Rupture of a dissecting aneurysm into the superior vena cava in Marfan's syndrome

Aneurysmal dilatation of the aorta with subsequent dissection or rupture occurs frequently in patients with Marfan's syndrome. These complications are among the major causes of death. We report the case of a 51-year-old man with annulo-aortic ectasia in Marfan's syndrome. Acute aortic dissection and rupture into the superior vena cava occurred 8 years after aortic valve replacement. The preoperative diagnosis was made by right heart catheterization and computed tomography. A markedly increased left-to-right shunt occurred with rapid enlargement of the fistula due to the fragility of the aortic wall characteristic of Marfan's syndrome. Postmortem examination demonstrated severe medial necrosis with rupture of the aortic wall into the superior vena cava which was adherent to the suture line of the aortotomy from the previous surgery. Type A aortic dissection with severe congestive heart failure strongly suggested rupture into the pulmonary circulation.     

Ruptured sinus of Valsalva aneurysm simulating endocarditis

OBJECTIVE: To report on a patient with Wilms' tumor treated by partial nephrectomy with 31 years survival. METHODS: Herein we describe a 33-year-old patient who had undergone surgery for a right renal tumor at age 2 years and 10 months. A partial nephrectomy was performed because the patient had left ureterohydronephrosis. Histological analysis of the surgical specimen disclosed a nephroblastoma or Wilms' tumor. RESULTS/CONCLUSIONS: The progressive deterioration of the left urinary tract, despite attempts to correct this condition, warranted its complete suppression. The patient has remained symptom-free and leads an active social and working life 31 years after the first operation, which makes this an exceptional case in the world literature.     

Massive sinus of Valsalva aneurysm presenting with coronary insufficiency

A 50-year-old man with a massive acquired sinus of Valsalva aneurysm presenting with coronary insufficiency is presented. Annuloaortic ectasia and severe aortic insufficiency mandated composite aortic valve and root replacement, with reimplantation of the coronary arteries. Clinical characteristics, treatment principles, and surgical outcomes are described.     

Rupture of a sinus of Valsalva aneurysm into the right ventricle. A case report in the cardiology clinic at the University Hospital Center of Dakar]

Human cytolytic T lymphocytes specific for autologous Burkitt's lymphoma express the gamma, delta T cell receptor and recognize immunoglobulin idiotype in an MHC-unrestricted manner. Antibodies against a member of the heat shock protein 70 family inhibit this specific cytotoxicity, implicating these molecules in tumor recognition and antigen presentation. Such data is relevant to the design of novel immunotherapies for cancer and provides new insights into target recognition by gamma, delta T cells.     

Giant intracranial aneurysm associated with Marfan's syndrome. Case report

The authors describe a giant intracranial internal carotid aneurysm in a patient with Marfan's syndrome. Treatment consisted of internal carotid ligation with subsequent thrombosis of the lesion. The patient's course was complicated by carotid arterial dissection and possible laminar thrombosis within the aneurysm increasing the size of the lesion. Three clinical syndromes, Marfan's, Ehlers-Danlos, and pseudoxanthoma esasticum, are discussed. The literature is reviewed with reference to the hazards of treatment of vascular pathology in patients with these connective tissue disorders.     

Fistula of the sinus of Valsalva

Operative repair of sinus of Valsalva aneurysms and fistulas was performed in 10 patients between 1960 and 1976. Associated cardiac defects were corrected in 6 patients. There were no postoperative deaths, and the 8 patients observed to date are still alive and well. Only 2 patients required a second operation. The employment of a transchanber or a transaortic approach or both continues to be a matter of individual preference.     

Morphometric features of ruptured congenital sinus of Valsalva aneurysm: implication for surgical treatment

Five hearts with ruptured congenital sinus of Valsalva aneurysm were studied. In 3 hearts of Caucasian patients, the sinus of Valsalva aneurysms were located in the immediate vicinity of the commissure between the noncoronary and right aortic cusps with rupture from the noncoronary sinus to the right atrium (n = 2) and from the right sinus to the right ventricle (n = 1). In 2 hearts of indigenous North Americans, the defects were sited in the immediate vicinity of the commissure between right and left aortic cusps with rupture into the right ventricle; both patients had an associated conal septal hypoplasia ventricular septal defect and aortic insufficiency. The diameters of the rupture holes at the base of the sinus of Valsalva aneurysms in the five hearts ranged from 0.4 to 1.1 cm (mean 0.7 cm). Histologic examination of longitudinal sections through the ruptured sinus of Valsalva showed 0.8 to 1.7 cm (mean 1.1 cm) wide areas in which there was lack of continuity between the aortic media and the aortic annulus. Conclusions. This study shows that the site of congenital weakness in sinus of Valsalva aneurysm in indigenous North American patients may be similar to that in Oriental patients, whereas the site tends to be different in Occidental patients. It also emphasizes the importance of patch closure rather than suture closure of ruptured sinus of Valsalva aneurysms.     

Paraplegia in a patient with Marfan's syndrome as a result of a thoraco-abdominal aortic aneurysm repair

Marfan's syndrome is a hereditary disorder involving a deficit in connective tissue collagen. Physical findings include musculoskeletal, ocular, and cardiovascular abnormalities. A 29-year-old man with a history of Marfan's syndrome was admitted to the hospital with back and chest pain secondary to a dissecting aortic aneurysm. He later underwent surgical aortic bypass graft surgery. Postoperatively, he was paraplegic. Our impression was anterior spinal artery syndrome due to prolonged cross-clamping of the aorta during surgical repair. This paper shows the risk of paralysis resulting from surgical repair of an aortic aneurysm as a poorly documented complication of Marfan's syndrome.     

Ruptured aneurysm of the sinus of Valsalva into the left ventricle: a case report and review of the literature

This report describes a case of right coronary sinus of Valsalva aneurysm which ruptured into the left ventricle. The diagnosis was made with two-dimensional transthoracic echocardiography which showed an abnormal structure extending from the aortic root into the left ventricle adjacent to the interventricular septum. Subsequent examinations with transesophageal echocardiography and aortic root angiography and surgical findings confirmed the diagnosis of transthoracic echocardiography. The patient underwent aortic valve replacement. At follow-up 12 months later, the patient was without symptoms and repeated echocardiographic examinations showed no recurrence.     

An autopsy case of the sinus of Valsalva aneurysm involved with tuberculous inflammation, leading to complete heart block

A case is presented of unruptured aneurysm of the non coronary sinus of Valsalva, causing involvement of A-V node and right coronary artery compression. The patient was a 68 year-old woman with a complaint of general fatigue. Electrocardiogram showed complete A-V block. Computed tomography showed an aneurysm of the non coronary sinus of Valsalva. A temporary pacemaker was implanted, but the patient developed respiratory failure and heart failure and died. At autopsy, macroscopically disseminated tuberculosis was noted in both lungs and kidneys. Microscopically a tuberculous inflammatory lesion extending into the A-V node was found. We report this rare case with some consideration of the literature.     

Obstetric complications in Marfan's syndrome pregnancy

Marfan syndrome is usually inherited as an autosomal dominant trait with high degree of penetrance. It is caused by an abnormal fibrillin gene located on chromosome 15q. Cardiovascular involvement in Marfan syndrome has been overstressed, although very little attention has been given to obstetric complications. Marfan syndrome may be responsible of cervical incompetence, abnormal placental site and post partum haemorrhagic complications. A 22-year-old woman with Marfan syndrome had mitral regurgitation since childhood. In addition aortic root dilatation was documented over six years by means of echocardiography and had been followed up regularly in a district hospital. Echocardiography six months before pregnancy had shown minimal mitral and aortic regurgitation and aortic root dilatation of 4.1 cm; left ventricular function was normal. Repeat echocardiography evaluations during pregnancy confirmed an aortic root dilatation. Routine booking and screening investigations were all within normal limits. At the 25th week, admission was necessary following a vaginal bleeding, without pain contraction. Echography showed a placenta praevia and cervical dilatation 2.8 cm of diameter. Bed rest and intravenous thocolitic therapy were immediately enhanced. A cervical cerclage, as described by McDonald, was placed. At the 37th week the patient was admitted and cerclage removed before the caesarean section. A healthy female of 2900 g was born. The postoperative period was favourable and patient was discharge after 7 days. In the present case, it is suggested that cervical incompetence and placenta praevia may be caused by an alteration of microfibrillar fibers.     

Aortic peri-valvular abscess, mycotic aneurysm of Valsalva sinus, and fistula to the right ventricle in a context of acute infectious endocarditis

OBJECTIVE: Techniques for in vitro culturing and autotransplantation have been developed for a variety of human cells and are used today in several fields of medicine. In reconstructive surgery within the genitourinary tract, autologous urothelial cells cultured in vitro could be of considerable value but have not yet been used clinically. The aim of this study was to facilitate transplantation of cultured urothelium by establishing a reliable method for culturing urothel on an immunologically inert and biodegradable structure. METHODS: Normal human urothelial cells were cultured in vitro using a feeder-cell system. To achieve an optimal carrier structure, cells were removed enzymatically from a split thickness skin graft. Human urothelial cells were then seeded on the cell-free dermis and incubated in vitro. The seeded dermis samples were investigated histologically and with immunohistochemical methods at days 7, 14 and 21. RESULTS: The human urothelial cells incubated in vitro reached confluence after 7-10 days and the cells could be cultured through 9 passages with preserved proliferative potential. When the cells were seeded on a cell-free dermis they attached, formed colonies and became confluent and stratified up to three cell layers after 21 days of incubation. The urothelial origin of the cells was confirmed by immunohistochemical staining against cytokeratin. CONCLUSION: The advantages of culturing the urothelial cells on a cell-free dermis include a short time lag until grafts are available, probably facilitated transplantation procedure, transplantation of undifferentiated cells and the formation of a vascularised base under the new urothelium. The method described in this study may be of great value in providing autologous urothelium for reconstructive surgery in the genitourinary tract.     

False aneurysm of the ascending aorta after surgery for Marfan's syndrome. Report of a case and review of the literature

A case is described in which false aneurysm of the ascending aorta developed following corrective surgery for Marfan's syndrome. During the initial procedure, a dissecting aortic aneurysm and incompetent aortic valve were managed by replacing the valve with a Starr-Edwards prosthesis and the ascending aorta with a woven Dacron aortic prosthesis. Two years later the operation was repeated with the use of antibiotic cover, because the patient developed a paraprosthetic leak following subacute bacterial endocarditis with Staphylococcus aureus. The woman remained well for 6 months, after which she developed a false aneurysm of the ascending aorta and aortic incompetence which necessitated repeating the operation for a third time. The patient remains well one year later. Relevant literature available on false aneurysm of the ascending aorta is also reviewed.