Intracardiac thrombus in Behçet’s disease: four new cases and a comprehensive literature review

Abstract

Behçet's disease (BD) is a multisystem inflammatory disorder. Intracardiac thrombus formation (ICTF) is an uncommon but important complication of BD. To highlight recent insights into this disease, we aimed to review ICTF and other systemic involvements associated with ICTF in BD. We conducted a comprehensive review of the relevant literature in MEDLINE and EMBASE from 1966 to 2014 to analyze cumulated data about ICTF in BD. We aimed to evaluate 93 cases of BD with ICT (group 1), four of which have been recently identified and have not been discussed in the relevant literature yet, and to compare the frequency of pulmonary, venous and arterial involvements in group 1 and general Behçet population (group 2). The right heart was the most common site of ICTF in group 1. Pulmonary involvement, venous involvement (especially venous thrombosis) and arterial involvement were more frequent in group 1 than in group 2 (56 vs. 0.7 %, 42 vs. 10 % and 38 vs. 0.8 %, respectively, p < 0.0001). The diagnosis of BD should be considered if a patient presents with a mass in the right-sided cardiac chambers, even in the absence of the characteristic clinical manifestations of the illness. This approach is particularly applicable if the patient is a young man from the Mediterranean basin or the Middle East. All Behçet patients with ICTF must be investigated with thoracic computed tomography for pulmonary and arterial involvements and lower extremity venous Doppler ultrasonography for venous thrombosis, regardless of whether they are symptomatic for these systems.

Intracardiac thrombosis in Behçet's Disease successfully treated with immunosuppressive agents: A case of vascular pathergy phenomenon

“…BD is unique among other vasculitides as it usually affects veins rather than arteries and it has significant thrombotic tendency associated with vascular inflammation, which cannot be explained by thrombophilic factors (3). The most common type of vascular involvement is lower extremity of detection of ICT, fever, hemoptysis, dyspnea, and cough were the predominant symptoms (11,12). In 40-56% of patients with ICT PAI was detected, 42-56% of them had venous thrombosis, and 52-55% had PE (5,11,12).…”

“…The most common type of vascular involvement is lower extremity of detection of ICT, fever, hemoptysis, dyspnea, and cough were the predominant symptoms (11,12). In 40-56% of patients with ICT PAI was detected, 42-56% of them had venous thrombosis, and 52-55% had PE (5,11,12). ICT is uniformly associated with an elevated ESR but it is a poor indicator of disease activity (11,13).…”

“…It may be present as pericarditis, myocarditis, endocarditis with valvular regurgitation, endomyocardial fibrosis, coronary arteritis, Valsalva sinus aneurysms and as intracardiac thrombus (ICT) (8,9,10). ICT are strongly associated with pulmonary artery involvement and are mostly located on the right side of the heart probably because of the vena cava extension (3,8,11,12). These thrombi contain inflammatory cell infiltrates and are tightly bound to the underlying endocardium or myocardium (3).…”

Intracardiac Thrombosis and Coronary-to-Pulmonary Artery Fistula with Pulmonary Embolism and Budd-Chiari Syndrome in Behçet's Disease: A Case Report

“…Only 66 cases of BD patients with ICT have been reported in the literature. [8] The right heart is the most common localization for the thrombosis, but the reason for the frequency of rightsided ICT associated with BD is still unknown. Soulami et al [9] postulated that endomyocardial fibrosis has a role in the development of ICT in some patients.…”

Vascular Manifestations of Behçet’s Disease

“…Increased plasma homocysteine levels are also a risk factor for thrombosis in BD. It was shown that mean plasma homocysteine levels in BD patients were substantially higher in comparison to that in healthy subjects, which led to the suggestion that another conceivable pathogenic mechanism of thrombosis in BD may be related to the presence of antiphospholipid antibodies, which have been reported in 18% of cases in a study [103].…”