endocarditis in adults, Aronin and colleagues [2] report that Austrian's triad is present in 42% of the patients, and it carries a mortality rate greater than 50%.
Our patient had a sinus of Valsalva aneurysm (SVA) that may have predisposed him to aortic valve endocarditis and fistula formation. An SVA can be either focal (involving one sinus) or diffuse (involving more than one sinus) and account for 0.1% to 3.5% of all congenital heart defects typically arising from mural deficiencies within the aortic media or from outpouching defects in the sinus [3, 4]. Complications of an SVA include rupture, significant aortic insufficiency, conduction abnormalities, and encroachment on coronary arteries or on the right ventricular outflow tract.
A transesophageal echocardiogram is helpful in evaluating an SVA and allows for thorough assessment of the aortic root and surrounding structures. On transesophageal echocardiogram an SVA appears as filamentous or "windsock" structures protruding into adjacent cardiac chambers. Color flow Doppler facilitates detection and localization of a potential rupture or a fistula or both. The majority of SVAs involves the right coronary sinus; however an aneurysm of the noncoronary sinus occurs in approximately 5% to 15% of SVA patients [4].
Different surgical techniques are used to correct the defect in a ruptured SVA including resection and patch closure, excision, and direct suturing of the defect and suture ligation at the base of the aneurysm [5]. Accordingly in our case the noncoronary SVA was repaired with concomitant closure of the fistula using glutaraldehyde autologous pericardium. The patient had no perioperative surgical complications, his pneumonia and meningitis resolved, and he was discharged home in good condition on postoperative day 13.
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References
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- Austrian R. Pneumococcal endocarditis, meningitis, and rupture of the aortic valve Arch Int Med 1957;99:539-544.[Medline]
- Aronin SI, Mulcherjee SK, West JC, et al. Review of pneumococcal endocarditis in adults in the penicillin era Clin Infect Dis 1998;26:165-171.[Medline]
- Kakos GS, Kilmen JW, Williams TE, Hosier DM. Diagnosis and management of sinus of Valsalva aneurysm in children Ann Thorac Surg 1974;17:474.[Medline]
- Takach TJ, et al. Sinus of Valsalva aneurysm or fistulamanagement and outcome. Ann Thorac Surg 1999;68:1573-1577.[Abstract/Free Full Text]
- Burakovsky VI, Podsolkov VP, Sabirow BN, Nasedkina MA, Alekian BG, Dvinyaninova NB. Ruptured congenital aneurysm of the sinus of Valsalva: clinical manifestations, diagnosis, and results of surgical corrections J Thorac Cardiovasc Surg 1988;95:836-841.[Abstract]
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Abstract
Introduction
