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CASE REPORT
Year : 2014  |  Volume : 55  |  Issue : 4  |  Page : 354-355  

Cardiac left ventricular thrombus in protein C deficiency


Department of Cardiovascular Surgery, Imam Ali Heart Center, Kermanshah University of Medical Sciences, Kermanshah, Iran

Date of Web Publication21-Jul-2014

Correspondence Address:
Reza Faraji
Department of Cardiovascular Surgery, Imam Ali Heart Center, Kermanshah University of Medical Sciences, Kermanshah
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0300-1652.137229

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   Abstract 

We report an exceptional case of, 33-year-old woman presenting with, dyspnoea and chest pain, Cardio respiratory sign and symptom related to diastolic dysfunction caused by mass effect of thrombosis on diastolic filling of left ventricule (LV). The common aetiologies of these devastating complication results in thrombophillia diagnosis, and echocardioghraphy showed a large mass in left ventricular cavity. In laboratory exam, protein C-S deficiency was confirmed however, others related test of thrombophillia were negative. The patient underwent cardiopulmonary bypass with thrombosis extraction and her sign and symptom, recovered uneventfully. This case report illustrates an exceedingly rare case of thrombophilia-induced left ventricular clot formation.

Keywords: Chest pain, dysfunction, dyspnoea, left ventricule, protein C deficiency, thrombosis


How to cite this article:
Sabzi F, Faraji R. Cardiac left ventricular thrombus in protein C deficiency. Niger Med J 2014;55:354-5

How to cite this URL:
Sabzi F, Faraji R. Cardiac left ventricular thrombus in protein C deficiency. Niger Med J [serial online] 2014 [cited 2021 May 15];55:354-5. Available from: https://www.nigeriamedj.com/text.asp?2014/55/4/354/137229


   Introduction Top


Intra-cardiac thrombosis is a rare cardiac disorder characterised by the development of clot formation in the cardiac chamber with or without other identifiable predisposing factors. The combination of left ventricular (LV) thrombosis with normal systolic function and hypercoaguability can cause thromboembolic complications including systemic thrombi. [1] There are only one case reports of left ventricle thrombosis with transient deficiency of protein C in a hepatitis case in the literature to date, [2] our case illustrates the first case of LV thrombosis with thrombophilia and illustrates the use of extensive laboratory exam in young patient with left ventricular clot formation to roll out predisposing factors for prevention of recurrent intra-cardiac thrombosis formation.


   Case report Top


We present a case of a 33-year-old woman with normal cardiac ejection function who was admitted to our hospital in severe dyspnoea: her left ventricular ejection fraction was 60% The patient had no history of thromboembolic events, and perioperative transesophageal echocardiography (TEE) show large thrombus in LV [Figure 1] and [Figure 2]. On physical examination, she was tachycardic with a heart rate of 95bpm and a normal blood pressure of 110/70 mmHg. The heart sounds were within normal limits with normal breath sounds bilaterally. Initial blood work including the complete blood count, electrolytes, liver function tests and cardiac biomarkers were within normal limits. The electrocardiogram demonstrated sinus tachycardia and the chest X-ray confirmed normal cardiac sellout without pulmonary venous congestion. With a diagnosis of thrombi, the patient was appropriately started parenteral heparin for the LV thrombi. Recent myocardial infarction was ruled out by electrocardiography and cardiac enzyme measurements. Left ventricular function was normal and no intra-cardiac septal defects were seen. The aortic and mitral valves were normal, as were left atrial dimensions and contours. A coronary angiogram showed no obstructions. Complete blood count and blood biochemistry were within normal limits, except for prolonged erythrocyte sedimentation and a reduced protein C and S level. The protein C level was 30% (normal: >70) and protein S level was 50% (normal:65%). There are no abnormal values of antithrombin III, lupus anticoagulant, and anticardiolipin antibodies, HLA-B5, HLA-B27, anti-nuclear antibody and rheumatoid factor were negative. Ophthalmological examination showed no significant finding and hepatic function tests were normal. On echocardioghraphic exam in four chamber view, a rounded mass was seen attached to the anterior wall of the left ventricle [Figure 1] and [Figure 2]. Its shape and characteristics, on echocardiography, were consistent with a mural thrombus. Under standard cardiopulmonary bypass, left atrial was opened between the interatrial grooves. The ventricular muscle was normal in appearance. In the left ventricular cavity, lower than the trabeculi at the apex, was a 4 × 4.0-cm mass that was held against the septum by fibrous trabeculi. The endocardial surface adjacent to the thrombus had a slightly roughened appearance. The ventricular side of the thrombus was smooth and boss elated. The mass was not attached to the papillary muscles, and was excised with its trabecular attachments. On histological examination it consisted of organised thrombus. The patient was re-anticoagulated with warfarin before discharge. The patient had an uneventful postoperative recovery and thrombosis formation was not occurred with periodical follow-up examination.
Figure 1: Four chamber echocardiography showed left ventricular mass

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Figure 2: M-mode echocardiography revealed a mass in left ventricule

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   Discussion Top


Cardiac LV thrombus is most commonly associated with an area of myocardial infarction. LV thrombi can also develop in congestive heart failure and cardiomyopathies. It is unusual for cardiac intraventricular thrombus to form in the high shear environment of the left ventricle when the heart is structurally and functionally normal. We find no previous report of this happening in protein C deficiency. [3] Esmon demonstrated that protein C binds to the endothelial cell surface protein thrombomodulin and is converted to activated protein C (APC) by thrombin. The APC molecule then interacts with protein S to inactivate two critical coagulation cofactors, factors Va and VIIIa. [4] Marlar reviewed genetic, clinical manifestation and diagnosis of protein C deficiency and found that deficiency of protein C is usually transmitted in an autosomal dominant manner. In the heterozygous state the typical manifestations are recurrent venous thrombosis and associated pulmonary embolism. [5] Gruber exhibits that the homozygous state, which is rare, shows itself as fulminant intravascular coagulation. [6] Seligsohn demonstrated that absence of protein C activity despite normal amounts of the protein is due to the presence of a dysfunctional protein C molecule. [7] The clinical importance of LV thrombosis is the difficulty in making the diagnosis based on the initial clinical findings. Clinicians should be aware of this differential diagnosis in a particular patient who has not risk factors for intra-cardiac thrombosis presenting with dyspnoea. A TTE should be the initial diagnostic resource that would prompt TEE assessment in cases highly suggestive of LV thrombosis.


   Conclusions Top


Pathological examination of the LV mass revealed an organised thrombus. Results of the patient's laboratory tests for hypercoagulability showed a protein C-S deficiency (47% of normal activity). Warfarin was prescribed to treat the hypercoagulability. At 4-month follow-up, an echocardiogram showed no evidence of recurrent clot in the patient's LV, and the patient's protein C-S levels had not normalised; therefore, the warfarin was continued.

 
   References Top

1.Srichai MB, Junor C, Rodriguez LL, Stillman AE, Grimm RA, Lieber ML, et al. Clinical imaging, and pathological characteristics of left ventricular thrombus: A comparison of contrast-enhanced magnetic resonance imaging, transthoracic echocardiography, and transesophageal echocardiography with surgical or pathological validation. Am Heart J 2006;152:75-84.  Back to cited text no. 1
    
2.Ozkutlu S, Ozbarlas N, Saraclar M, Oztunc F. Left ventricular thrombosis due to acquired protein C deficiency diagnosed by two-dimensional echocardiography. Jpn Heart J 1992;33:253-8.  Back to cited text no. 2
    
3.Delewi R, Zijlstra F, Piek JJ. Left ventricular thrombus formation after acute myocardial infarction. Heart 2012;98:1743-9.  Back to cited text no. 3
    
4.Esmon CT. Regulation of blood coagulation. Biochim Biophys Acta 2000;1477:349-60.  Back to cited text no. 4
[PUBMED]    
5.Marlar RA, Mastovich S. Hereditary protein C deficiency: A review of the genetics, clinical presentation, diagnosis and treatment. Blood Coagul Fibrinolysis 1990;1:319-30.  Back to cited text no. 5
    
6.Gruber A, Mori E, del Zoppo GJ, Waxman L, Griffin JH. Alteration of fibrin network by activated protein C. Blood 1994;83:2541-8.  Back to cited text no. 6
    
7.Seligsohn U, Berger A, Abend M, Rubin L, Attias D, Zivelin A, et al. Homozygous protein C deficiency manifested by massive venous thrombosis in the newborn. N Engl J Med 1984;310:559-62.  Back to cited text no. 7
[PUBMED]    


    Figures

  [Figure 1], [Figure 2]


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