Intracardiac thrombosis in a young female as first presentation of primary antiphospholipid syndrome: A case report
Stylianos T. Panopoulos, Maria G. Tektonidou
Mediterr J Rheumatol 2016; 27(4):194-97
mediterranean journal
of RHEUMATOLOGY
EAAHNIKH PEYMATOAOTIA
E-ISSN: 2459-3516
mediterranean journal of rheumatology June 2016 | Volume 27 | Issue 4
mediterranean journal
of RHEUMATOLOGY
EAAHNIKH PEYMATQAOriA
27 4
2016
© Panopoulos S T, Tektonidou M G
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International I
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CASE REPORT
Intracardiac thrombosis in a young female as first presentation of primary antiphospholipid syndrome: A case report
Stylianos T. Panopoulos, Maria G. Tektonidou
First Propaedeutic Department of Internal Medicine, Rheumatologie Unit, Laikon Hospital and Joint Academic Rheumatology Programme of School of Medicine, National and Kapodistrian University of Athens
ABSTRACT
Primary antiphospholipid syndrome (PAPS) is an entity characterized by spontaneous and recurrent abortion and recurrent vascular thromboses (arterial and venous). Intracardiac thrombosis is a rare but life-threatening complication of PAPS. Herein we describe a 21 year-old woman admitted to hospital due to left pleurodynia and shortness of breath with no history of thrombotic events. Helix chest tomography scan disclosed pulmonary embolism as well as a filling defect of the inferior vena cava, which in subsequent cardiac magnetic resonance (MRI) proved to be intracardiac thrombus. Laboratory tests showed triple positivity for antiphospholipid antibodies, renal involvement and thrombocytopenia; PAPS, possibly catastrophic, was diagnosed. The patient was treated with iv pulses of corticosteroids, cyclophosphamide, intravenous immunoglobulin and oral anticoagulation (INR levels between 2.5 and 3), improved gradually and was discharged after 15
days of hospitalization. At 6-month follow-up new cardiac MRI revealed complete resolution of the thrombus. Patients with APS that present with pulmonary embolism should be investigated for the possibility of intracardial thrombus. Indefinite anticoagulation treatment in these patients is warranted due to high recurrence rates.
Mediterr J Rheumatol 2016;27(4):194-7 https://doi.org/10.31138/mjn27A.194
Corresponding author:
Maria Tektonidou, MD, PhD First Propaedeutic Department of Internal Medicine, Rheumatologie Unit Laikon Hospital, School of Medicine, National and Kapodistrian University of Athens
75 Mikras Asias Str., 11527 Athens, Greece Tel: 2107462710, Fax: 2107462710, Email: mtektonidou@gmail.com
Keywords: primary antiphospholipid syndrome, cardiac thrombosis, treatment.
Cite this article as: Panopoulos S T, Tektonidou M G. Intracardiac thrombosis in a young female as first presentation of primary antiphospholipid syndrome: A case report. Mediterr J Rheumatol 2016;27(4):194-7.
CASE
A 21-year-old woman with no previous medical or family history of thrombotic disease or history of any drug abuse presented at the emergency room with left pleurodynia, shortness of breath and fever. On examination she was in respiratory distress, with tachypnea (20 breaths/min), blood pressure of 140/80 mmHg, tachycardia (120 beats/min) and body temperature 38.2oC. Chest auscultation revealed decreased breath sounds of the left lower lung field with no other abnormal findings from clinical examination. Arterial blood gas analysis showed pH: 7.413; pO2: 61 mmHg; pOO^ 31.4 mmHg and bicarbonate: 22.4 mmol/L. On electrocardiogram there was sinus tachycardia without significant ST-T segment change and on chest X-ray left pleural effusion was noted. Subsequent helix chest tomography scan disclosed pulmonary embolism as well as a filling defect of the inferior vena cava at its junction with the right atrium after contrast agent injection; a finding that was suspicious of intracardiac thrombus. However, transthoracic echocardiography did not confirm the presence of intracardiac mass or any other morphological abnormalities of the valves. Cardiac magnetic resonance (MRI) is not available in our hospital. Addi-
tionally, venous duplex examination of the legs showed normal blood flow with absence of cloats in any deep vein, rulling out the possibility of deep vein thrombosis (DVT).
Laboratory tests revealed highly elevated inflammatory markers, positive antinuclear antibody 1/640 (speckled pattern), a prolonged activated partial prothrombin time (APTT) of 61s, mild thrombocytopenia (80,000/pL) and active urine sediment indicative of renal involvement. Treatment with Enoxaparin 8000 iu bid and pred-nisolone iv 1 mg/kg/day was initiated with the clinical suspicion of systemic lupus erythematosus (SLE). Further immunological tests showed highly elevated IgG anticardiolipin (115 GPL) and IgG anti-p2-glycoprotein antibodies and positive lupus anticoagulant (LA), whilst anti-double stranded DNA and anti-Smith antibodies were tested negative. Complements levels C3 and C4 were within normal limits. The third day of her hospital-ization, she developed liveloid vasculitis in both hands. The patient did not undergo renal biopsy due to high risk for new embolic event in case of temporary withdrawal of anticoagulation.
The patient was diagnosed with primary antiphospho-lipid syndrome (PAPS) with clinical evidence of ongoing
Figure 1. Computed tomography scan of the lungs shows a filling defect (arrow) in the inferior vena cava-right atrium, suspicious of intracardiac thrombosis
mediterranean journal
of RHEUMATOLOGY
EAAHNIKH PEYMATQAOriA
27 4
2016
Table 1. Differential diagnosis of intracardiac masses
Cardiac Mass T1 weighed sequence T2 weighed sequence LGE
Benign tumors
Myxoma isointense hyperintense heterogenous
Fibroma isointense hypointense increased enhancement
Lipoma hyperintense hyperintense no uptake
Rabdomyoma isointense isointense no/minimal uptake
Malignant tumors
Angiosarcoma heterogenous heterogenous heterogenous
Lymphoma isointense isointense no/minimal uptake
Rabdomyosarcoma isointense hyperintense homogenous
Metastasis hypointense hyperintense heterogenous
Thrombus hyperintense if acute (hypo- if chronic) hyperintense if acute (hypo- if chronic) no uptake
catastrophic syndrome because of the development in less than one week period of pulmonary embolism with the potentially coexistent intracardiac thrombosis, renal involvement and livedoid vasculitis, in association with positive LA test and high levels of IgG anticardiolipin and anti-p2-glycoprotein antibodies. She was treated with 3 iv pulses of 1 gr methyl-prednisolone, 1 iv pulse of 1.2 gr cyclophosphamide (750 mg/m2, patient's BSA: 1.5m2) and intravenous immunoglobulin 400 mg/kg for 5 consecutive days. The patient improved gradually and was discharged after 15 days of hospitalization in good general condition. Her treatment included per os methylprednisolone 32 mg/day, mycophenolate mofetil 2gr/day and hydroxychloroquine 400 mg/day. The cardiac MRI in an outpatient diagnostic center showed a papillary mass (2x2cm), with hyperintense signal on T1-and hypointense signal on T2-weighted sequence and no enhancement after gadolinium injection, situated at the inferior vena cava at its junction with the right atrium, confirming the presence of intracardiac thrombus. At 6-month follow-up the patient was in perfect clinical condition without any symptoms, continued the same treatment (dosage of methylprednisone was tapered to 8 mg/day) and a new cardiac MRI that was performed revealed complete resolution of the thrombus.
DISCUSSION
APS has a variety of clinical manifestations with DVT being the most common as it develops in approximately 36-40% of patients, whilst pulmonary embolism is manifested in approximately 9% of APS patients.1 Valvular abnormalities represent the most common cardiac manifestations ranging from 12 to 35% of cases.1-2 Other heart manifestations include myocardial infarc-
tion, pulmonary hypertension, dilated myocardiopathy, coronary artery thrombosis and intracardial thrombosis. Intracardiac thrombosis is a rare but potentially life-threatening cardiac complication of APS as it can cause pulmonary and systemic embolic events. The differential diagnosis of intracardiac masses includes mainly benign or malignant tumors (most commonly myxoma), and thrombus (Table 1).3 Right atrial thrombi can either originate from venous emboli that have become entrapped in the right heart or may develop in situ in the right atrium. In several cases, these thrombi are poorly visualised on trans-thoracic echocardiogra-phy (TTE), and a trans-oesophageal echocardiography (TEE) may be necessary for their detection. In general, TTE and TEE provide information only for the location and size of the intracardiac mass and further imaging by CT scan and, moreover, MRI is required in order to characterize the tissue composition and to differentiate between tumors and thrombus.4 The existing data on the management of intracardiac thrombosis in patients with APS are insufficient, as only sporadic cases with this rare manifestation have been reported.3,5,6,7 In some of these cases including the one presented in this report, complete resolution of thrombi with anticoagulation alone has been described, but surgical intervention was in several cases required to remove the thrombus due to the great size of the mass and the high risk for recurrent systemic embolism. According to recommendations published in 2003 by a committee consensus for the treatment of cardiac disease in APS,8 administration of intensive warfarin anticoagulation is always recommended in case of intrac-ardiac thrombosis, while the decision for surgical intervention is individualised depending on the position and
size of the thrombus, the hemodynamic condition of the patient and the risk of recurrent events. The maintenance treatment for APS patients with thrombotic events, given the risk of recurrence,9 requires lifelong anticoagulation treatment with warfarin (targeted to an international normalized ratio of 2.0-3.0). If thrombotic events recur, warfarin should be increased by means of high-intensity therapy. Alternatively, addition of anti-platelets to anticoagulation treatment can also be considered.10
In conclusion, the presence of intracardial thrombi is a rare but life-threatening complication of APS. Patients with APS that present with pulmonary embolism, especially if there is no proof of DVT, should be investigated for the possibility of intracardial thrombus. Indefinite anticoagulation treatment in these patients is warranted due to high recurrence rates.
CONFLICT OF INTEREST
The authors declare no conflict of interest.
the 13th International Congress on antiphospholipid antibodies. Lupus 2011;20:206-18.
REFERENCES
1. Cervera R, Piette J C, Font J, Khamashta M A, Shoenfeld Y, Camps M T, et al. Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 2002;46:1019-27.
2. Cervera R, Tektonidou M G, Espinosa G, Cabral A R, González E B, Erkan D, et al. Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations (I): catastrophic APS, APS nephropathy and heart valve lesions. Lupus 2011;20:165-73.
3. Cianciulli T F, Saccheri M C, Redruello H J, Cosarinsky L A, Celano L, Trila C S, et al. Right atrial thrombus mimicking myxoma with pulmonary embolism in a patient with systemic lupus erythematosus and secondary antiphospholipid syndrome. Tex Heart Inst J 2008;35:454-7.
4. Mavrogeni S I, Sfikakis P P Kitas G D, Kolovou G, Tektonidou MG. Cardiac involvement in antiphospholipid syndrome: The diagnostic role of noninvasive cardiac imaging. Semin Arthritis Rheum 2016;45:611-6.
5. Lim E, Wicks I, Roberts L J. Intracardiac thrombosis complicating antiphospholipid antibody syndrome. Intern Med J 2004;34:135-7.
6. Cianciulli T F, Saccheri M C, Lax J A, Neme R O, Sevillano J F, Maiori M E, et al. Left ventricular thrombus mimicking primary cardiac tumor in a patient with primary antiphospholipid syndrome and recurrent systemic embolism. Cardiol J 2009;16:560-3.
7. Aguilar J A, Summerson C. Intracardiac thrombus in anti-phospholipid antibody syndrome. J Am Soc Echocardiogr 2000;13:873-5.
8. Lockshin M, Tenedios F, Petri M, McCarty G, Forastiero R, Krilis S,
et al. Cardiac disease in the antiphospholipid syndrome: recommendations for treatment. Committee consensus report. Lupus 2003;12:518-23.
9. Cervera R, Khamashta M A, Shoenfeld Y, Camps M T, Jacobsen
S, Kiss E, et al. Morbidity and mortality in the antiphospholip-id syndrome during a 5-year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis 2009;68:1428-32.
10. Ruiz-Irastorza G, Cuadrado M J, Ruiz-Arruza I, Brey R, Crowther M, Derksen R, et al. Evidence-based recommendations for the prevention and long-term management of thrombosis in anti-phospholipid antibody-positive patients: report of a task force at