Spontaneous right atrial thrombus in a patient with Crohn's disease: an unusual right atrial mass
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《血管的通路杂志》
Morriston Cardiac Centre, Morriston Hospital, Swansea, SA6 6NL, UK
Abstract
Crohn's disease is associated with systemic manifestations including increased risk of thromboembolism. We report a young female patient with active Crohn's disease who presented with spontaneous right atrial thrombus.
Key Words: Right atrial thrombus; Crohn's disease
1. Introduction
Crohn's disease (CD) is associated with systemic manifestations including increased risk of thromboembolism. Although systemic vascular complications are not uncommon, cardiac involvement is rare. We report a young female patient with CD who presented with spontaneous right atrial thrombus.
2. Case report
A 45-year-old female patient with 8 years history of CD was admitted with abdominal pain. A right atrial mass was incidentally discovered during investigation. Targeted questioning revealed gradually increasing exertional dyspnoea during the previous months. She denied chest pain, palpitation or orthopnoea. Apart from partial small bowel resection for Crohn's disease 7 years previously her medical history included asthma and systemic hypertension. There were no conventional risk factors for deep vein thrombosis.
Physical examination was unremarkable except for mild obesity. The chest X-ray was normal. Computed tomography showed a 2.5-cm mass in the right atrium near the inferior vena cava (IVC) orifice. Lungs, liver, kidneys, renal veins and IVC were free from masses. Transesophageal echocardiogram (TEE) confirmed the findings. There was no evidence of deep vein thrombosis on Duplex scanning and V/Q scan was negative. She received a therapeutic dose of enoxaparin sodium to cover for the possibility of the mass being thrombotic. A repeat TEE (Fig. 1) in the tertiary centre confirmed a moderately mobile, homogenous, pyramidal-shaped mass (4x2.5 cm) with irregular surface, attached by a broad stalk to the right atrial wall close to the opening of the IVC, lateral to the ostium of the coronary sinus. It did not interfere with blood flow or with tricuspid valve function. The mass had not changed since the initial TEE, in spite of a month of anticoagulation. The heart was otherwise structurally normal.
Baseline laboratory studies had shown a normal full blood count and fibrinogen level, but a slightly shortened activated partial thromboplastin time at 21.3 s (reference range 23–30 s) and the INR was 1.0.
The patient had the mass removed under cardiopulmonary bypass and deep hypothermic circulatory arrest, with a preoperative diagnosis of right atrial myxoma. At operation the mass was found to be attached to the posterior wall of the right atrium just above the IVC opening. No other mass was found inside the right atrium. IVC was patent and free. The mass was very friable but its base ‘peeled off’ easily from the atrial wall, leaving a smooth, glistening endocardial surface. Histological examination of the mass revealed organised fibrin clot with no evidence of myxoma (Fig. 2). The patient was discharged home with long-term warfarin following an uneventful recovery.
3. Discussion
The commonest causes of right atrial thrombus are prolonged central venous catheter and transvenous pacing leads. There are few reports of spontaneous right atrial thrombus in patients with multisystem inflammatory disease such as Bechet's syndrome and systemic lupus erythomatosis [1,2]. To our knowledge this is the first report of spontaneous atrial thrombosis in patients with CD to appear in the English literature. Venous and arterial thrombosis is an uncommon but well-recognised complication of CD. Incidence of systemic thromboembolic events is higher in patients with CD than normal population. The most extensive study by Talbot et al. found thromboembolic complications developed in 92 (1.3%) of 7199 patients with inflammatory bowel diseases, and this was associated with very high mortality [3]. Deep venous thrombosis with or without pulmonary embolism is the commonest presentation, but patients may also present with stroke, myocardial ischaemia or arterial thrombosis. In CD various coagulopathies have been described as possible causes for increased incidence of thrombosis. CD is associated with qualitative and quantitative platelet disorders, increased fibrinogen, fibrinopeptide A, factor V, factor VIII and anticardiolipin antibodies along with deficiency of antithrombin III and protein S [4–6]. Thus, patients with CD are exposed to a procoagulant and potentially antifibrinolytic environment. In CD there is no uniform pattern of coagulation changes, suggesting that multiple mechanisms may interact in producing thromboembolic complications. Higher concentration of prothrombotic factors are regarded as an increased risk for the development of thrombotic complications in CD. Due to lack of uniform changes in the coagulation system and apparent lack of relationship between the disease activity and incidence of thromboembolic episodes, risk assessment and prevention of such complication is difficult.
Primary thrombi develop within the right atrium and are usually immobile. Secondary thrombi result from venous embolization and are usually highly mobile. The serpigenous nature of the migrating thromboemboli is characteristic and, in contrast, a pedunculated mass attached to the interatrial septum suggests the diagnosis of myxoma. A primary thrombus attached to the atrial wall especially in an unusual location poses a difficult diagnostic challenge and TEE can be very useful [7], but the echocardiographic diagnosis of an intracavitary mass remains challenging because of the similar echo densities of thrombi and myxoma [8]. In some reports, the diagnosis could only be made retrospectively once the intracavitary mass started to reduce in size or disappeared with anticoagulation treatment [1,8,9].
Management largely depends on the effect of the thrombus on haemodynamic function of the heart, cause of thrombosis and the patient's general condition. Thrombolysis with streptokinase or t-PA supplanted by intravenous heparin therapy has been used successfully achieving a complete resolution of the thrombus [1,9]. Surgical removal of the thrombus is considered in physically fit patients with deterioration of cardiac function, when the diagnosis of atrial myxoma cannot be ruled out, or when thrombolysis is contraindicated. In this type of patient lack of any effect of anticoagulation therapy on the size and shape of the mass favoured the diagnosis of atrial myxoma and surgical intervention was undertaken. Close proximity of the mass to the IVC precluded double caval cannulation, hence short duration of circulatory arrest was used to resect the mass and exclude any pathology in the IVC.
4. Conclusion
Right atrial thrombus should strongly be considered in the differential diagnosis of intracardiac masses in patients with procoagulant conditions such as Crohn's disease. The clinical and echocardiographic features of this pathology may be atypical in this context.
References
Cemri M, Erkan A, Ozdemir M, Cengel A. Behcet's disease with a large free right atrial thrombus. Eur J Echocardiogr 2002; 3:233–235.
Vacca A, Garau P, Meloni L, Montisci R, Mameli A, Passiu G, Mathieu A. Right atrial thrombosis in systemic lupus erythomatosis. Letter to editor. Clin Exp Rheumatol 2001; 19:761.
Talbot RW, Heppel J, Dozois RR, Beart RW. Vascular complications of inflammatory bowel disease. Mayo Clin Proc 1986; 61:140–145.
Vecchi M, Cattaneo M, de Francis R, Mannucci PM. Risk of thromboembolic complications in patients with inflammatory bowel disease. Study of hemostasis measurements. Int J Clin Lab Res 1991; 21:165–170.
Hudson M, Chitoli A, Hutton RA, Smith MSH, Pounder RE, Wakefield AJ. Thrombotic vascular risk factors in inflammatory bowel disease. Gut 1996; 38:733–737.
Novacek G, Vogelsang H, Genser D, Moser G, Gagl A, Ehringer H, Koppensteiner R. Changes in blood rheology caused by Crohn's disease. Eur J Gastroenterol Hepatol 1996; 8:1089–1093.
Schwartzbard AZ, Tunick PA, Rosenzweig BP, Kronzon I. The role of transesophageal echocardiography in the diagnosis and treatment of right atrial thrombi. J Am Soc Echocardiogr 1999; 12:64–69.
Sasvary F, Murin J, Duris I, Pontuch P, Sedlak T, Labas P. Intracavitary thrombosis – an unusual complication in ulcerative colitis. Bratisl Lek Listy 1996; 97:669–672.
Rose PS, Punjabi NM, Pearse DB. Treatment of right heart thromboemboli. Chest 2002; 121:806–814.(Mesbah Rahman, Joyce Thek)
Abstract
Crohn's disease is associated with systemic manifestations including increased risk of thromboembolism. We report a young female patient with active Crohn's disease who presented with spontaneous right atrial thrombus.
Key Words: Right atrial thrombus; Crohn's disease
1. Introduction
Crohn's disease (CD) is associated with systemic manifestations including increased risk of thromboembolism. Although systemic vascular complications are not uncommon, cardiac involvement is rare. We report a young female patient with CD who presented with spontaneous right atrial thrombus.
2. Case report
A 45-year-old female patient with 8 years history of CD was admitted with abdominal pain. A right atrial mass was incidentally discovered during investigation. Targeted questioning revealed gradually increasing exertional dyspnoea during the previous months. She denied chest pain, palpitation or orthopnoea. Apart from partial small bowel resection for Crohn's disease 7 years previously her medical history included asthma and systemic hypertension. There were no conventional risk factors for deep vein thrombosis.
Physical examination was unremarkable except for mild obesity. The chest X-ray was normal. Computed tomography showed a 2.5-cm mass in the right atrium near the inferior vena cava (IVC) orifice. Lungs, liver, kidneys, renal veins and IVC were free from masses. Transesophageal echocardiogram (TEE) confirmed the findings. There was no evidence of deep vein thrombosis on Duplex scanning and V/Q scan was negative. She received a therapeutic dose of enoxaparin sodium to cover for the possibility of the mass being thrombotic. A repeat TEE (Fig. 1) in the tertiary centre confirmed a moderately mobile, homogenous, pyramidal-shaped mass (4x2.5 cm) with irregular surface, attached by a broad stalk to the right atrial wall close to the opening of the IVC, lateral to the ostium of the coronary sinus. It did not interfere with blood flow or with tricuspid valve function. The mass had not changed since the initial TEE, in spite of a month of anticoagulation. The heart was otherwise structurally normal.
Baseline laboratory studies had shown a normal full blood count and fibrinogen level, but a slightly shortened activated partial thromboplastin time at 21.3 s (reference range 23–30 s) and the INR was 1.0.
The patient had the mass removed under cardiopulmonary bypass and deep hypothermic circulatory arrest, with a preoperative diagnosis of right atrial myxoma. At operation the mass was found to be attached to the posterior wall of the right atrium just above the IVC opening. No other mass was found inside the right atrium. IVC was patent and free. The mass was very friable but its base ‘peeled off’ easily from the atrial wall, leaving a smooth, glistening endocardial surface. Histological examination of the mass revealed organised fibrin clot with no evidence of myxoma (Fig. 2). The patient was discharged home with long-term warfarin following an uneventful recovery.
3. Discussion
The commonest causes of right atrial thrombus are prolonged central venous catheter and transvenous pacing leads. There are few reports of spontaneous right atrial thrombus in patients with multisystem inflammatory disease such as Bechet's syndrome and systemic lupus erythomatosis [1,2]. To our knowledge this is the first report of spontaneous atrial thrombosis in patients with CD to appear in the English literature. Venous and arterial thrombosis is an uncommon but well-recognised complication of CD. Incidence of systemic thromboembolic events is higher in patients with CD than normal population. The most extensive study by Talbot et al. found thromboembolic complications developed in 92 (1.3%) of 7199 patients with inflammatory bowel diseases, and this was associated with very high mortality [3]. Deep venous thrombosis with or without pulmonary embolism is the commonest presentation, but patients may also present with stroke, myocardial ischaemia or arterial thrombosis. In CD various coagulopathies have been described as possible causes for increased incidence of thrombosis. CD is associated with qualitative and quantitative platelet disorders, increased fibrinogen, fibrinopeptide A, factor V, factor VIII and anticardiolipin antibodies along with deficiency of antithrombin III and protein S [4–6]. Thus, patients with CD are exposed to a procoagulant and potentially antifibrinolytic environment. In CD there is no uniform pattern of coagulation changes, suggesting that multiple mechanisms may interact in producing thromboembolic complications. Higher concentration of prothrombotic factors are regarded as an increased risk for the development of thrombotic complications in CD. Due to lack of uniform changes in the coagulation system and apparent lack of relationship between the disease activity and incidence of thromboembolic episodes, risk assessment and prevention of such complication is difficult.
Primary thrombi develop within the right atrium and are usually immobile. Secondary thrombi result from venous embolization and are usually highly mobile. The serpigenous nature of the migrating thromboemboli is characteristic and, in contrast, a pedunculated mass attached to the interatrial septum suggests the diagnosis of myxoma. A primary thrombus attached to the atrial wall especially in an unusual location poses a difficult diagnostic challenge and TEE can be very useful [7], but the echocardiographic diagnosis of an intracavitary mass remains challenging because of the similar echo densities of thrombi and myxoma [8]. In some reports, the diagnosis could only be made retrospectively once the intracavitary mass started to reduce in size or disappeared with anticoagulation treatment [1,8,9].
Management largely depends on the effect of the thrombus on haemodynamic function of the heart, cause of thrombosis and the patient's general condition. Thrombolysis with streptokinase or t-PA supplanted by intravenous heparin therapy has been used successfully achieving a complete resolution of the thrombus [1,9]. Surgical removal of the thrombus is considered in physically fit patients with deterioration of cardiac function, when the diagnosis of atrial myxoma cannot be ruled out, or when thrombolysis is contraindicated. In this type of patient lack of any effect of anticoagulation therapy on the size and shape of the mass favoured the diagnosis of atrial myxoma and surgical intervention was undertaken. Close proximity of the mass to the IVC precluded double caval cannulation, hence short duration of circulatory arrest was used to resect the mass and exclude any pathology in the IVC.
4. Conclusion
Right atrial thrombus should strongly be considered in the differential diagnosis of intracardiac masses in patients with procoagulant conditions such as Crohn's disease. The clinical and echocardiographic features of this pathology may be atypical in this context.
References
Cemri M, Erkan A, Ozdemir M, Cengel A. Behcet's disease with a large free right atrial thrombus. Eur J Echocardiogr 2002; 3:233–235.
Vacca A, Garau P, Meloni L, Montisci R, Mameli A, Passiu G, Mathieu A. Right atrial thrombosis in systemic lupus erythomatosis. Letter to editor. Clin Exp Rheumatol 2001; 19:761.
Talbot RW, Heppel J, Dozois RR, Beart RW. Vascular complications of inflammatory bowel disease. Mayo Clin Proc 1986; 61:140–145.
Vecchi M, Cattaneo M, de Francis R, Mannucci PM. Risk of thromboembolic complications in patients with inflammatory bowel disease. Study of hemostasis measurements. Int J Clin Lab Res 1991; 21:165–170.
Hudson M, Chitoli A, Hutton RA, Smith MSH, Pounder RE, Wakefield AJ. Thrombotic vascular risk factors in inflammatory bowel disease. Gut 1996; 38:733–737.
Novacek G, Vogelsang H, Genser D, Moser G, Gagl A, Ehringer H, Koppensteiner R. Changes in blood rheology caused by Crohn's disease. Eur J Gastroenterol Hepatol 1996; 8:1089–1093.
Schwartzbard AZ, Tunick PA, Rosenzweig BP, Kronzon I. The role of transesophageal echocardiography in the diagnosis and treatment of right atrial thrombi. J Am Soc Echocardiogr 1999; 12:64–69.
Sasvary F, Murin J, Duris I, Pontuch P, Sedlak T, Labas P. Intracavitary thrombosis – an unusual complication in ulcerative colitis. Bratisl Lek Listy 1996; 97:669–672.
Rose PS, Punjabi NM, Pearse DB. Treatment of right heart thromboemboli. Chest 2002; 121:806–814.(Mesbah Rahman, Joyce Thek)