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...

The fundamental role of pulmonary vascular resistance in the Fontan circulation is obvious. Medications decreasing this resistance may have an impact on the fate of this population. Hence, we assessed noninvasively the effect of oral sildenafil on the ventriculo-arterial coupling in patients with Fontan circulation. In a single-center, prospective case series study, 23 patients with fenestrated extracardiac total cavopulmonary connection age 12-31 years were enrolled in this study. Clinical characteristics and echocardiographic examination were performed before and after a 1 week course of sildenafil at 0.5 mg/kg every 8 h. Sildenafil had no effect on heart rate and blood pressure. However, oxygen saturation was significantly increased with sildenafil (87.6 ± 4.3 vs. 90.1 ± 3.6; P < 0.0001). The calculated noninvasive ventricular end-systolic elastance (Ees) was greater after sildenafil compared with the pre-sildenafil values (1.59 ± 0.17 vs. 1.72 ± 0.27 mm Hg/ml; P = 0.001). Moreover, significant decreases in arterial elastance (Ea) (1.62 ± 0.53 vs. 1.36 ± 0.43 mm Hg/ml; P < 0.0001), ventricular end-diastolic elastance (Eed) (0.05 ± 0.021 vs. 0.04 ± 0.013; P = 0.002), and, finally, ventriculo-arterial coupling index (0.99 ± 0.26 vs. 0.76 ± 0.15; P < 0.0001) were found after sildenafil administration. The intolerable side effects that led to stopping the sildenafil occurred only in one (4 %) patient. Sildenafil has increased ventricular systolic elastance and improved ventriculo-arterial coupling in patients palliated with Fontan circulation. Short-term sildenafil was well tolerated in most of the patients with only minor side effects.

Avoiding cardiopulmonary bypass in fenestrated extracardiac total cavopulmonary connection had no direct effect on the early outcome variables.