European Journal of Echocardiography 2008 9(1):105-106; doi:10.1016/j.euje.2007.03.032
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2007. For permissions please email: journals.permissions@oxfordjournals.org.
Brain abscess caused by paradoxical embolization in Ebstein's anomaly
M.T. Lønnebakken1,2,*,
G. Greve1,3,
E. Leirgul1 and
E. Gerdts1,2
1 Department of Heart Disease, Haukeland University Hospital, 5021 Bergen, Norway
2 Institute of Medicine, University of Bergen, Norway
3 Institute of Clinical Medicine, University of Bergen, Norway
Received 29 November 2006; accepted after revision 24 March 2007; online publish-ahead-of-print 30 June 2007.
* Corresponding author. Tel: +47 55 97 22 20; fax: +47 55 97 51 50. E-mail address: mai.tone.lonnebakken{at}helse-bergen.no
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Abstract
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A 25-year-old woman presented with
Streptococcus milleri brain
abscess. Previous cardiac history was unremarkable. In search
for a source of embolism echocardiography was performed and
revealed a previous undiagnosed Ebstein's anomaly of moderate
severity with apical displacement of the septal leaflet of the
tricuspid valve and a secundum atrial septal defect (ASD) with
left to right shunt. The combination of increased right atrial
pressure caused by Ebstein's and an ASD with possibility of
transient right to left shunt predispose for paradoxical embolization.
The most likely reason for development of a brain abscess in
this patient is septic embolization from an infectious focus
outside the heart. Ebstein's anomaly can remain undiagnosed
until adulthood if the right ventricle, in spite of the smaller
size, is haemodynamically well functioning.
Keywords: Brain abscess; Ebstein's anomaly; Atrial septal defect; Paradoxical embolization; Congenital heart disease
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Medical history
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A 25-year-old woman was admitted after five days of persistent
headache and progressive left facial paralysis. She had a medical
history of malignant melanoma and migraine, including a migraine
attack accompanied by a transient right hemiparalysis some years
ago. Previous cardiac history was unremarkable.
Physical examination revealed a left central facial paralysis, reduced coordination of the left arm, and a pansystolic murmur 2/6 located at the lower left sternal edge. Electrocardiogram showed sinus rhythm, normal axis and right ventricular conduction delay. Blood tests were normal including oxygen saturation 98%, and negative blood cultures.
MRI of the brain demonstrated a brain abscess in the right thalamus region with compression of the third ventricle and development of hydrocephalus (Figure 1). Due to signs of increased intracranial pressure and progression of left sided hemiparalysis the abscess was drained by stereotactic craniotomy. Streptococcus milleri was identified in the abscess-liquid, and the patient was treated with antibiotics with slow regression of neurological symptoms.
Echocardiography revealed an Ebstein's anomaly of moderate severity
with apical displacement of the septal leaflet of the tricuspid
valve, moderate to severe tricuspid regurgitation and atrializing
of a large part of the right ventricle (displacement index of
the tricuspid valve was 24.1 mm/m
2). The ratio between the combined
area of the right atrium and atrialized part of the right ventricle,
and the functional right ventricle and left heart was 0.84 (
Figures 2 and 3). Transoesophageal echocardiography showed an ASD of secundum
type, with left to right shunt (
Figure 4). No valvular
vegetations were found.
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Figure 3 Transthoracic echocardiogram: the apical four-chamber view shows apical displacement of the septal leaflet of the tricuspid valve (TV). A part of the right ventricle (RV) is atrialized leaving a relatively small functional right ventricle.
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Discussion
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Ebstein's anomaly is a rare congenital cardiac malformation
accounting for <1% of all congenital cardiac disease.
1 The
severity and symptoms vary according to the degree of malformation
of the tricuspid valve and the size and function of the right
ventricle. Echocardiographic measurement of the ratio of the
combined area of the right atrium and atrialized right ventricle
to that of the functional right ventricle and left heart in
a four-chamber view at end-diastole has been used for classification
into four different grades with increasing severity.
1 Our patient
had a grade 2 Ebstein's anomaly. The right ventricle was smaller
than usual, but haemodynamically well functioning, explaining
why our patient remained asymptomatic and undiagnosed until
adulthood.
Ebstein's anomaly is often associated with other cardiac malformations, including ASD or patent foramen ovale in >80%, as also demonstrated in the present patient.1 In general brain abscess has a cardiac aetiology in about 5% of cases, valvular heart disease with endocarditis being the most common (2–4%) but congenital heart disease with possibility of right to left shunt and risk of paradoxical embolization is also a possible cause.2 The most likely cause for the brain abscess in our patient is paradoxical septic embolization through the ASD from an unknown infectious focus.1,2 Even though transoesophageal echocardiography revealed a left to right shunt through the ASD, the increased right atrial pressure caused by Ebstein's anomaly may predispose for transient shunt reversal and paradoxical embolization.
Theoretically, the previous migraine attack with transient right hemiparalysis experienced by our patient might have been another episode of paradoxical cerebral embolization.3 She was also using contraceptives at that time, which could predispose for thromboembolic events.
While device closure of ASD cannot be recommended in all patients with Ebstein's anomaly and ASD, a history of paradoxical embolization is an indication for closure.4 This was also planned for our patient but was delayed because of pregnancy.
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Supplementary material
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Supplementary data associated with this article can be found in the online version.
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References
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- Attenhofer Jost CH, Connolly HM, Edwards WD, Hayes D, Warnes CA, Danielson GK. Ebstein's anomaly – review of a multifaceted congenital cardiac condition. Swiss Med Wkly (2005) 135:269–81.[Medline]
- Li W, Sommerville J. Infective endocarditis in the grown-up congenital heart (GUCH) population. Eur Heart J (1998) 19:166–73.[Abstract/Free Full Text]
- Azarbal B, Tobis J, Sub W, Chan V, Dao C, Gaster R. Association of interatrial shunts and migraine headaches: impact of transcatheter closure. J Am Coll Cardiol (2005) 45:489–92.[Abstract/Free Full Text]
- Herrmann HC, Silvstry FE, Glaser R, See V, Kasner S, Brandbury D, et al. Percutaneous patent foramen ovale and atrial septal defect closure in adults: results and device comparison in 100 consecutive implants at a single center. Catheter Cardiovasc Interv (2005) 64:197–203.[CrossRef][Web of Science][Medline]
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