Right atrial thrombosis associated with sagittal sinus thrombosis caused by protein C-S deficiency
DOI:
https://doi.org/10.31729/jnma.370Abstract
Right atrial thrombi may develop within the right atrium or may have peripheral venous origins that, on their way to the final destination site lodge in, right atrium. Right atrial thrombosis is classified as two type: A (mobile, thin) and B (non mobile and attached to atrial wall) that they are probably of cardiac origin due to local pathology, indwelling catheter, atrial fibrillation, stasis, rheumatological or hematological disease like protein C or S deficiency. Protein C deficiency has been reported to be a risk factor for thrombosis in multiple organs as a result of inactivation of factor of Va. We describe a case of a huge right atrial thrombus with cerebral sagittal sinus thrombosis, in a protein C-S deficient patient, in which right atrial clot was successfully removed surgically and sagittal vein treated conservatively with full recovery of his central neurologic symptom.References
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14- Koeleman BP, Reitsma PH, Allaart CF, Bertina R. MActivated protein C resistance as an additional risk factor for thrombosis in protein C-deficient families. Blood. 1994;84:1035-1031.
15- Allaart CF, Poort SR, Rosendaal FR, Reitsma PH, Bertina R. M Increased risk of venous thrombosis in carriers of hereditary protein C deficiency defect. Lancet. 1993;341:138-134.
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24- Salunke P, Garg R, Futane S. Acute presentation of sagittal sinus thrombosis following closed head injury without overlying fracture. Acta Neurochir (Wien). 2013; [Epub ahead of print].
25- Katsanos AH, Katsanos KH, Kosmidou M, Giannopoulos S, Kyritsis AP, Tsianos EV. Cerebral sinus venous thrombosis in inflammatory bowel diseases. QJM. 2012; [Epub ahead of print].
26- Okunola PO, Ofovwe GE, Abiodun MT, Azunna CP. Superior sagittal sinus thrombosis complicating typhoid Fever in a teenager. Pediatr. 2012;doi:10.1155/2012/201203.
27- Samanta SK, Mahapatra N, Aich B, Sarkar N, Chatterjee A. An unusual case of transient cortical blindness with sagittal sinus thrombosis in a case of Henoch-Schonlein purpura. Nepal J Ophthalmol. 2012;4:333-5.
28- Graff-Radford J, Jones DT, Pruthi RK, Pruthi RK, Flemming KDA. neurological complication of a uterine fibroid. Neurocrit Care. 2013;18:95-93.
29- Singh G, Kaif M, Deep A, Nakaji P. High-voltage electrical burn of the skull causing thrombosis of the superior sagittal sinus. J Clin Neurosci. 2011;18:1552-4.
30- Huang PH, Su JJ, Lin PH. Iron deficiency anemia - a rare etiology of sinus thrombosis in adults. Acta Neurol Taiwan. 2010;19:125-30.
31- Bruhn HD. Pathophysiology and diagnosis of prethrombosis. Lab Med. 1993;17:164-158.
32- Halbmayer WM, Mannhaiter C, Feichtinger C. The prevalence of factor XII deficiency in 103 orally anticoagulated outpatients suffering from recurrent venous and/or arterial thromboembolism. Thromb Haemost. 1992;68:290-285.
33- Lodi S, Isa L, Pollini E, Bravo AF, Scalvini A. Defective intrinsic fibrinolytic activity in a patient with severe factor XII-deficiency and myocardial infarction. Scand J Haematol. 1984;33:82-80.
34- Ratnoff OD, Colopy JE. Familial hemorrhagic trait associated with deficiency of clot-promoting fraction of plasma. J Clin Invest. 1955;34:613-602.
35- Ferro JM, Canhao P, Stam J, Bousser MG. BarinagarrementerÃa F; for the ISCVT investigators. Prognosis of cerebral vein and dural sinus thrombosis. Stroke. 2004;35:670-664.
36- Rousseaux M, Lesoin F, Barbaste P, Jomin M. Infarctus cerebelleux pseudo-tumoral d’origine veineuse. Rev Neurol. 1988;144:211-209.
37- Eng LJ, Longstreth WT Jr, Shaw CM, Eskridge JM, Balhs FH. Cerebellar venous infarction. Neurology. 1990;40:838-837.
38- Ushiwata I, Saiki I, Murakami T, Kanaya H, Konno J, Wada S. Transverse sinus thrombosis accompanied by intracerebellar hemorrhage. No Shinkei Geka. 1989;17:55-51.
39- Nayak AK, Karnad D, Mahajan MV, Shah A, Meisheri YV. Cerebellar venous infarction in chronic suppurative otitis media. Stroke. 1994;25:1060-1058.
40- Krespi Y, Gurol ME, Coban O, Tuncay R, Bahar S. Venous infarction of brainstem and cerebellum. J Neuroimaging. 2001;11:431-425.
41- Nakase H, Shin Y, Nakagawa I, Kimura R, Sakaki T. Clinical features of postoperative cerebral venous infarction. Acta Neurochir. 2005;147:626-621.
42- Masuoka J, Wakamiya T, Mineta T, Takase Y, Kawashima M, Matsushima T. Thrombosis of the superior petrosal vein mimicking brain tumor. Neurol Med Chir. 2009;49:359-61.
2- McBane RD, Tafur A, Wysokinski WE. Acquired and congenital risk factors associated with cerebral venous sinus thrombosis. Thromb Res. 2010;126:87-81.
3- Marlar RA, Mastovich S. Hereditary protein C deficiency: a review of the genetics, clinical presentation, diagnosis and treatment. Blood Coagul Fibrinolysis. 1990;1:330-319.
4- Pescatore SL. Clinical management of protein C deficiency. Expert Opin Pharmacother. 2001;2:439- 431.
5- Price DT, Ridker PM. Factor V Leiden mutation and the risks for thromboembolic disease. Ann Intern Med. 1997;127:903-895.
6- Anderson JA, Weitz JI. Hypercoagulable states. Crit Care Clin. 2011;27:933-52.
7- Pai N, Ghosh K, Shetty S. Acquired and Heritable Thrombophilia in Indian Patients With Pediatric Deep Venous Thrombosis (DVT). Adv Exp Med Biol. 2013;735:155-72.
8- Risitano AM. Paroxysmal nocturnal hemoglobinuria and the complement system: recent insights and novel anticomplement strategies. Adv Exp Med Biol. 2013;735:155-72.
9- Qureshi I, Meshaka R, Donohue C, Ali A. Massive pulmonary embolism and thrombophilia. BMJ. 2013. doi:10.1136/bcr-2012-008197.
10- Van der Meer FJ, Koster Vandenbroucke JP, Briet E, Rosendaal FR. The Leiden Thrombophilia Study (LETS). Thromb Haemost. 1997;78:635-631.
11- Dahlback B, Carlsson M, Svensson PJ. Familial thrombophilia due to a previously unrecognized mechanism characterized by poor anticoagulant response to activated protein C: predication of a cofactor to activated protein C. Proc Natl Acad Sci. 1993;90:1008-1004.
12- Bertina RM, Koeleman BO, Koster T, Rosendaal FR, Dirven RJ, De Ronde H, et al. Mutation in blood coagulation factor V associated with resistance to activated protein C. Nature. 1994;369:67-64.
13- Seligsohn U, Zivelin A. Thrombophilia as a multigenic disorder. Thromb Haemost. 1997;78:301-297.
14- Koeleman BP, Reitsma PH, Allaart CF, Bertina R. MActivated protein C resistance as an additional risk factor for thrombosis in protein C-deficient families. Blood. 1994;84:1035-1031.
15- Allaart CF, Poort SR, Rosendaal FR, Reitsma PH, Bertina R. M Increased risk of venous thrombosis in carriers of hereditary protein C deficiency defect. Lancet. 1993;341:138-134.
16- Miletich J, Sherman L, Broze GJ. Absence of thrombosis in subjects with heterozygous protein C deficiency. N Engl J Med. 1987;317:996-991.
17- Svennson PJ, Dahlback B. Resistance to activated protein C as a basis for venous thrombosis. N Engl J Med. 1994;330:522-517.
18- Marcos LA, Majid R, Hamzeh N, Mehta N, Escobar M. An acute intracerebral vein thromboses in AIDS with protein C and S deficiency. Int J STD AIDS. 2008;19:61-59.
19- Nagumo K, Fukushima T, Takahashi H, Sakakibara Y, Kojima S, Akikusa B. Thyroid crisis and protein C deficiency in a case of superior sagittal sinus thrombosis. Brain Nerve. 2008;59:271-6.
20- Boulos P, Kouroukis C, Blake G. Superior sagittal sinus thrombosis occurring at high altitude associated with protein C deficiency. Acta Haematol. 1999;102:106-104.
21- Lefebvre P, Lierneux B, Lenaerts L, et al. Cerebral venous thrombosis and procoagulant factors. Angiology. 1998;49:563-71.
22- Confavreux C, Brunet P, Petiot P, Van Maldergem L, Marecaux G, Daune M, et al. Congenital protein C deficiency and superior sagittal sinus thrombosis causing isolated intracranial hypertension. J Neurol Neurosurg Psychiatry. 1994;57:657-655.
23- Weber J, Vida M, Greiner K. Sagittal sinus thrombosis with malignant brain oedema: pathophysiology of cortical veins after decompressive craniectomy. Acta Neurochir (Wien). 2013; [Epub ahead of print].
24- Salunke P, Garg R, Futane S. Acute presentation of sagittal sinus thrombosis following closed head injury without overlying fracture. Acta Neurochir (Wien). 2013; [Epub ahead of print].
25- Katsanos AH, Katsanos KH, Kosmidou M, Giannopoulos S, Kyritsis AP, Tsianos EV. Cerebral sinus venous thrombosis in inflammatory bowel diseases. QJM. 2012; [Epub ahead of print].
26- Okunola PO, Ofovwe GE, Abiodun MT, Azunna CP. Superior sagittal sinus thrombosis complicating typhoid Fever in a teenager. Pediatr. 2012;doi:10.1155/2012/201203.
27- Samanta SK, Mahapatra N, Aich B, Sarkar N, Chatterjee A. An unusual case of transient cortical blindness with sagittal sinus thrombosis in a case of Henoch-Schonlein purpura. Nepal J Ophthalmol. 2012;4:333-5.
28- Graff-Radford J, Jones DT, Pruthi RK, Pruthi RK, Flemming KDA. neurological complication of a uterine fibroid. Neurocrit Care. 2013;18:95-93.
29- Singh G, Kaif M, Deep A, Nakaji P. High-voltage electrical burn of the skull causing thrombosis of the superior sagittal sinus. J Clin Neurosci. 2011;18:1552-4.
30- Huang PH, Su JJ, Lin PH. Iron deficiency anemia - a rare etiology of sinus thrombosis in adults. Acta Neurol Taiwan. 2010;19:125-30.
31- Bruhn HD. Pathophysiology and diagnosis of prethrombosis. Lab Med. 1993;17:164-158.
32- Halbmayer WM, Mannhaiter C, Feichtinger C. The prevalence of factor XII deficiency in 103 orally anticoagulated outpatients suffering from recurrent venous and/or arterial thromboembolism. Thromb Haemost. 1992;68:290-285.
33- Lodi S, Isa L, Pollini E, Bravo AF, Scalvini A. Defective intrinsic fibrinolytic activity in a patient with severe factor XII-deficiency and myocardial infarction. Scand J Haematol. 1984;33:82-80.
34- Ratnoff OD, Colopy JE. Familial hemorrhagic trait associated with deficiency of clot-promoting fraction of plasma. J Clin Invest. 1955;34:613-602.
35- Ferro JM, Canhao P, Stam J, Bousser MG. BarinagarrementerÃa F; for the ISCVT investigators. Prognosis of cerebral vein and dural sinus thrombosis. Stroke. 2004;35:670-664.
36- Rousseaux M, Lesoin F, Barbaste P, Jomin M. Infarctus cerebelleux pseudo-tumoral d’origine veineuse. Rev Neurol. 1988;144:211-209.
37- Eng LJ, Longstreth WT Jr, Shaw CM, Eskridge JM, Balhs FH. Cerebellar venous infarction. Neurology. 1990;40:838-837.
38- Ushiwata I, Saiki I, Murakami T, Kanaya H, Konno J, Wada S. Transverse sinus thrombosis accompanied by intracerebellar hemorrhage. No Shinkei Geka. 1989;17:55-51.
39- Nayak AK, Karnad D, Mahajan MV, Shah A, Meisheri YV. Cerebellar venous infarction in chronic suppurative otitis media. Stroke. 1994;25:1060-1058.
40- Krespi Y, Gurol ME, Coban O, Tuncay R, Bahar S. Venous infarction of brainstem and cerebellum. J Neuroimaging. 2001;11:431-425.
41- Nakase H, Shin Y, Nakagawa I, Kimura R, Sakaki T. Clinical features of postoperative cerebral venous infarction. Acta Neurochir. 2005;147:626-621.
42- Masuoka J, Wakamiya T, Mineta T, Takase Y, Kawashima M, Matsushima T. Thrombosis of the superior petrosal vein mimicking brain tumor. Neurol Med Chir. 2009;49:359-61.
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2013-06-30
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Sabzi, F., & Faraji, R. (2013). Right atrial thrombosis associated with sagittal sinus thrombosis caused by protein C-S deficiency. Journal of Nepal Medical Association, 52(190). https://doi.org/10.31729/jnma.370
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