Mid-aortic Dysplastic Syndrome Research Articles

Visual analysis of abdominal aortitis treatment using the CiteSpace bibliometric method.

Abdominal aortitis can induce aneurysms, and tumor rupture can lead to organ ischemia or even sudden death. At present, there is a lack of extensive understanding and identification of key problems in the treatment of abdominal aortitis, which needs to be further analyzed using bibliometric analysis. To discuss the research hotspot and development trend of abdominal aortitis treatment. We searched the English literature (published from January 1, 2000 to March 12, 2024) on the treatment of abdominal aortitis in the Web of Science database. Then, we identified and screened duplicate literature using CiteSpace 6.1R2 software. We conducted an analysis of the number of papers, a co-occurrence analysis of the authors and institutions, and co-occurrence and cluster analyses of the keywords. Then, we drew the author, institution, and keywords of the studies into graphs for visualization. Finally, we expounded on the author, institutional network interactions, and hot keywords of the studies on the treatment of abdominal aortitis. We included 210 English literature articles involving 190 authors; the author cooperation team was mainly represented by Caradu Caroline, Berard Xavier, Lu Guanyi, Harada Kenichi, and Sharma Ashish K. In the keyword analysis, high-frequency keywords include abdominal aortic aneurysm (38), abdominal aorta (24), Takayasu arteritis (22), etc. The three most central keywords were disease (0.69), classification (0.68), and abdominal aortic aneurysm (0.55). The first nine clusters of keywords are case report, abdominal aortic aneurysm, Takayasu arteritis, dyspnea hematuria, aortic elastic, IgG4-related disease, report, mid aortic dysplastic syndrome, and statin. In the keyword emergent analysis, 14 emergent words were obtained. Among them, seven keywords with strong abruptness were Takayasu arteritis, abdominal aortic aneurysm, disease, retroperitoneal fibrosis, expression, management, and large vessel vasculitis. In the past 3 years, the incidences of abdominal aortic aneurysm (intensity: 4.62) and inflammation (intensity: 1.99) were higher. The number of published papers is on the increase, but the cooperation among authors is scattered. The research focus is mainly on the pathogenesis and treatment of abdominal aortitis-related diseases.

Surgical Treatment of 52 Patients with Midaortic Dysplastic Syndrome

Surgical Treatment of 52 Patients with Midaortic Dysplastic Syndrome

A familial case of Blau syndrome caused by a novel NOD2 genetic mutation.

Blau syndrome (BS) is a rare autosomal dominant, inflammatory syndrome that is characterized by the clinical triad of granulomatous dermatitis, symmetric arthritis, and recurrent uveitis. Mutations in the nucleotide oligomerization domain 2 (NOD2) gene are responsible for causing BS. To date, up to 30 Blau-associated genetic mutations have been identified within this gene. We report a novel NOD2 genetic mutation that causes BS. A girl, aged 8 years, and her brother, aged 10 years, developed erythematous skin rashes and uveitis. The computed tomography angiogram of the younger sister showed features of midaortic dysplastic syndrome. The brother had more prominent joint involvement than the sister. Their father (38 years) was also affected by uveitis; however, only minimal skin involvement was observed in his case. The paternal aunt (39 years) and her daughter (13 years) were previously diagnosed with sarcoidosis. Mutational analysis revealed a novel c.1439 A>G mutation in the NOD2 gene in both siblings. The novel c.1439 A>G mutation in the NOD2 gene was found in a familial case of BS. Although BS is rare, it should always be considered in patients presenting with sarcoidosis-like features at a young age. Early diagnosis of BS and prompt multisystem workup including the eyes and joints can improve the patient's outcome.

Surgical Treatment of Middle Aortic Syndrome with Takayasu Arteritis or Midaortic Dysplastic Syndrome

Middle aortic syndrome (MAS) is a rare condition characterized by severe stenosis of the distal thoracic or abdominal aorta. The aims of this study are to define the anatomic characteristics of MAS and to review the various surgical methods and their outcomes in terms of long-term durability Ten adult patients were diagnosed with MAS caused by Takayasu arteritis (TA) or midaortic dysplastic syndrome and underwent surgical treatment between July 1992 and January 2013. The aortic lesions were mostly suprarenal (n=7) and stenoses were commonly found in the celiac axis (n=6), SMA (n=7), and renal artery (n=6). Indications for operation were uncontrolled hypertension in six patients and lower extremity claudication in four. Eight aortic bypasses, one supraceliac aortic interposition graft, and one bilateral aorto-renal bypass were performed. Adjunctive renal bypass with saphenous vein graft (n=4) and IMA reimplantation (n=2) were performed simultaneously. There was no post-operative mortality, and one complication of iliac dissection at the distal anastomosis site was detected and treated by stenting. Hypertension was cured or improved in five of the six patients, and lower extremity claudication improved in all of them. With a median follow up of 60 months (range, 12-263), all the aortic bypasses were patent and one adjunctive renal artery bypass graft with aortic bypass was occluded 29 months post-operatively. Aortic bypass for MAS is safe and shows excellent long-term durability. Considering the patients are relatively young with a long life expectancy, aggressive surgical treatment could be beneficial. Lifelong follow up to monitor complications and disease progression is necessary.

Mid-aortic dysplastic syndrome as a rare cause of hypertension in young

Introduction: Mid-aortic syndrome (MAS), coarctation of abdominal aorta is a rare disease with only 200 reported cases. It is characterized by constriction of distal thoracic and/or abdominal aorta and its branches, therefore is also known as abdominal aortic coarctation. The MAS is characterized radiologically by severe narrowing of abdominal aorta and its branches and most of these patients usually die due to progressive severe hypertension before the age of 35-40 if left untreated. Case Report: A 13-year-old boy was admitted with persistent headache and vomiting for one month and repeated generalized tonic clonic seizures for two days. His past history was unremarkable. The highlight of the clinical examination blood pressure was 240/150 mmHg in both the upper limbs, and all peripheral pulses were palpable. Blood pressure was similar in upper and lower limbs and a systolic bruit heard over the epigastrium. Ultrasonography showed a localized narrowing of a suprarenal segment of the abdominal aorta with an abrupt focal dilatation of the abdominal aorta approx 1.7cm below origin of superior mesenteric artery. Magnetic resonance angiography of the aorta and its branches showed a distinct fusiform dilatation of abdominal aorta just below the origin of the superior mesenteric artery. Focal narrowing of abdominal aorta was noted just proximal to the dilatation. Conclusion: We diagnosed a case of mid-aortic coarctation with post stenotic dilatation associated with left renal artery stenosis leading to secondary hypertension in a teenage boy presenting with reversible posterior leukoencephalopathy and seizures. The boy was referred for vascular surgery but the patient refused any operative procedure.

Revascularization of midaortic dysplastic syndrome

Revascularization of midaortic dysplastic syndrome

Letters to Editor Hypertension without renovascular stenosis: a rare case of abdominal aorta coarctation treated with percutaneous transluminal angioplasty

Letters to Editor Hypertension without renovascular stenosis: a rare case of abdominal aorta coarctation treated with percutaneous transluminal angioplasty

Abdominal aorta coarctation: The first three case reports in our literature

Congenital coarctation of the thoracic aorta at the ligamentum arteriosum or the aortic arch is well recognized. But a much less common variety (0.5-2.0%) of aortic coarctation is located in the distal thoracic aorta or abdominal aorta or both and is often called "middle aortic syndrome" or "mid-aortic dysplastic syndrome". This represents serious pathological condition and indicates multidisciplinary therapy approach. From 1996 to 2007, at the Vascular Surgery Clinic of the Institute for Cardiovascular Diseases "Dedinje", Belgrade, three patients were treated due to abdominal aorta coarctation, two females aged 55 and 50 and a 4-year-old child. The patients were treated surgically (by-pass with a prosthetic graft and patch angioplasty) and endovascular- percutaneous transluminal angioplasty (PTA) with and without a stent. The follow-up period was 3-70 months. In the 50-year-old patient, angiography showed severe narrowing of the suprarenal segment of the abdominal aorta. Thoraco-abdominal bypass with a 16 mm dacronic tubular graft was performed. In the 4-year-old patient angiography also showed a suprarenal aorta narrowing. In the first act patch angioplasty was performed and after PTA of the visceral arteries was done on several occasions. In the 55-year-old patient, after diagnostic angiography, infrarenal aorta coarctation was registered. PTA was performed with stent placement. All patients were asymptomatic on control check-ups. Abdominal coarctation is a pathological disease which is seldom found in vascular surgery. Angiography is of major importance for setting the diagnosis and for the control of the results of surgical and nonsurgical treatment. The combination of surgical and endovascular treatment in our patients showed very good results in the studied period.

Coarctation of the Abdominal Aorta

A 14-year old male with no previous medical history presented with headache, left third cranial nerve palsy, and generalized tonic-clonic seizures. Magnetic resonance imaging of the brain revealed subarachnoid hemorrhage secondary to an aneurysm of the left posterior communicating artery. The patient underwent successful transcatheter coil occlusion of the aneurysm, and his neurological symptoms improved (Figure 1). Figure 1. A, Cerebral angiogram demonstrating a large ruptured aneurysm (arrow) of the posterior communicating artery. B, Angiogram after endovascular coil embolization showing exclusion of the aneurysm. During admission, he remained hypertensive, with an arterial blood pressure of 170/96 mm Hg in the upper limbs despite medical treatment with atenolol, amlodipine, and enalapril. On physical examination, an abdominal systolic bruit was heard, and weak femoral pulses were noted. Blood pressure in the right and left arms was similar, but there was a significant differential …

Aortic stenosis: spectrum of diseases depicted at multisection CT.

Aortic stenosis, or narrowing of the aortic lumen, has many causes. It may originate in coarctation or pseudocoarctation of the aorta, midaortic dysplastic syndrome, atherosclerosis, Takayasu arteritis, aortic dissection, or various intraaortic and periaortic diseases or as a result of aortic surgical repair. The impedance of blood flow through the stenotic segment may lead to the development of various collateral arterial pathways, according to the location of stenosis. Aortography is the standard technique for evaluating aortic stenosis; however, helical computed tomography (CT), particularly multisection CT, may provide additional information or in some cases may be used instead of arteriography. Multisection CT can depict the aorta and thoracoabdominal collateral pathways in less than 1 minute and provide high-quality arterial-phase imaging data suitable for multiple two-dimensional and three-dimensional reformations. To produce a useful differential diagnosis, the imaging specialist must be able to recognize the type of stenosis and the configuration of collateral circulatory pathways.

The mid-aortic dysplastic syndrome. Surgical considerations with a 2 to 18 year follow-up and selective histopathological study

Mild-aortic dysplastic syndrome is usually presented with advanced hypertension in young individuals in association with either weak or absent femoral pulses, due to diffuse narrowing of the aorta in its mid thoraco-abdominal course. There is frequent involvement of visceral arterial branches such as renal and superior mesenteric arteries. Although the disease has been popularised by the appealing name of "abdominal coarctation", the term mid-aortic dysplastic syndrome is more appropriate. In spite of the fact that the syndrome was described almost three decades ago, its exact aetiology remains obscure and pathogenesis speculative. Surgical revascularisation remains the only therapeutic remedy, in dealing with this particular group of young hypertensive patients. The clinical presentation, angiographic assessment and long-term outcome, following reconstruction in 11 patients (mean age 24.4 years) with mid-aortic dysplastic syndrome were evaluated, in an effort to determine the effectiveness of surgery. Late follow-up, slightly exceeding 16 years (mean 5.6 years) has shown normal and relief from hypertension in practically all individuals. Furthermore, in order to elucidate at least some aspects of histopathology, studies were undertaken upon specimens from the aortic wall and the renal, carotid, lumbar and brachial arteries. The results suggested predominantly dysplastic features in the media, intima and particularly along the course of internal lamina. Aortography, using different projections, revealed variable patterns of high mid-aortic stenosis with or without associated renal artery disease. All patients were hypertensive (mean blood pressure 170 mmHg) and co-exist renal artery disease, unilateral in three and bilateral in three cases, was detected.(ABSTRACT TRUNCATED AT 250 WORDS)