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Image in cardiology
Diagnostic dilemma: Intracardiac mass in a woman with Behçet's syndrome
Dilema diagnóstico: masa intracardiaca en una paciente con síndrome de Behçet
Edilberto Núñez-Cabarcasa, Nilson López-Ruizb,
Corresponding author
nlopez@hptu.org.co

Corresponding author at: Calle 78B 69-240, Medellín, Colombia. Tel.: +57 4 4459000x9143; fax: +57 4 4411440.
, Alex Ramírez-Rincónc
a Postgraduate Division, Universidad Pontificia Bolivariana, Medellín, Colombia
b Cardiology Division, Hospital Pablo Tobón Uribe, Medellín, Colombia
c Internal Medicine Division, Hospital Pablo Tobón Uribe, Medellín, Colombia
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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; Two-dimensional echocardiography&#58; parasternal short axis view showing a mass &#40;asterisk&#41; on the posterior wall of the right atrium &#40;RA&#41;&#46; &#40;B&#41; Three-dimensional echocardiography&#58; right ventricle &#40;RV&#41; inflow tract view displaying a round shaped mass &#40;asterisk&#41; with a stalk attached to the posterior wall of the RA&#46; &#40;C&#41; Delayed-enhancement cardiovascular magnetic resonance demonstrating the lack of gadolinium contrast uptake &#40;asterisk&#41; as the thombus is avascular&#46; &#40;D&#41; Three-dimensional echocardiography&#58; normal RV inflow tract view without evidence of thrombus after sixth months of anticoagulation therapy&#46; LA&#58; left atrium&#46;</p>"
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for characterizing the pseudotumor&#46; The pregadolinium gradient echo-CMR revealed low signal intensity in the tumor and the contrast-enhanced scan showed lack of enhancement of the tumor mass &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>C&#41; favoring the diagnosis of a thrombus&#46; Concurrently&#44; a computed pulmonary angiography ruled out both embolism and aneurisms&#46; The fever subsided and the patient&#39;s status improved over the days&#44; and then she was discharged on anticoagulant therapy with warfarin&#46; On a new transthoracic echocardiogram&#44; performed 6 months later &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>D&#41;&#44; there was no evidence of the thrombus previously seen in the right atrium&#44; so warfarin was withheld&#46; She has remained stable one year later without symptoms of thrombus recurrence&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">In one of the largest case series published so far&#44; 52 &#40;6&#37;&#41; patients from a cohort of 807 patients with Beh&#231;et&#39;s disease &#40;BD&#41; had some form of cardiac involvement including pericarditis&#44; endocarditis&#44; myocardial infarction&#44; endomyocardial fibrosis&#44; and aneurysms of the coronary arteries of sinus of Valsalva&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> However&#44; intracardiac thrombosis is a rare finding with only 32 cases reported in the medical literature&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> In an analysis of 25 patients&#44; 56&#37; of them had clinical manifestations like fever&#44; hemoptysis&#44; dyspnea&#44; and cough&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Cardiac thrombi affected most often the right side &#40;right ventricle&#44; 52&#37;&#59; right atrium&#44; 24&#37;&#41; and were unusual in the left or both chambers &#40;4 and 16&#37; of cases&#44; respectively&#41;&#46; Besides&#44; intracardiac thrombi were also associated with thrombosis of the vena cava and pulmonary embolism &#40;28 and 60&#37; of cases&#44; respectively&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a> The echocardiographic appearance of thrombi may suggest a tumor&#44; and several patients have undergone cardiac surgery but histological examination demonstrated thrombi in all cases&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> CMR is a useful technique for the differential diagnosis between cardiac thrombus and tumor and might be helpful in difficult cases like the one presented here&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> There is no consensus about the optimal treatment of intracardiac thrombosis in BD&#46; Most patients receive immunosuppressive therapy &#40;usually steroids in combination with azathioprine or cyclophosphamide&#41; along with anticoagulation but some have reported the thrombi resolution only with immunosuppression particularly when hemoptysis or pulmonary artery aneurysms preclude the use of anticoagulants&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;4</span></a> Complete remission of intracardiac thrombosis has occurred in 75&#37; of cases with corticosteroids and anticoagulation&#46; Even though surgical resection seems an option when thrombi persist despite optimal medical therapy&#44;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> the risk of recurrence and death is an issue&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;5</span></a> Finally&#44; although the 5-year survival rate is lower in BD patients with heart involvement &#40;84&#37; vs&#46; 96&#37; in patients without cardiac lesions&#41;&#44;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> the type of affectation is heterogeneous and prognosis differs accordingly&#46; Notwithstanding&#44; except for the risk of pulmonary embolism&#44; the long-term prognosis of BD patients with intracardiac thrombi seems to be good as long as they receive only medical management&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Financial support</span><p id="par0015" class="elsevierStylePara elsevierViewall">None&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Conflict of interest</span><p id="par0020" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflict of interest&#46;</p></span></span>"
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