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Cardiac manifestations of Sneddon's syndrome
International Journal of Cardiology 190 (2015) 275–276
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the editor
Cardiac manifestations of Sneddon's syndrome Fernando Dominguez a, Burkert Pieske b, Sebastian Kelle b,⁎ a b
Hospital Universitario Puerta de Hierro, Department of Cardiology, Madrid, Spain German Heart Institute Berlin, Department of Internal Medicine/Cardiology, Berlin, Germany
a r t i c l e
i n f o
Article history: Received 17 April 2015 Accepted 18 April 2015 Available online 22 April 2015 Keywords: Sneddon's syndrome Cardiac magnetic resonance Echocardiography Cardiac manifestations of systemic disease
Sneddon's syndrome (SS) is rare, non-inflammatory arteriopathy characterized by cerebrovascular disease and livedo reticularis (LR). It generally affects women between 20 and 42 years of age [1], so this entity is increasingly recognized as a cause of stroke in young adults [2]. The pathophysiology of SS is not fully understood, but the presence of antiphospholipid antibodies in 50% of patients [3], as well as the skin and brain biopsy findings, suggests a thrombotic vasculopathy of medium and small arteries. Other organs can be affected by this arteriopathy, including the heart. However, cardiac manifestations of SS are not only restricted to ischemic coronary disease. We present the case of a 32-year old woman who was admitted for atypical chest pain and anterior ST-segment depression in the ECG. An
Fig. 1. A: Livedo reticularis affecting the patient's right hand. B: Transthoracic echocardiogram. Parasternal long axis view with evidence of thickened mitral leaflets (white arrow). C: Apical 4-chamber view with color Doppler showing central jet of mitral regurgitation (effective regurgitant orifice area: 0.25 cm2, PISA radius 0.7 cm).
⁎ Corresponding author at: German Heart Institute Berlin, Department of Internal Medicine/Cardiology, Augustenburger Platz 1, 13353 Berlin, Germany. E-mail address: [email protected] (S. Kelle).
http://dx.doi.org/10.1016/j.ijcard.2015.04.165 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
276
F. Dominguez et al. / International Journal of Cardiology 190 (2015) 275–276
Fig. 2. CMR images. A: Mid short axis view, late gadolinium enhancement (LGE): subendocardial scar with a 25% transmurality in the inferolateral segment (white arrow). B: Basal short axis view, LGE pattern consistent with a localized intramural fibrosis in the anterolateral segment (white arrow).
elevated troponin T confirmed the diagnosis of NSTEMI, but the coronary angiography did not evidence significant stenosis of the epicardial arteries. The patient's past history included an ischemic stroke which was suspected after an acute episode of speech difficulty. A brain MRI confirmed the diagnosis, as it evidenced a small infarct involving the rostral region of the left lentiform nucleus. Furthermore, the patient had coexisting violaceous skin lesions with an irregular net-like pattern confined to the hands and legs, and the Dermatology department confirmed the presence of livedo reticularis (Fig. 1A). A definitive diagnosis of SS was established, and the patient initiated oral anticoagulation therapy due to positive anti phospholipid antibodies and 3 previous spontaneous abortions. The cardiac study was completed by a transthoracic echocardiogram, which showed preserved left ventricular ejection fraction and a significant thickening of the mitral valve leaflets (Fig. 1B), with a secondary moderate mitral regurgitation (Fig. 1C). Despite the normal coronary angiography, a cardiac magnetic resonance (CMR) was conducted in order to rule out myocardial damage. Surprisingly, it evidenced delayed myocardial enhancement consistent with subendocardial scar, with a 25% transmurality in the mid inferolateral segment (Fig. 2A) and a localized intramural fibrosis in the basal anterolateral segment (Fig. 2B). Mitral valve thickening and coronary vasculopathy are the main cardiac manifestations of SS that can be found in literature [4,5]. Therefore, combining imaging techniques is crucial to confirm diagnosis. In our case, CMR was especially useful because it could detect scars potentially related to microvascular coronary disease despite a normal coronary angiography. Although typical CMR findings for cardiac vasculopathy in SS have been previously described [6], this is to the best of our knowledge the first report of a SS with coexisting coronary and heart valve abnormalities.
Multi-organ involvement is a hallmark of autoimmune systemic diseases, and the heart is an important target. As cardiac abnormalities in this context might increase morbidity and mortality [7,8], close follow-up including clinical evaluation and non-invasive imaging techniques could help to individualize patients' therapies. Conflict of interest The authors report no relationships that could be construed as a conflict of interest. References [1] I.B. Sneddon, Cerebro-vascular lesions and livedo reticularis, Br. J. Dermatol. 77 (1965) 180–185. [2] S.M. Boesch, A.L. Plorer, A.J. Auer, W. Poewe, F.T. Aichner, S.R. Felber, N.T. Sepp, The natural course of Sneddon syndrome: clinical and magnetic resonance imaging findings in a prospective six year observation study, J. Neurol. Neurosurg. Psychiatry 74 (4) (2003) 542–544. [3] C. Frances, T. Papo, B. Wechsler, J.L. Laporte, V. Biousse, J.C. Piette, Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients, Medicine 78 (4) (1999) 209–219. [4] L.A. Kalashnikova, E.L. Nasonov, V.V. Borisenko, V.B. Usman, L.Z. Prudnikova, V.U. Kovaljov, A.F. Kushekbaeva, Sneddon's syndrome: cardiac pathology and antiphospholipid antibodies, Clin. Exp. Rheumatol. 9 (4) (1991) 357–361. [5] S. Scheuermann, C. Schlundt, STEMI of the anterior wall associated with Sneddon's syndrome, Herz 39 (3) (2014) 352–353. [6] K. Nassenstein, F. Breuckmann, J. Dissemond, J. Barkhausen, Sneddon's syndrome: cardiac involvement detected by magnetic resonance imaging, Heart 92 (11) (2006) 1660. [7] A. Tincani, C.B. Rebaioli, M. Taglietti, Y. Shoenfeld, Heart involvement in systemic lupus erythematosus, anti-phospholipid syndrome and neonatal lupus, Rheumatology 45 (Suppl. 4) (2006) iv8–iv13. [8] G. Denas, S.P. Jose, A. Bracco, G. Zoppellaro, V. Pengo, Antiphospholipid syndrome and the heart: a case series and literature review, Autoimmun. Rev. 14 (3) (2015) 214–222.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the editor
Cardiac manifestations of Sneddon's syndrome Fernando Dominguez a, Burkert Pieske b, Sebastian Kelle b,⁎ a b
Hospital Universitario Puerta de Hierro, Department of Cardiology, Madrid, Spain German Heart Institute Berlin, Department of Internal Medicine/Cardiology, Berlin, Germany
a r t i c l e
i n f o
Article history: Received 17 April 2015 Accepted 18 April 2015 Available online 22 April 2015 Keywords: Sneddon's syndrome Cardiac magnetic resonance Echocardiography Cardiac manifestations of systemic disease
Sneddon's syndrome (SS) is rare, non-inflammatory arteriopathy characterized by cerebrovascular disease and livedo reticularis (LR). It generally affects women between 20 and 42 years of age [1], so this entity is increasingly recognized as a cause of stroke in young adults [2]. The pathophysiology of SS is not fully understood, but the presence of antiphospholipid antibodies in 50% of patients [3], as well as the skin and brain biopsy findings, suggests a thrombotic vasculopathy of medium and small arteries. Other organs can be affected by this arteriopathy, including the heart. However, cardiac manifestations of SS are not only restricted to ischemic coronary disease. We present the case of a 32-year old woman who was admitted for atypical chest pain and anterior ST-segment depression in the ECG. An
Fig. 1. A: Livedo reticularis affecting the patient's right hand. B: Transthoracic echocardiogram. Parasternal long axis view with evidence of thickened mitral leaflets (white arrow). C: Apical 4-chamber view with color Doppler showing central jet of mitral regurgitation (effective regurgitant orifice area: 0.25 cm2, PISA radius 0.7 cm).
⁎ Corresponding author at: German Heart Institute Berlin, Department of Internal Medicine/Cardiology, Augustenburger Platz 1, 13353 Berlin, Germany. E-mail address: [email protected] (S. Kelle).
http://dx.doi.org/10.1016/j.ijcard.2015.04.165 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
276
F. Dominguez et al. / International Journal of Cardiology 190 (2015) 275–276
Fig. 2. CMR images. A: Mid short axis view, late gadolinium enhancement (LGE): subendocardial scar with a 25% transmurality in the inferolateral segment (white arrow). B: Basal short axis view, LGE pattern consistent with a localized intramural fibrosis in the anterolateral segment (white arrow).
elevated troponin T confirmed the diagnosis of NSTEMI, but the coronary angiography did not evidence significant stenosis of the epicardial arteries. The patient's past history included an ischemic stroke which was suspected after an acute episode of speech difficulty. A brain MRI confirmed the diagnosis, as it evidenced a small infarct involving the rostral region of the left lentiform nucleus. Furthermore, the patient had coexisting violaceous skin lesions with an irregular net-like pattern confined to the hands and legs, and the Dermatology department confirmed the presence of livedo reticularis (Fig. 1A). A definitive diagnosis of SS was established, and the patient initiated oral anticoagulation therapy due to positive anti phospholipid antibodies and 3 previous spontaneous abortions. The cardiac study was completed by a transthoracic echocardiogram, which showed preserved left ventricular ejection fraction and a significant thickening of the mitral valve leaflets (Fig. 1B), with a secondary moderate mitral regurgitation (Fig. 1C). Despite the normal coronary angiography, a cardiac magnetic resonance (CMR) was conducted in order to rule out myocardial damage. Surprisingly, it evidenced delayed myocardial enhancement consistent with subendocardial scar, with a 25% transmurality in the mid inferolateral segment (Fig. 2A) and a localized intramural fibrosis in the basal anterolateral segment (Fig. 2B). Mitral valve thickening and coronary vasculopathy are the main cardiac manifestations of SS that can be found in literature [4,5]. Therefore, combining imaging techniques is crucial to confirm diagnosis. In our case, CMR was especially useful because it could detect scars potentially related to microvascular coronary disease despite a normal coronary angiography. Although typical CMR findings for cardiac vasculopathy in SS have been previously described [6], this is to the best of our knowledge the first report of a SS with coexisting coronary and heart valve abnormalities.
Multi-organ involvement is a hallmark of autoimmune systemic diseases, and the heart is an important target. As cardiac abnormalities in this context might increase morbidity and mortality [7,8], close follow-up including clinical evaluation and non-invasive imaging techniques could help to individualize patients' therapies. Conflict of interest The authors report no relationships that could be construed as a conflict of interest. References [1] I.B. Sneddon, Cerebro-vascular lesions and livedo reticularis, Br. J. Dermatol. 77 (1965) 180–185. [2] S.M. Boesch, A.L. Plorer, A.J. Auer, W. Poewe, F.T. Aichner, S.R. Felber, N.T. Sepp, The natural course of Sneddon syndrome: clinical and magnetic resonance imaging findings in a prospective six year observation study, J. Neurol. Neurosurg. Psychiatry 74 (4) (2003) 542–544. [3] C. Frances, T. Papo, B. Wechsler, J.L. Laporte, V. Biousse, J.C. Piette, Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients, Medicine 78 (4) (1999) 209–219. [4] L.A. Kalashnikova, E.L. Nasonov, V.V. Borisenko, V.B. Usman, L.Z. Prudnikova, V.U. Kovaljov, A.F. Kushekbaeva, Sneddon's syndrome: cardiac pathology and antiphospholipid antibodies, Clin. Exp. Rheumatol. 9 (4) (1991) 357–361. [5] S. Scheuermann, C. Schlundt, STEMI of the anterior wall associated with Sneddon's syndrome, Herz 39 (3) (2014) 352–353. [6] K. Nassenstein, F. Breuckmann, J. Dissemond, J. Barkhausen, Sneddon's syndrome: cardiac involvement detected by magnetic resonance imaging, Heart 92 (11) (2006) 1660. [7] A. Tincani, C.B. Rebaioli, M. Taglietti, Y. Shoenfeld, Heart involvement in systemic lupus erythematosus, anti-phospholipid syndrome and neonatal lupus, Rheumatology 45 (Suppl. 4) (2006) iv8–iv13. [8] G. Denas, S.P. Jose, A. Bracco, G. Zoppellaro, V. Pengo, Antiphospholipid syndrome and the heart: a case series and literature review, Autoimmun. Rev. 14 (3) (2015) 214–222.