Discussion

Behçet’s disease is a multi-systemic disease that affects young adults, especially in Mediterranean, Middle East, and Far East countries. Since there is no specific laboratory test, it is very difficult to diagnose. Clinical features such as orogenital aphthae, ocular and skin lesions, arthritis and neurological, gastrointestinal, vascular, and pulmonary symptoms are helpful for diagnosis. Cardiovascular involvement in Behçet’s disease is estimated to range from 7 to 46% in adult Behçet’s disease patients. ^{Reference Gürgün, Ercan and Ceyhan1} In Behçet’s disease, vasculitis lesions may affect veins and arteries of all sizes. It has been reported that venous involvement is 29%, and arterial involvement varies from 8 to 18%. ^{Reference Gürgün, Ercan and Ceyhan1} Intracardiac thrombosis is an extremely rare complication of Behçet’s disease. It is more common in male gender and advanced ages, but it is very rare in the children. ² The diagnosis of Behçet’s disease in childhood is difficult due to its rarity and lack of diagnostic criteria. The initial symptoms occur in 4–26% of the patients under the age of 16 years. ^{Reference Yıldız, Köker, Adrovic, Şahin, Barut and Kasapçopur3} Although cardiovascular complications are extremely rare, they may occur as the first sign of the disease in childhood. ^{Reference Demir, Sag, Kaya Akca, Hazirolan, Bilginer and Ozen4} Our case had only two critera for diagnosis of Behçet’s disease and did not meet diagnostic criteria of Behçet’s disease. However, she presented with intracardiac thrombosis and peripheral pulmonary arterial embolism which could be considered pathognomic for Behçet’s diseae in childhood. ^{Reference Demir, Sag, Kaya Akca, Hazirolan, Bilginer and Ozen4} There were no clinical and laboratory findings of other diseases that would explain these thrombosis events. There are no large series of cases that include children published. In one study, 2 of 26 patients with paediatric Behçet’s disease were encountered with severe atypical cardiac presentations. ^{Reference Ben-David, Gur, Ilivitzki, Khoury, Bentur and Butbul5} These presentations were intracardiac thrombi and left anterior descending coronary arterial obstruction causing myocardial infarction, and pulmonary arterial aneurysm with pulmonary embolism. These two patients had a delayed diagnosis of Behçet’s disease, demonstrating the need to be highly suspicious of this disease in young patients with intracardiac thrombosis. ^{Reference Ben-David, Gur, Ilivitzki, Khoury, Bentur and Butbul5} In another study, 46 patients with Behçet’s disease were evaluated retrospectively. Ten patients (21.7%) had thrombosis and this was the first sign of Behçet’s disease in seven patients. One of them had pulmonary artery thrombosis, and one patient had intracardiac thrombosis. All patients had received anticoagulant therapy with immunosuppressive treatment. ^{Reference Guzelkucuk and Gokcebay6} Intracardiac thrombosis related to Behçet’s disease can be seen in both ventricular chambers. Right ventricular thrombus is frequently associated with aneurysmatic dilatation of the pulmonary vessels, which reflects systemic inflammatory processes and pulmonary vasculitis. We also detected mild pulmonary arterial dilatation in our patient by CT examination. In a review performed in adult patients with Behçet’s disease, intracardiac thrombus is more frequent in patients with pulmonary arterial involvement than patients without such involvement (19.7–0.3%). ^{Reference Eroglu, Torgutalp and Baysal7}

Optimal therapeutic strategies for patients with intracardiac thrombosis relating to Behçet’s disease still remain controversial. Surgical treatment can be performed in patients with massive thrombosis and heart failure, despite optimal medical treatment. These patients may also undergo surgery due to the suspicion of an intracardiac tumour. In controlled studies, surgical treatment is not supported due to the risk of recurrence and post-operative complications. Cardiac thrombosis may occur again in a short time if corticosteroid treatment is not given after surgical excision. ^{Reference Abidov and Alpert8} Previous reports suggest that combined immunosuppressive prednisone and cyclophosphamide therapy are required to treat Behçet’s disease-related intracardiac thrombosis. Intracardiac thrombosis related to Behçet’s disease may show resolution when treated only immunosupressive treatment and/or anticoagulation. The benefit of anticoagulation in the vasculitis of Behçet’s disease is still debated. However, venous, pulmonary arterial, and intracardiac thrombosis in Behçet’s disease are usually managed with anticoagulation and immunsuppressive treatment. The only contraindication to anticoagulation is the presence of pulmonary artery aneurysm due to the risk of rupture. ^{Reference Hatemi, Christensen and Bang9}

In our case, after 1 month of treatment with combined high doses of immunosuppressive, corticosteroid, and low-dose anticoagulation, highly mobile intracardiac thrombi and multiple pulmonary arterial thrombi persisted. Intracardiac thrombosis in our case was considered to be at the critical dimension that could lead to sudden death by occluding the pulmonary artery ^{Reference Cetin, Ekici and Ünal10} and was therefore surgically removed. Although recurrent thrombosis is frequent in Behçet’s disease, we did not observe any clinical or laboratory evidence of recurrent thrombosis in our case until the last examination.