1/118. Clinical, pathological and molecular genetic findings in a case of neonatal marfan syndrome.An infant with neonatal marfan syndrome is described who presented with arachnodactyly, distinctive dysmorphic features and prolapse of both atrioventricular valves and dilatation of both the aortic and pulmonary root. She died in cardiac failure shortly after pacemaker implantation, due to dysrhythmia and severe mitral insufficiency. At autopsy, apart from myxomatous changes of the valves and dilated aortic and pulmonary roots, an aneurysm of the sinus of valsalva of the pulmonary valve and abnormal myxomatous connective tissue surrounding the AV node were also found. Molecular genetic studies showed a point mutation in the fibrillin 1 gene that creates a new N-glycosylation site, which has been described once before.- - - - - - - - - - ranking = 1keywords = aneurysm (Clic here for more details about this article) |
2/118. Mitral valve replacement through dilated aortic annulus in Marfan's syndrome.A 30-year-old man presented with severe aortic regurgitation due to an ascending aortic aneurysm and mitral valve insufficiency in conjunction with active endocarditis. He underwent combined aortic root replacement by a composite graft and mechanical mitral valve replacement. After excision of the aortic valve cusps the mitral valve was approached through the extremely dilated aortic annulus. This technique is seldom used. Mitral valve replacement through the aortic root can be advantageous in selected patients to minimize trauma and reduce myocardial ischemic time.- - - - - - - - - - ranking = 1keywords = aneurysm (Clic here for more details about this article) |
3/118. Separate revascularization of the visceral arteries in thoracoabdominal aneurysm repair.We successfully repaired a ruptured aortic patch containing the visceral arteries after thoracoabdominal aneurysm repair in a young patient with marfan syndrome. This unexpected and tricky situation was treated by separate revascularization of each visceral artery using small-caliber prosthetic grafts as interposition between the aortic prosthesis and the ostia of the visceral arteries.- - - - - - - - - - ranking = 5keywords = aneurysm (Clic here for more details about this article) |
4/118. Surgical repair of type B aortic dissection complicated by early postoperative lung vein and artery thrombosis.A 24-year old man with marfan syndrome previously operated for abdominal aortic aneurysm and type A dissection sustained a type B dissection. He underwent graft replacement of the descending and upper abdominal aorta, complicated by infarction of the left upper lobe and lobectomy was carried out. The postoperative course was uneventful. The mechanism for this rare complication is discussed.- - - - - - - - - - ranking = 1keywords = aneurysm (Clic here for more details about this article) |
5/118. Combined heart transplantation and resection of dissecting aneurysm of ascending aorta and aortic arch: a case report.A 21-year-old male patient had suffered from palpitation and exertional dyspnea since October, 1997. He was admitted to our hospital, and a series of examinations were performed. Chest computed tomography (CT) revealed marked dilatation of the ascending aorta (about 7.5 cm at the proximal portion) and aortic annulus, an intimal flap in the ascending aorta and aortic arch was also noted. cardiac catheterization revealed the pulmonary capillary wedge pressure was 33 mmHg, pulmonary artery pressure was 47/38 mmHg with a mean of 35.4. The cardiac index was 1.01 l/min/m2. Poor left ventricular contractility was shown by a left ventricular ejection fraction (LVEF) of 13.8% and a right ventricular ejection fraction (RVEF) of 5.13% by a radionuclide angiogram (rna) study. Under the diagnosis of dilated cardiomyopathy and dissecting aortic aneurysm of the ascending aorta and aortic arch, he was put on a waiting list for heart transplantation. On November 11, 1997 he received heart transplantation. Resection of the dissecting aneurysm of the ascending aorta and the aortic arch and replacement with a 26 mm Vascutek graft were performed first under deep hypothermia and retrograde cerebral perfusion. Then while he was rewarming up, heart implantation was performed. He was discharged 30 days after surgery and has been doing well since then. As far as we know, no literature regarding combined heart transplantation and resection of a dissecting aneurysm of the ascending aorta and aortic arch has been reported.- - - - - - - - - - ranking = 7keywords = aneurysm (Clic here for more details about this article) |
6/118. Takayasu's arteritis concurrent with marfan syndrome--a case report.marfan syndrome (MS) is a dominantly inherited connective tissue disorder characterized by arachnodactyly, tall stature, the presence of aortic aneurysm, and lens dislocation. Takayasu's arteritis (TA) is a chronic vasculitis that primarily affects the aorta and its branches. The authors report the first case of TA in a patient with MS. The simultaneous presence of TA and MS could be a coincidence, however; the pathogenesis of TA might be linked with autoimmunity induced by abnormal extracellular matrix protein derived from the genetic mutations in MS.- - - - - - - - - - ranking = 1keywords = aneurysm (Clic here for more details about this article) |
7/118. Giant aneurysm following coil occlusion of patent ductus arteriosus.A case is described in which a giant aneurysm developed following successful PDA coil occlusion in an infant with marfan syndrome. This rare and severe complication brings into question the need for careful evaluation of these children before and after transcatheter occlusion.- - - - - - - - - - ranking = 5keywords = aneurysm (Clic here for more details about this article) |
8/118. Coronary ostial aneurysms after composite graft replacement.Coronary ostial aneurysms after composite graft replacement of the ascending aorta and aortic valve is a rare complication. We report two patients with marfan syndrome who developed coronary ostial aneurysms at the sites of the coronary anastomosis, presumably because of oversized windows made in the graft. They were successfully treated by redo composite graft replacement. To prevent this complication, it is important to consider that the hole made in the tube graft should not be larger than the diameter of the respective coronary ostium to avoid exposure of the diseased aortic wall to the circulating blood as much as possible, and that the suture used to anastomose the coronary buttons should pass through the rim of the ostium rather than through the aortic wall surrounding it.- - - - - - - - - - ranking = 6keywords = aneurysm (Clic here for more details about this article) |
9/118. Aneurysm of a jejunal branch of the superior mesenteric artery in a patient with Marfan's syndrome.A case of post-traumatic aneurysm of a jejunal branch of the superior mesenteric artery in a patient with Marfan's syndrome is reported. Ascending aortic involvement is well known in Marfan's syndrome but no association with visceral artery aneurysms has been previously described. The blunt abdominal trauma preceding the detection of the aneurysm may have been the precipitating cause in a predisposed patient. Because of the high risk of rupture, aneurysms of the superior mesenteric artery branches should be treated. Excision or ligation without restoring continuity are the most common surgical procedures; endovascular embolization is an alternative option especially in high risk patients.- - - - - - - - - - ranking = 4keywords = aneurysm (Clic here for more details about this article) |
10/118. Clinical considerations in the chiropractic management of the patient with marfan syndrome.OBJECTIVE: To describe the chiropractic management of a patient with whiplash-associated disorder and a covert, concomitant dissecting aneurysm of the thoracic aorta caused by marfan syndrome or a related variant. CLINICAL FEATURES: A 25-year-old man was referred by his family physician for chiropractic assessment and treatment of neck injuries received in a motor vehicle accident. After history, physical examination, and plain film radiographic investigation, a diagnosis of whiplash-associated disorder grade I was generated. INTERVENTION AND OUTCOME: The whiplash-associated disorder grade I was treated conservatively. Therapeutic management involved soft-tissue therapy to the suspensory and paraspinal musculature of the upper back and neck. Rotary, manual-style manipulative therapy of the cervical and compressive manipulative therapy of the thoracic spinal column were implemented to maintain range of motion and decrease pain. The patient achieved full recovery within a 3-week treatment period and was discharged from care. One week after discharge, he underwent a routine evaluation by his family physician, where an aortic murmur was identified. Diagnostic ultrasound revealed a dissecting aneurysm measuring 78 mm at the aortic root. Immediate surgical correction was initiated with a polyethylene terephthalate fiber graft. The pathologic report indicated that aortic features were consistent with an old (healed) aortic dissection. There was no evidence of acute dissection. Six month follow-up revealed that surgical repair was successful in arresting further aortic dissection. CONCLUSION: The patient had an old aortic dissection that pre-dated the chiropractic treatment (which included manipulative therapy) for the whiplash-associated disorder. Manipulative therapy, long considered an absolute contraindication for abdominal and aortic aneurysms, did not provoke the progression of the aortic dissection or other negative sequelae. The cause, histology, clinical features, and management considerations in the treatment of this patient's condition(s) are discussed.- - - - - - - - - - ranking = 3keywords = aneurysm (Clic here for more details about this article) |
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