CE: ; JCM-D-13-00672; Total nos of Pages: 3; JCM-D-13-00672 Images in cardiovascular medicine Uncorrected Ebstein’s anomaly with atrial septal defect complicated by brain abscess in an adult patient Roberto Scarsinia, Maria A. Priolia, Elena G. Milanoa, Valentina Benettia, Micol Rebonatoa, Giovanni Puppinib, Franco Alessandrinic and Corrado Vassanellia We report the case of a 56-year-old male patient affected by a severe form of Ebstein’s anomaly (type C of Carpentier classification) with secundum atrial septal defect, who presented to the emergency department with impaired consciousness, seizures, and trismus. The brain computed tomography scan showed evidence of a mass located in the frontal lobe, confirmed by brain MRI consistent with brain abscess. Both echocardiography and cardiac MRI showed no evidence of valvular vegetation. This case shows how the combination of increased atrial pressure and bidirectional shunt through atrial septal defect may lead to paradoxical embolization. A 56-year-old patient presented to the emergency department unresponsive with eye deviation, seizures, and trismus. Physical examination showed lip cianosis and acrocyanosis, mild finger clubbing, normal heart rate, and a holosystolic murmur. The patient was apyretic. Laboratory analysis revealed increased hematocrits and mild leukocytosis. ECG showed right atrial dilatation and complete right bundle branch block. Recent medical history included an episode of gingivitis associated with J Cardiovasc Med 2014, 15:000–000 Keywords: atrial septal defect, brain abscess, congenital heart disease, Ebstein’s anomaly a Section of Cardiology, Department of Medicine, bSection of Radiology and Section of Neuroradiology, Department of Pathology and Diagnostics, University of Verona, Verona, Italy c Correspondence to Roberto Scarsini, MD, Section of Cardiology, Department of Medicine, University of Verona, Verona, Italy Tel: +39 0458122320; fax: +39 0458027307; e-mail: scarsini.roberto@gmail.com Received 19 December 2013 Revised 4 April 2014 Accepted 7 April 2014 fever and sore throat 2 weeks before; after that, the patient underwent professional teeth cleaning. He had been diagnosed with severe Ebstein’s anomaly (type C of Carpentier classification1) and ostium secundum atrial septal defect (ASD) in young age, but he always refused any surgical treatment. In the emergency department, the patient underwent brain computed tomography (CT) scan with evidence of a nodular lesion Fig. 1 Brain MRI showing brain abscess located in the right frontal lobe with perilesional enhancement consistent with edema and tissutal necrosis. (a) axial view; (b) sagital view. 1558-2027 ß 2014 Italian Federation of Cardiology DOI:10.2459/JCM.0000000000000127 Copyright © Italian Federation of Cardiology. Unauthorized reproduction of this article is prohibited. CE: ; JCM-D-13-00672; Total nos of Pages: 3; JCM-D-13-00672 2 Journal of Cardiovascular Medicine 2013, Vol 00 No 00 Fig. 2 Four-chamber view of transthoracic echocardiography showing the severe apical displacement of the septal leaflet of the tricuspid valve and the increased dimension of the right atrium and atrialized ventricle. in the right frontal lobe. Further investigation with brain MRI was performed showing a ring-enhancing lesion of 2.5 cm of diameter consistent with brain abscess (Fig. 1). Fig. 4 Color Doppler transthoracic echocardiography showing ostium secundum atrial septal defect with left-to-right shunt. Transthoracic echocardiography showed a dilated right atrium and an atrialized ventricle with severe displacement of the septal leaflet of the tricuspid valve (Fig. 2). Doppler analysis revealed severe tricuspidal regurgitation Fig. 3 Apical four-chamber view of transthoracic echocardiography with evidence of severe tricuspid regurgitation. Copyright © Italian Federation of Cardiology. Unauthorized reproduction of this article is prohibited. CE: ; JCM-D-13-00672; Total nos of Pages: 3; JCM-D-13-00672 Uncorrected Ebstein’s anomaly Scarsini et al. 3 Fig. 5 Cardiac MRI. Four-chamber view showing the atrialized part of the right ventricle and the apical displacement of the septal tricuspid valve leaflet (a); atrial short-axis cine with evidence of atrial septal defect (ASD) with bidirectional shunt (b). and a bidirectional shunt through the ASD (Figs. 3 and 4). Cardiac MRI was performed in order to evaluate better cardiac anatomy (Fig. 5). Both echocardiography and MRI excluded the presence of valvular vegetations. Ebstein’s anomaly is often associated with ASD or patent foramen ovale.4 Brain abscess due to paradoxical embolization in Ebstein’s anomaly has been described by Lønnebakken et al.5 The patient was treated with ceftriaxone and metronidazole. Blood cultures were persistently negative. The follow-up MRI performed after 1 month of antibiotic therapy showed partial regression of the brain lesion. Seriate brain MRI documented a complete regression of the brain lesion after 18 months. In our patient, the combination of elevated right atrial pressure, due to intracardiac anatomy and severe tricuspid regurgitation, with a right-to-left shunt through the ASD increased consistently the risk of paradoxical embolization, which may have been caused by an episode of transient bacteremia. In patients with cyanotic congenital heart diseases, the right to left shunt allows septic emboli or bacteria to reach the cerebral circulation, avoiding the physiological pulmonary filter.2 Moreover, increased blood viscosity may lead to low perfusion and microinfarction in some brain areas where microorganism can sustain growth and multiply to form abscess.3 Brain abscess treatment includes antibiotic therapy and neurosurgical treatment, which should be reserved for patient with unstable condition, with large or multiple brain lesions.3 References 1 2 3 4 5 Carpentier A, Chauvaud S, Macé L, et al. A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1988; 96:92–101. Sung C, Jung J, Lee S, et al. Brain abscess in an adult with atrial septal defect. Clin Cardiol 2010; 33:e51–e53. Mazumdar D, Jhawar S, Goel A. Brain abscess: an overview. Int J Surg 2011; 9:136–144. Frescura C, Angelini A, Daliento L, Thiene G. Morphological aspects of Ebstein’s anomaly in adults. Thorac Cardiovasc Surg 2000; 48:203–208. Lønnebakken MT, Greve G, Leirgul E, Gerdts E. Brain abscess caused by paradoxical embolization in Ebstein’s anomaly. Eur J Echocardiogr 2008; 9:105–106. Copyright © Italian Federation of Cardiology. Unauthorized reproduction of this article is prohibited.