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Báo cáo y học: "Sinus of valsalva aneurysm in Blau’s syndrom"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học Wertheim cung cấp cho các bạn kiến thức về ngành y đề tài: Sinus of valsalva aneurysm in Blau’s syndrome. | Mourad and Tang Journal of Cardiothoracic Surgery 2010 5 16 http www.cardiothoracicsurgery.Org content 5 1 16 JOTS JOURNAL OF CARDIOTHORACIC SURGERY CASE REPORT Open Access Sinus of valsalva aneurysm in Blau s syndrome Faisal Mourad Augustine Tang Abstract Blau syndrome is a rare granulomatous disorder inherited in an autosomal dominant manner characterized by the early appearance of granulomatous arthritis skin rash and anterior uveitis. There are very few data on the cardiovascular manifestations of Blau syndrome. Here we report the first case of sinus of valsava aneurysm in Blau syndrome. In isolated unruptured aneurysms of a sinus of Valsalva without compromise of the aortic valve and or the coronary ostia repair may be accomplished by simple placation of the aneurysm or excision of the aneurysm s and patch closure of the defect s between the aortic annulus and the sinu-vascular ridge. Because of the particular conditions in our case the repair was performed with replacement of the aortic valve and root using a composite graft employing a modified Bentall s technique. Case Report A 37 year old lady with Blau syndrome was being followed up by transthoracic echocardiogram for mild to moderate mitral valve prolapse was referred to us after accidental discovery of an echogenic mass associated with the aortic root and right atrium. She had been complaining of slight shortness of breath complying with NYHA grade I. Past medical history included congenital bilateral ptosis De Quervain s thyroiditis congenital bilateral hearing problems. Apart from her cardiac manifestations the clinical picture was consistent with Blau s Syndrome as suggested by The Clinical Genetics Department in St. Mary s hospital Manchester. Her older sister died as a neonate shortly after birth from complex congenital heart disease although no further detail was available. On examination she was found to have saddle nose deformity and camptodactyly. She also has a strikingly erythematous rash .