Lupus (2014) 23, 721–723 http://lup.sagepub.com
LETTER TO THE EDITOR Intracardiac multiple thrombus formation as a rare manifestation of primary antiphospholipid antibody syndrome: a case report Sir, Antiphospholipid syndrome (APS) has been described by Graham R. Hughes as a combination of the clinical symptoms of arterial and venous thromboembolism, with the presence of autoantibodies.1,2 Other features of APS include recurrent spontaneous abortion, thrombocytopenia, chorea, migraine, cutaneous symptoms, and valvular heart disease. Antiphospholipid antibodies may be associated with some connective tissue diseases, such as systemic lupus erythematosus, as well as some infectious diseases. The disease may accompany HIV, hepatitis C, syphilis, and other infections, including cytomegalovirus.3,4 APS can sometimes be detected in healthy patients. In the absence of an underlying disorder, the syndrome is classified as primary.5,6 The following report details the case of a patient with intracardiac thrombus formation as a first manifestation of primary antiphospholipid antibody syndrome. A 25-year-old man was admitted to our cardiovascular surgery department with dyspnea of increasing severity, exhaustion, cognitive disturbance and vertigo. Three days before the hospital admission, he suffered shortness of breath and palpitation, and sought attention in the emergency room for further evaluation. Cardiac auscultation revealed a third heart sound (gallop rhythm) and grade III/VI systolic murmur on the second–third intercostal space over the left sternal border. Transthoracic and transoesophageal echocardiography revealed a mobile mass measuring 32 25 25 mm, adhered to both leaflets of the mitral valve (Figure 1(a) and (b)). The mass was closely attached to the free edge of the mitral leaflets. Myxoma and bacterial/nonbacterial vegetation were considered in the differential diagnosis of the mass. Following the decision of the our hospital’s council of cardiology and cardiovascular surgery, the patient went to the Correspondence to: Ihsan Sami Uyar, Sifa University Hospital, Bornova Health Application and Research Center, Cardiovascular Surgery Department Sanayii Cad. No: 7, 35100 Bornova, Izmir, Turkey. Email: [email protected] Received 29 December 2013; accepted 18 February 2014
operation room with a cardiopulmonary bypass to undergo surgical removal of the mass, due to progressive dyspnea and possible impending fatal embolism. During surgery, a 3 3 2 cm wellorganized, thrombus-like mass adherent to the left atrial surface of the both leaflets of the mitral valve became visible. The mass was friable and close to the leaflets’ free edge. The surgical team easily separated the mass from the leaflet, and completely removed it (Figure 2). The surgical specimen underwent histopathological examination. Intraoperative examination revealed that aortic, pulmonary, and tricuspid valve apparatus were not affected and normal. The histopathology reported blood fibrin, mesothelial cells, organized necrotic thrombus formation, or vegetation-like lesions. His other system questioning was normal. Anti-cardiolipin IgM was at 20.2 U/ ml (normal 12); anti-cardiolipin IgG at 120 U/ ml (normal 12); anti-b2-glycoprotein-1 at 200 U/ml (normal 20); lupus anticoagulant at 89.4 sn (normal 31–44). According to international classification criteria, the patient was diagnosed with primary APS because of the suspected intracardiac thrombosis, marked by prolonged activated partial thromboplastin time and elevated anti-cardiolipin antibody titers. His general condition was good following surgery. Postoperative recovery was uneventful, and he was discharged in good condition with anticoagulation treatment. In conclusion, although intracardiac mass caused by APS is uncommon, it should be kept in mind while searching for an accurate diagnosis. Early removal of the mass and maintenance anticoagulation therapy are essential for the prevention of undesired catastrophic events.7,8
Funding This research received no specific grant from any funding agency in the public, commercial, or notfor-profit sectors.
Conflict of interest statement The authors have no conflicts of interest to declare.
! The Author(s), 2014. Reprints and permissions: http://www.sagepub.co.uk/journalsPermissions.nav
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10.1177/0961203314527368
Letter to the Editor I Sami Uyar et al.
722
Figure 1(a) and (b) Transoesophageal echocardiography revealed a mobile mass in size of 32 25 25 mm, adhered to both leaflets of the mitral valve (yellow and red arrows).
Lupus Downloaded from lup.sagepub.com at UNIV OF MISSISSIPPI on June 17, 2015
Letter to the Editor I Sami Uyar et al.
723
Figure 2 A photograph of the operative specimen shows a mass with irregular surfaces mimicking vegetation.
3 Asherson RA, Cervera R. ‘Primary,’ ‘secondary’ and other variants of the antiphospholipid syndrome. Lupus 1994; 3: 293–298. 4 Triplett DA. Assays for detection of antiphospholipid antibodies. Lupus 1994; 3: 281–287. 5 Bertero MT, Bazzan M, Carignola R, et al. Antiphospholipid syndrome in northwest Italy (APS Piedmont Cohort): demographic features, risk factors, clinical and laboratory profile. Lupus 2012; 21: 806–809. 6 Ruiz-Irastorza G, Cuadrado MJ, Ruiz-Arruza I, et al. Evidencebased recommendations for the prevention and long-term management of thrombosis in antiphospholipid antibody-positive patients: Report of a task force at the 13th International Congress on antiphospholipid antibodies. Lupus 2011; 11: 206–218. 7 Alarco´n-Segovia D, Boffa MC, Branch W, et al. Prophylaxis of the Antiphospholipid Syndrome: A Consensus Report. Lupus 2003; 12: 499–503. 8 Tincani A, Branch W, Levy RA, et al. Treatment of pregnant patients with antiphospholipid syndrome. Lupus 2003; 12: 524–529.
I Sami Uyar1, B Uyar2, I Erdogan3, O Nermin Sivrikoz4, S Kobak5 and E Alp Alayunt1
References 1 Hughes GR. The antiphospholipid syndrome: Ten years on. Lancet 1993; 342: 341–344. 2 Ruiz-Irastorza G, Khamashta MA, Hughes GR. Antiaggregant and anticoagulant therapy in systemic lupus erythematosus and Hughes’ syndrome. Lupus 2001; 10: 241–245. Review.
1 Cardiovascular Surgery Department, Medical Faculty, Sifa University, Izmir, Turkey; 2Dermatology Department, Medical Faculty, Sifa University, Izmir, Turkey; 3Cardiology Department, Medical Faculty, Sifa University, Izmir, Turkey; 4Pathology Department, Medical Faculty, Sifa University, Izmir, Turkey; and 5 Rheumatology Department, Medical Faculty, Sifa University, Izmir, Turkey
Lupus Downloaded from lup.sagepub.com at UNIV OF MISSISSIPPI on June 17, 2015
LETTER TO THE EDITOR Intracardiac multiple thrombus formation as a rare manifestation of primary antiphospholipid antibody syndrome: a case report Sir, Antiphospholipid syndrome (APS) has been described by Graham R. Hughes as a combination of the clinical symptoms of arterial and venous thromboembolism, with the presence of autoantibodies.1,2 Other features of APS include recurrent spontaneous abortion, thrombocytopenia, chorea, migraine, cutaneous symptoms, and valvular heart disease. Antiphospholipid antibodies may be associated with some connective tissue diseases, such as systemic lupus erythematosus, as well as some infectious diseases. The disease may accompany HIV, hepatitis C, syphilis, and other infections, including cytomegalovirus.3,4 APS can sometimes be detected in healthy patients. In the absence of an underlying disorder, the syndrome is classified as primary.5,6 The following report details the case of a patient with intracardiac thrombus formation as a first manifestation of primary antiphospholipid antibody syndrome. A 25-year-old man was admitted to our cardiovascular surgery department with dyspnea of increasing severity, exhaustion, cognitive disturbance and vertigo. Three days before the hospital admission, he suffered shortness of breath and palpitation, and sought attention in the emergency room for further evaluation. Cardiac auscultation revealed a third heart sound (gallop rhythm) and grade III/VI systolic murmur on the second–third intercostal space over the left sternal border. Transthoracic and transoesophageal echocardiography revealed a mobile mass measuring 32 25 25 mm, adhered to both leaflets of the mitral valve (Figure 1(a) and (b)). The mass was closely attached to the free edge of the mitral leaflets. Myxoma and bacterial/nonbacterial vegetation were considered in the differential diagnosis of the mass. Following the decision of the our hospital’s council of cardiology and cardiovascular surgery, the patient went to the Correspondence to: Ihsan Sami Uyar, Sifa University Hospital, Bornova Health Application and Research Center, Cardiovascular Surgery Department Sanayii Cad. No: 7, 35100 Bornova, Izmir, Turkey. Email: [email protected] Received 29 December 2013; accepted 18 February 2014
operation room with a cardiopulmonary bypass to undergo surgical removal of the mass, due to progressive dyspnea and possible impending fatal embolism. During surgery, a 3 3 2 cm wellorganized, thrombus-like mass adherent to the left atrial surface of the both leaflets of the mitral valve became visible. The mass was friable and close to the leaflets’ free edge. The surgical team easily separated the mass from the leaflet, and completely removed it (Figure 2). The surgical specimen underwent histopathological examination. Intraoperative examination revealed that aortic, pulmonary, and tricuspid valve apparatus were not affected and normal. The histopathology reported blood fibrin, mesothelial cells, organized necrotic thrombus formation, or vegetation-like lesions. His other system questioning was normal. Anti-cardiolipin IgM was at 20.2 U/ ml (normal 12); anti-cardiolipin IgG at 120 U/ ml (normal 12); anti-b2-glycoprotein-1 at 200 U/ml (normal 20); lupus anticoagulant at 89.4 sn (normal 31–44). According to international classification criteria, the patient was diagnosed with primary APS because of the suspected intracardiac thrombosis, marked by prolonged activated partial thromboplastin time and elevated anti-cardiolipin antibody titers. His general condition was good following surgery. Postoperative recovery was uneventful, and he was discharged in good condition with anticoagulation treatment. In conclusion, although intracardiac mass caused by APS is uncommon, it should be kept in mind while searching for an accurate diagnosis. Early removal of the mass and maintenance anticoagulation therapy are essential for the prevention of undesired catastrophic events.7,8
Funding This research received no specific grant from any funding agency in the public, commercial, or notfor-profit sectors.
Conflict of interest statement The authors have no conflicts of interest to declare.
! The Author(s), 2014. Reprints and permissions: http://www.sagepub.co.uk/journalsPermissions.nav
Downloaded from lup.sagepub.com at UNIV OF MISSISSIPPI on June 17, 2015
10.1177/0961203314527368
Letter to the Editor I Sami Uyar et al.
722
Figure 1(a) and (b) Transoesophageal echocardiography revealed a mobile mass in size of 32 25 25 mm, adhered to both leaflets of the mitral valve (yellow and red arrows).
Lupus Downloaded from lup.sagepub.com at UNIV OF MISSISSIPPI on June 17, 2015
Letter to the Editor I Sami Uyar et al.
723
Figure 2 A photograph of the operative specimen shows a mass with irregular surfaces mimicking vegetation.
3 Asherson RA, Cervera R. ‘Primary,’ ‘secondary’ and other variants of the antiphospholipid syndrome. Lupus 1994; 3: 293–298. 4 Triplett DA. Assays for detection of antiphospholipid antibodies. Lupus 1994; 3: 281–287. 5 Bertero MT, Bazzan M, Carignola R, et al. Antiphospholipid syndrome in northwest Italy (APS Piedmont Cohort): demographic features, risk factors, clinical and laboratory profile. Lupus 2012; 21: 806–809. 6 Ruiz-Irastorza G, Cuadrado MJ, Ruiz-Arruza I, et al. Evidencebased recommendations for the prevention and long-term management of thrombosis in antiphospholipid antibody-positive patients: Report of a task force at the 13th International Congress on antiphospholipid antibodies. Lupus 2011; 11: 206–218. 7 Alarco´n-Segovia D, Boffa MC, Branch W, et al. Prophylaxis of the Antiphospholipid Syndrome: A Consensus Report. Lupus 2003; 12: 499–503. 8 Tincani A, Branch W, Levy RA, et al. Treatment of pregnant patients with antiphospholipid syndrome. Lupus 2003; 12: 524–529.
I Sami Uyar1, B Uyar2, I Erdogan3, O Nermin Sivrikoz4, S Kobak5 and E Alp Alayunt1
References 1 Hughes GR. The antiphospholipid syndrome: Ten years on. Lancet 1993; 342: 341–344. 2 Ruiz-Irastorza G, Khamashta MA, Hughes GR. Antiaggregant and anticoagulant therapy in systemic lupus erythematosus and Hughes’ syndrome. Lupus 2001; 10: 241–245. Review.
1 Cardiovascular Surgery Department, Medical Faculty, Sifa University, Izmir, Turkey; 2Dermatology Department, Medical Faculty, Sifa University, Izmir, Turkey; 3Cardiology Department, Medical Faculty, Sifa University, Izmir, Turkey; 4Pathology Department, Medical Faculty, Sifa University, Izmir, Turkey; and 5 Rheumatology Department, Medical Faculty, Sifa University, Izmir, Turkey
Lupus Downloaded from lup.sagepub.com at UNIV OF MISSISSIPPI on June 17, 2015
