Aortic Arch Syndromes
Conditions resulting from abnormalities in the arteries branching from the ASCENDING AORTA, the curved portion of the aorta. These syndromes are results of occlusion or abnormal blood flow to the head-neck or arm region leading to neurological defects and weakness in an arm. These syndromes are associated with vascular malformations; ATHEROSCLEROSIS; TRAUMA; and blood clots.
Surgical treatment of nonaneurysmal aortic arch lesions in patients with systemic embolization. (1/58)
PURPOSE: Atherosclerotic lesions of the aortic arch are potential sources of arterial embolism. Here we investigate whether surgery, with the necessary circulatory supports, can be proposed as a good option for treatment of this problem. Study of these lesions on a national scale in France has made possible the assessment for future indications of techniques and results of the surgical management of aortic arch lesions, which retrospectively proved to be embolic. METHODS: Thirty-eight patients, (19 men and 19 women) underwent surgery between 1976 and 1996 in 17 French cardiovascular surgical centers. The average age at the time of surgery was 49 +/- 12 years (range, 31 to 82 years). Atherosclerotic lesions were detected with transesophagial echocardiography (n = 19), angiography of the aortic arch (n = 16), computed tomography (n = 9), and magnetic resonance imaging (n = 10). Surgery consisted of thrombectomy and endarterectomy (n = 22), aortic resection and graft replacement (n = 10), and patch aortoplasty (n = 5; one thrombus disappeared spontaneously before surgery was performed). RESULTS: The average postoperative period was 30 months (range, 3 to 82 months). Contact was lost with four patients after a follow-up period of 12 months. On pathologic specimens obtained at surgery, an atherosclerotic plaque was found in 73% of the cases (n = 28). In 15% of the cases, the aorta appeared normal (n = 6) and four other types of lesion were identified: angiosarcoma (n = 1), ectasia at the insertion of the remains of the ductus arteriosus (n = 1), rupture of tunica intima (n = 1), and a fibroblastic plaque (n = 1). A thrombus was identified in 26 cases, attached to the arterial wall in 18 cases. When transesophagial echocardiographic results showed mobile lesions (n = 22), histopathologic examination of specimens allowed the detection of a thrombus in 18 cases and an atherosclerotic plaque with a mobile projection in four cases. The postoperative mortality rate was 2.6%. The morbidity rate (28.9%; n = 11) was related to neurologic complications (n = 6), vascular complications (n = 4), and infection (n = 1). Four cases (12%) were reoperated. CONCLUSION: Nonaneurysmal aortic arch lesions are a frequent and still underestimated source of stroke and peripheral embolization. Surgery with circulatory support can be recommended in good operative candidates with recurrent critical events despite medical management and with high embolic potential (young patients with no calcified plaques). (+info)PARADOXES OF TAKAYASU'S DISEASE. (2/58)
Takayasu's disease (or arteritis) has been defined as an "idiopathic aortitis usually affecting young women." It can come to light from very spectacular and often quite puzzling clinical manifestations. Six cases of Takayasu's disease were investigated at the UCLA Hospital in the years 1961-1962, and signs and symptoms of central nervous system involvement were found in five of the patients. This relatively high incidence of neurological deficit prompted a review of case reports in the literature and this in turn led to a series of "unexpected" findings in the historical evolution of the illness as well as in its anatomopathological aspects. The study indicated that Takayasu's disease is frequently associated with neurological manifestations, at times very severe. In addition, the disease appears to be far more extensive than its classical description suggests. New criteria for the diagnosis of Takayasu's disease must include, among other things, special emphasis on the disseminated nature of the disease. (+info)Common variable immunodeficiency syndrome with right aortic arch: a case report. (3/58)
BACKGROUND: Common variable immunodeficiency syndrome predominantly affects adults. It is characterized by low production of all the major classes of immunoglobulins. We report a case of common variable immunodeficiency syndrome with right aortic arch. An association of right-sided arch and common variable immunodeficiency syndrome has not been previously reported. CASE PRESENTATION: A 41-year-old female patient presented with a history of recurrent pneumonia, sinusitis, otitis media, diarrhoea, cystitis since childhood. Biochemical and immunocytochemical analysis revealed common variable immunodeficiency syndrome and radiological evaluation confirmed right aortic arch and aberrant left subclavian artery. CONCLUSION: Common variable immunodeficiency syndrome syndrome is a clinical entity that should be kept in mind in patients with recurrent infections of different sites. (+info)Subclavian steal in Takayasu's arteritis. A hemodynamic study by means of ultrasonic Doppler flowmetry. (4/58)
Blood flow in the vertebral artery and the upper extremity was studied in five cases of Takayasu's arteritis with subclavian steal by use of ultrasonic Doppler flowmetry and finger plethysmography. The diagnosis of subclavian steal was made by observation of flow reversal in the vertebral artery on the subclavian steal side during grip exercise and, in addition, the vertebral flow change with brachial artery occlusion. The blood flow increase of both internal cartotid and non-affected (non-subclavian steal side) vertebral arteries during a common carotid compression was almost normal in patients with Takayasu's arteritis in this study. During carotid compression on the side of the subclavian steal, ipsilateral vertebral blood flow greatly decreased, and the amplitude the ipsilateral finger plethysmogram decreased slightly or moderately. It is suggested that there are significantly important factors in suppressing sumptoms of vertebrobasilar ischemia in these patients with Takayasu's arteritis with subclavian steal. These factors are believed to be (1) good function of the circle of Willis, (2) good blood supply to the brain stem, and (3) collateral circulation to the distal subclavian artery not via the vertebral artery. (+info)Major vascular anomalies in Turner syndrome: prevalence and magnetic resonance angiographic features. (5/58)
BACKGROUND: Turner syndrome (TS) is associated with aortic coarctation and dissection; hence, echocardiographic evaluation of all patients is currently recommended. X-ray angiography in clinically symptomatic patients has suggested a range of other vascular anomalies, but the true prevalence of such lesions in TS is unknown. To better understand the prevalence and pathogenesis of cardiovascular defects in TS, we prospectively evaluated a group of asymptomatic adult volunteers with TS using magnetic resonance (MR) angiography. METHODS AND RESULTS: A total of 85 adults with TS and 27 normal female adult volunteers underwent gadolinium-enhanced 3D MR angiography. A high prevalence of aortic anomalies was seen in women with TS, including elongation of the transverse arch (49%), aortic coarctation (12%), and aberrant right subclavian artery (8%). Venous anomalies were also prominent, including persistent left superior vena cava (13%) and partial anomalous pulmonary venous return (13%). None of these anomalies were found in healthy female controls. The constellation of elongation of the transverse arch, aortic coarctation, and persistent left superior vena cava was significantly associated with women with TS. Neck webbing and increased thoracic anterior-to-posterior dimension diameters were strong predictors for arterial and venous anomalies. CONCLUSIONS: Thoracic vascular anomalies are common in TS, occurring in approximately 50% of a group not preselected for cardiovascular disease. The highly significant association between neck webbing, increased chest diameter, and these vascular anomalies suggests that in utero, centrally localized lymphatic obstruction may contribute to these cardiovascular deformities in TS. Improved recognition of these often-undetected vascular lesions may be important for identification of patients in need of closer cardiovascular monitoring. (+info)Aortic root replacement with a freestyle stentless valve for aortitis syndrome with ascending aortic aneurysm and aortic regurgitation. (6/58)
A 47-year-old woman who had been diagnosed as having aortitis syndrome underwent aortic root replacement for an ascending aortic aneurysm and aortic regurgitation. Because the patient has been treated with steroids for more than 20 years, a Freestyle stentless valve was used to avoid the risk of valve detachment. There were no complications observed during the postoperative course. Although long-term follow-up will be necessary to observe the valve durability, the Freestyle stentless valve seems to be useful for aortic root replacement in patients at high risk of valve detachment due to aortitis syndrome. (+info)Protruding aortic arch thrombus: treatment with minimally invasive surgical approach. (7/58)
BACKGROUND: Protruding aortic arch thrombus is associated clinically with life-threatening emboli. Definitive treatment for aortic arch thrombus removal has demanded complicated vascular surgical procedures, with high morbidity and mortality. METHODS AND RESULTS: Transesophageal echocardiography (TEE) enabled diagnosis of a protruding thrombus at the aortic arch in 5 patients, and a simultaneous lesion in the descending aorta in 1 patient. Four patients had visceral emboli, coinciding with peripheral emboli in 2 patients, and the fifth patient had peripheral and cerebral emboli. One patient had had ischemic stroke and femoral emboli a few months previously. Mean patient age was 51 years. None had clinical evidence of coronary or peripheral atherosclerotic occlusive disease. Risk factors included hypertension (n = 2), smoking (n = 4), and preexisting thrombophilia (n = 4). Five patients underwent TEE-guided aortic balloon thrombectomy from the arch with a 34-mm occluding balloon catheter. One patient also underwent balloon thrombectomy from the descending aorta with a 14F Foley catheter. Access into the aorta was obtained through the iliac artery (n = 4) during laparotomy because of visceral ischemia or through the transfemoral approach (n = 2). Previous procedures included superior mesenteric embolectomy (n = 3), segmental bowel resection (n = 1), splenectomy (n = 1), and peripheral arterial embolectomy n = 3). Real-time intraoperative TEE enabled visualization of the protruding thrombus and assisted with maneuvering of the balloon catheter. At completion peripheral thrombectomy thrombus material was retrieved in 4 patients. Postoperatively there were no clinically proved new procedure-related visceral emboli, and all patients received anticoagulant therapy thereafter. Follow-up TEE within 2 weeks and up to 7 years revealed no recurrent aortic arch thrombus. CONCLUSIONS: TEE-guided aortic balloon thrombectomy used in 6 procedures was effectively completed without visceral or peripheral ischemic complications. It enabled removal of the life-threatening source of emboli from the proximal aorta, thereby averting the need of major aortic surgery. (+info)Unusual vascular ring anomaly in a foal. (8/58)
A 2.5-month-old filly was presented with signs of esophageal obstruction. The filly was euthanized and postmortem examination revealed a vascular ring anomaly. The vascular ring anomaly was not caused by a persistent right aortic arch, which is the only vascular ring anomaly reported to occur in horses. (+info)Aortic Arch Syndromes are a group of clinical conditions characterized by the narrowing or obstruction of the aortic arch or its major branches, leading to various symptoms such as difficulty in swallowing, arm claudication, and compromised blood flow to the brain and other organs.
Aortic arch syndromes are a group of conditions that affect the aortic arch, which is the curved portion of the aorta that arises from the left ventricle of the heart and gives rise to the major branches of the arterial system. These syndromes are typically caused by congenital abnormalities or degenerative changes in the aorta and can result in various complications, such as obstruction of blood flow, aneurysm formation, and dissection.
There are several types of aortic arch syndromes, including:
1. Coarctation of the Aorta: This is a narrowing of the aorta at the point where it leaves the heart, just distal to the origin of the left subclavian artery. It can cause hypertension in the upper extremities and reduced blood flow to the lower extremities.
2. Aortic Arch Aneurysm: This is a localized dilation or bulging of the aorta in the region of the aortic arch. It can lead to dissection, rupture, or embolism.
3. Aortic Arch Dissection: This is a separation of the layers of the aortic wall, which can result from hypertension, trauma, or genetic disorders such as Marfan syndrome. It can cause severe chest pain, shortness of breath, and shock.
4. Kommerell's Diverticulum: This is an outpouching or bulge in the aorta at the origin of the ligamentum arteriosum, which is a remnant of the ductus arteriosus. It can cause compression of the airways or esophagus and increase the risk of dissection or rupture.
5. Abernethy Malformation: This is a rare congenital anomaly in which there is an abnormal connection between the portal vein and systemic venous circulation, leading to the bypass of the liver. It can cause various complications such as hepatic encephalopathy, pulmonary hypertension, and liver tumors.
The diagnosis and management of aortic arch syndromes require a multidisciplinary approach involving cardiologists, radiologists, surgeons, and other specialists. Treatment options may include medications, endovascular procedures, or surgical interventions depending on the severity and location of the lesion.