Behcet's disease is a multisystem disorder and classified as "vasculitic syndrome with a wide variety of clinical manifestations." Cardiac involvement is very rare but can occur with different presentations including: pericarditis, cardiomyopathy, endocarditis, endomyocardial fibrosis, intracavitary thrombosis, and coronary artery disease. Great vessel involvement is more common. Recurrent Phlebitis, commonly involving large vessels (superior vena cava, inferior vena cava, hepatic veins) and cerebral veins are the sole presentation in this regard. Arterial involvement is expressed by aneurysm or pseudoaneurysmal formation. Due to the wide variety of cardiovascular manifestations and the resulting high mortality, cardiac surgeons should be familiar with this disease. In this paper we review the articles and introduce our four cases presenting with aneurysm of ascending aorta with free aortic insufficiency, aneurysm of descending aorta, pulmonary artery aneurysm, and pseudoaneurysm of aortic arch. doi: 10.1111/j. 1540-8191.2008.00607.x (J Card Surg 200823:765-768) Behcet's disease (BD) was first discovered in 1937 by Hulusi Behcet. It is an autoimmune disease and is classified as a vasculitic syndrome. This disease is a multisystem disorder with a wide variety of clinical manifestations including skin, eye, musculoskeletal, neurologic, and cardiovascular presentations. 1,2 The spectrum of cardiac diseases may include pericarditis, coronary artery stenosis or aneurysm, myocarditis, cardiomyopathy, congestive heart failure, valvular pathology, endocarditis or endocavitary thrombosis, aneurysm of aorta and its branches, pulmonary artery aneurysm, or venous thrombosis. Due to its wide variety of pathologies, disease presentation may vary in each case.
CASE REPORT Case 1A 24-year-old man, a known case of BD, under followup by the rheumatology clinic in our center, developed exertional chest pain a few weeks prior to presentation to our ward. His workup, including chest xray (CXR), computed tomography (CT) scan, transthoracic echocardiography, and aortography, showed cardiomegaly, severe aortic insufficiency, and a huge aneurysm of ascending aorta as is shown in (Fig. 1). aortic valve no. 23. The patient had a smooth postoperative course and an uneventful recovery, but 20 days postoperative the patient was operated on once again Address for correspondence: Mehrab Marzban, M.D., Tehran Heart Center, Tehran, Iran. Fax: +98-21-88029256; e-mail: mehrabmarzban2007@yahoo.com due to massive pericardial effusion resulting from subxiphoid drainage. Now, two years postoperative, the patient is on maintenance prednisolone therapy.
Case 2A 50-year-old woman, a known case of BD with cutaneous and ocular manifestation on prednisolone and colchicine, presented to our center with a history of upper abdominal pain, mild dysphagia, and weight loss for a few weeks. Her workup, including CXR, echocardiography, CT scan, and aortogram, showed a saccular aneurysm of descending aorta, just above the diaphragm (Fig. 2). The patient...