We have presented an unusual patient with Behçet's disease and a large (10.1 × 14.8 cm) left ventricular pseudoaneurysm. Behçet's disease is a systemic disorder with mucocutaneous, ophthalmic, neurological, cardiovascular, pulmonary, gastrointestinal, urogenital and musculoskeletal involvement. Its vascular manifestations are thrombophlebitis and, less frequently, arterial lesions such as pseudoaneurysms, occlusions and stenoses [8]. About 8% of the patients with Behçet's disease have severe vascular complications such as arterial pseudoaneurysms and occlusions [1]. Pseudoaneurysms are the most common form of arterial involvement in Behçet's disease [3].
Cardiac involvement is rare in Behçet's disease [9] and engages only about 6% of patients [4]. Cardiac involvement in this disorder is a diffuse process that involves both cardiac structure and vascular elements. Higher incidences of interatrial septum aneurysm (6% to 31%), mitral valve prolapse (3% to 25%), mitral regurgitation (6% to 40%), and aneurysmal dilatations of valsalva sinus and ascending aorta were observed in the Behçet's disease patients than in the normal subjects [10]. Pericarditis, myocarditis, endocardial fibrosis, conduction defects, and aortic regurgitation were also observed [11]. Although left ventricular aneurysms with Behçet's disease have occasionally been reported, we found only one case of cardiac pseudoaneurysm in these patients reported in literature. Rolland et al. [4] reported a 29 years old patient with Behçet's syndrome and a false left ventricular aneurysm and coronary artery aneurysm, which were repaired under cardiopulmonary bypass with no postoperative complications.
Cardiac pseudoaneurysm is defined as a rupture of the myocardium that is contained by pericardial adhesions or the epicardial wall. This phenomenon can be explained by myocardial fragility induced by ischemia due to vasculitis process of Behçet's disease [4]. Because there was no evidence suggesting coronary artery disease in his coronary angiography of our patient, it could be assumed that this pseudoaneurysm was resulted from rupture of the left ventricle due to angiitis and the myocardial fragility induced by ischemia.
In contrast to a true ventricular aneurysm, in which the wall is composed of myocardial scar tissue, the wall of a pseudoaneurysm is composed of thick fibrous tissue and pericardium [12]. In our case, pseudoaneurysm was consisted of profuse fibrous tissues and thromboses in various stages.
Cardiac pseudoaneurysms have the potential to leak or rupture and can be the source of peripheral emboli [10]. Different reports have discussed that such a contained rupture has a greater propensity for rupture than a true aneurysm, whose wall contains myocardium. Rupture of a left ventricular pseudoaneurysm is usually fatal; hence appropriate recognition and treatment (early surgery) even for asymptomatic patients is strongly recommended [7].
The diagnosis of pseudoaneurysm is not straightforward and is rarely suggested by clinical signs and symptoms [7]. In our patient the pseudoaneurysm presented with nonspecific symptoms and signs. Thus, such a diagnosis was highly unlikely before getting the results of imaging. Various imaging methods have been used to diagnose pseudoaneurysm, including two-dimensional and contrast echocardiography [13], computed tomography, magnetic resonance imaging, and left ventricular angiography. Each has its advantages and disadvantages but echocardiography has become the most common examination used for first diagnosis because it can evaluate other associations such as valvular regurgitation, thrombus formation, and ventricular function, are often important supplements to clinical management [7].
Like our patient, chest radiography sometimes shows a localized bulge on the cardiac silhouette. On computed tomography, pseudoaneurysms are characterized by an abrupt disappearance of the myocardial wall at the border of the pseudoaneurysm. Magnetic resonance imaging shows the low signal of the pericardium, which constitutes the only wall of the pseudoaneurysm [7].
Surgical repair is usually recommended when a left ventricular pseudoaneurysm is detected [14, 15]. In this case surgical intervention was mandatory, partly due to the young age of the patient. However, in cases of post-infarction left ventricular pseudoaneurysm, which is one of the most common causes, routine surgical repair regardless of other clinical characteristics of the patient remains as a matter of discussion. Some authors believe that the necessity of surgical repair in these cases should be individualized for each patient [7, 16].
In patients with post-infarction left ventricular pseudoaneurysm, surgical repair of pseudoaneurysm was associated with an acceptable surgical mortality rate [7] and the long term outcome appears relatively benign [13] and late death was related primarily to the underlying disease or cardiac dysfunction [7]. There is no data on the long term prognosis of patients with cardiac pseudoaneurysms in Behçet's disease; however, long-term survival could not be expected given the diffuse involvement of cardiac structure and vascular elements [10].
Considering its fatality and nonspecific manifestations, one should consider cardiac pseudoaneurysms as a potential risk in any patient with Behçet's disease. Thanks to early diagnosis and surgery, our patient was treated successfully and had no complications in a follow-up period of 24 months.
