Ruptured aneurysm of the sinus of Valsalva in a patient with Behcet's disease

Koh, Kwang Kon; Lee, Ki Hoon; Kim, Sam Soo; Lee, Seung−Chul; Jin, Sung Hoon; Seong, In Whan

doi:10.1016/0167-5273(94)90186-4

Cited by 30 publications

(19 citation statements)

References 4 publications

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“…Endocardial involvement may be limited to heart valves or involve the endocardial surface of cardiac cavities. Several reports of valvular heart disease exist in literature including: mitral valve prolapse (MVP), aortic valve prolapse, mitral or aortic insufficiency, aneurysm of sinus of valsalva, and endocarditis 6‐8 . Endocardial involvement may come about as endocardial fibrosis involving the right or left ventricle, 9 and may be associated with intracavitary thrombus formation, 10 which may lead to pulmonary emboli.…”

Section: Discussionmentioning

confidence: 99%

Cardiac and Great Vessel Involvement in “Behcet's Disease”

Marzban

Mandegar

Karimi

et al. 2008

Journal of Cardiac Surgery

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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...

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Section: Discussionmentioning

confidence: 99%

Cardiac and Great Vessel Involvement in “Behcet's Disease”

Marzban

Mandegar

Karimi

et al. 2008

Journal of Cardiac Surgery

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“…Congenital weakness of the aortic annulus is implicated in the subgroup of patients with multiple aneurysms and a hereditary connective disorder, such as Ehlers-Danlos or Marfan syndrome [22, 23], resulting in diffuse enlargement of the sinuses rather than focal aneurysmal dilatation of a segment of a sinus, should be sought. There have been case reports suggesting Takayasu’s arteritis [24] or Behcet’s disease [25] as the cause of aneurysm formation.…”

Section: Acquired Aneurysmsmentioning

confidence: 99%

Aneurysms of the Sinuses of Valsalva

2006

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Sinus of Valsalva aneurysms are rare cardiac anomalies which may be acquired or congenital, most commonly involving the right or noncoronary sinuses. The congenital aneurysms are more common and often caused by weakness at the junction of the aortic media and the annulus fibrosus. Acquired aneurysms are caused by conditions affecting the aortic wall, such as infections (syphilis, bacterial endocarditis, or tuberculosis), trauma, or connective tissue disorders. Unruptured aneurysms are usually found incidentally during diagnostic studies. More commonly, sinus of Valsalvaaneurysms are diagnosed after clinical sequelae of rupture. Diagnosis of sinus of Valsalva aneurysm is facilitated by echocardiography, contrast aortography, and more recently, magnetic resonance imaging. Repair is generally required for ruptured aneurysms; unruptured aneurysms encroaching on nearby structures, causing myocardial ischemia, or having the potential to rupture warrant repair. A review of the literature is presented focusing on anatomy, clinical presentation of ruptured and unruptured aneurysms, noninvasive diagnostic modalities, and techniques for repair of this anomaly.

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“…2 Almost all such aneurysms project into the right atrium or right ventricle, but may rupture into the pulmonary artery, left ventricle, left atrium or the free pericardial cavity. 2,5 In the present case, the aneurysm projected into the right and left ventricles, and was at risk of rupture into these chambers.…”

Section: Discussionmentioning

confidence: 99%

“…Acquired etiologies include chronic inflammatory diseases, autoimmune disease and other conditions. 2,5 Only 7 cases of aneurysm of the sinus of Valsalva accompanying Behçet's disease have been reported, and only 2 were unruptured. 4,7 Some asymptomatic cases of ruptured aneurysm of the sinus of Valsalva have been reported.…”

Section: Discussionmentioning

confidence: 99%