Copyright © 2005, The European Society of Cardiology
Subaortic obstruction and complete atrioventricular block in Behçet's disease
aDepartment of Cardiology, Erasme Hospital, Université Libre de Bruxelles, 808 route de Lennik, B-1070 Brussels, Belgium
bDepartment of Cardiac Surgery, Erasme Hospital, Université Libre de Bruxelles, 808 route de Lennik, B-1070 Brussels, Belgium
cDepartment of Internal Medicine, Erasme Hospital, Université Libre de Bruxelles, 808 route de Lennik, B-1070 Brussels, Belgium
Received 17 February 2005; received in revised form 21 April 2005; accepted after revision 27 April 2005.
* Corresponding author. Tel.: +32 2555 3907; fax: +32 2555 4609. punger{at}ulb.ac.be
 |
Abstract |
|---|
 Top Abstract Case reportDiscussionReferences |
|---|
Left ventricular outflow tract obstruction may be dynamic, most commonly associated with hypertrophic cardiomyopathy, and, uncommonly, by congenital anomalies such as discrete subaortic stenosis. We describe a patient with Behçet's disease, presenting with a systolic murmur, fever, and syncope, in whom a diagnosis of subaortic obstruction caused by a pseudo-aneurysm dissecting the interventricular septum and associated with a complete atrioventricular block was made.
Keywords: Behçet's disease; Atrioventricular block; Subaortic obstruction
Behçet's disease is a rare chronic inflammatory disease of unknown etiology involving the small blood vessels.1 Sporadic cases with cardiac manifestations have been reported.2–7 We describe a patient with Behçet's disease presenting with a systolic murmur, fever, and syncope. A diagnosis of subaortic obstruction caused by a pseudo-aneurysm dissecting the interventricular septum associated with a complete atrioventricular (AV) block was made.
|
Case report |
|---|
|
TopAbstractCase reportDiscussionReferences |
|---|
A 41-year-old woman with Behçet's disease diagnosed 2 years earlier was hospitalized for relapsing fever and recurrent syncope. She reported a 3-month history of exercise intolerance and dyspnea. The patient had a several year history of erythema nodosum and recurrent oral aphthous ulcerations. Two years before admission, she had developed polyarthritis of the wrist, elbow, shoulder and knee, which did not respond to non-steroidal anti-inflammatory drugs, and methylprednisolone therapy had been started. Two months before admission, a deep vein thrombosis of the left calf and femoral veins had been diagnosed, and coumadin therapy had been started in addition to prednisone (30mg daily for 1 month). On physical examination, temperature was 37.6°C; there was a harsh ejection murmur over the aortic area, and a 2/6 decrescendo diastolic murmur. Electrocardiogram showed a normal sinus rhythm and PR interval, a complete right bundle branch block, and a left anterior hemi-block alternating with episodes of third degree AV block with a 35bpm escape rhythm of left bundle branch morphology, highly suggestive of infra-Hissian AV block (Fig. 1). Transthoracic and transesophageal echocardiography revealed a subaortic interventricular echo-free cavity, communicating with the aorta at the base of the right coronary cusp (Fig. 2). The Doppler-derived subaortic peak pressure gradient was 61mm Hg (Fig. 3). There was mild aortic regurgitation. No vegetations were seen. The erythrocyte sedimentation rate was 86mm/h, the white cell count was 13 200/mm3, with 81% polymorphonuclear cells, and the C-reactive protein was 11.6mg/dl. The pathergy phenomenon was present. Repeated blood cultures were negative. Serologic tests for Legionella, mycoplasm, Brucella, Bartonella, Chlamydia, Q fever, and Borrelia were negative.
|
|
|
The membranous septum was repaired with a glutaraldehyde-treated pericardial patch. Due to involvement of the aortic annulus by the inflammatory process, the aortic valve was replaced (23mm mechanical St Jude Medical prosthesis). A permanent pacemaker was implanted. Pathological examination of the removed valve disclosed no signs of endocarditis, and tissue cultures remained negative. Perioperative antibiotic therapy (penicillin, oxacillin, and gentamycin) was stopped, and immunosuppression (methylprednisolone, azathioprine and cyclosporin) was resumed. The post-operative course was uneventful; no cardiac event occurred during a 6-year follow-up period.
|
Discussion |
|---|
|
TopAbstractCase reportDiscussionReferences |
|---|
Behçet's disease is a multisystemic inflammatory disease typically characterized by oro-genital ulceration, chronic eye inflammation, and skin lesions.1 Cardiovascular manifestations are increasingly recognized during the course of the disease, and may occur in up to 30% of the patients.3 An inflammatory obliterative endarteritis affecting the vasa vasorum causes destruction of the media and thereby predisposes the arterial wall to aneurysm formation that eventually ruptures. These vascular lesions are true "arterial aphthae". Aortic pseudoaneurysm, fistulas from the aorta to the right atrium, and aortic valve perforation have been reported.4–6 The present case represents the first description of a complication of Behçet's disease consisting of a pseudoaneurysm at the base of the right sinus of Valsalva, which dissected the interventricular septum thereby creating a left ventricular outflow tract obstruction and inducing an AV conduction defect. Complete AV block has been described rarely during the course of Behçet's disease, and, to our knowledge, there has been only one report of complete AV block associated with an aneurysm of the sinus of Valsalva that ruptured into the left ventricular cavity.7 Subaortic obstruction by an abscess or a pseudoaneurysm has been documented rarely in conditions other than Behçet's disease, including infective endocarditis and after aortic valve replacement.8–10 Interestingly, an aneurysm of the sinus of Valsalva with conduction disturbances has been reported following infective endocarditis in a patient with Behçet's disease.11 In the present case, however, repeated blood cultures, pathological examination, tissue cultures and the lack of relapse despite early discontinuation of antibiotic therapy allowed us to rule out infective endocarditis. This case adds Behçet's disease to the list of unusual causes of subaortic obstruction, and emphasizes that a new onset murmur associated with fever should raise the possibility of diagnoses other than high output and/or valve regurgitation associated with infective endocarditis.
|
References |
|---|
|
TopAbstractCase reportDiscussionReferences |
|---|
- Sakane T., Takeno M., Suzuki N., Inaba G. Behçet's disease. N Engl J Med (1999) 341:1284–1291.
[Free Full Text] - Kaklamani V.G., Vaiopoulos G., Kaklamanis P.G. Behçet's disease. Semin Arthritis Rheum (1998) 27:197–217.[CrossRef][Web of Science][Medline]
- James D.G., Thomson A. Recognition of the diverse cardiovascular manifestation in Behcet's disease. Am Heart J (1982) 45:457–458.
- Rae S.A., Vandenburg M., Scholtz C.L. Aortic regurgitation and false aortic aneurysm formation in Behçet's disease. Postgrad Med J (1980) 56:438–439.
[Abstract/Free Full Text] - Melua A., Campbell N., McCluskey D., MacGowan S.W. Aorto-atrial fistula without aneurysm formation in Behcet's disease. Heart (1998) 80:200–201.
[Abstract/Free Full Text] - Tsui K.L., Lee K.W., Chan W.K., Chan H.K., Hon S.F., Leung T.C., et al. Behçet's aortitis and aortic regurgitation: a report of two cases. J Am Soc Echocardiogr (2004) 17:83–86.[CrossRef][Web of Science][Medline]
- Nojiri C., Endo M., Koyanagi H. Conduction disturbance in Behçet's disease. Association with ruptured aneurysm of the sinus of Valsalva into the left ventricular cavity. Chest (1984) 86:636–638.[CrossRef][Web of Science][Medline]
- Aoyagi S., Fukunaga S., Otsuka H., Akaiwa K., Yokokura Y., Yokokura H. Left ventricular outflow tract pseudoaneurysm after aortic valve replacement. A case report. J Heart Valve Dis (2004) 13:145–148.[Web of Science][Medline]
- Basmadjian A.J., Ducharme A., Ugolini P., Petitclerc R., Leung T.K., Tardif J.C. Obstruction of left ventricular outflow tract by vegetation and periaortic abscess. J Am Soc Echocardiogr (2000) 13:869–872.[CrossRef][Web of Science][Medline]
- Sanyal R.S., Roychoudhury D., Nanda N.C., Kim K.S., Osman K. Transthoracic and transesophageal echocardiographic diagnosis of severe subaortic obstruction produced by an abscess cavity. Am Heart J (1994) 128:1252–1255.[CrossRef][Web of Science][Medline]
- Okada K., Eishi K., Kitoh Y., Isobe F., Nakano K., Sasako Y. Huge aneurysm of the sinus of Valsalva following infective endocarditis in Behçet's disease. J Heart Valve Dis (1997) 6:179–180.[Web of Science][Medline]
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  C. Evereklioglu The migration pattern, patient selection with diagnostic methodological flaw and confusing naming dilemma in Behcet disease Eur J Echocardiogr, June 1, 2007; 8(3): 167 - 173. [Full Text] [PDF]
|
|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||