Gadolinium enhanced cardiovascular magnetic resonance in Anderson-Fabry disease. Evidence for a disease specific abnormality of the myocardial interstitium.

AIMS: Anderson-Fabry Disease (AFD), an X-linked disorder of sphingolipid metabolism, is a cause of idiopathic left ventricular hypertrophy but the mechanism of hypertrophy is poorly understood. Gadolinium enhanced cardiovascular magnetic resonance can detect focal myocardial fibrosis. We hypothesised that hyperenhancement would be present in AFD. METHODS AND RESULTS: Eighteen males (mean 43+/-14 years) and eight female heterozygotes (mean 48+/-12 years) with AFD underwent cine and late gadolinium cardiovascular magnetic resonance. Nine male (50%) had myocardial hyperenhancement ranging from 3.4% to 20.6% (mean 7.7+/-5.7%) of total myocardium; in males, percentage hyperenhancement related to LV mass index (r=0.78, P=0.0002) but not to ejection fraction or left ventricular volumes. Lesser hyperenhancement was also found in four (50%) heterozygous females (mean 4.6%). In 12 (92%) patients with abnormal gadolinium uptake, hyperenhancement occurred in the basal infero-lateral wall where, unlike myocardial infarction, it was not sub-endocardial. In two male patients with severe LVH (left ventricular hypertrophy) and systolic impairment there was additional hyperenhancement in other myocardial segments. CONCLUSIONS: These observations suggests that myocardial fibrosis occurs in AFD and may contribute to the hypertrophy and the natural history of the disease.     

Increased pregnancy loss in young women with aortoiliac disease

BACKGROUND: During clinical evaluation of young women with peripheral arterial occlusive disease, we were surprised by the high prevalence of pregnancy loss in women with segmental stenosis confined to the aortoiliac segment. We wondered if increased occurrence of miscarriage is the result of high expression of vascular and obstetrical risk factors in these patients, or if it is related to localization of disease. In a case-control study designed to investigate risk factors for peripheral arterial occlusive disease in young women, we assessed the risk of miscarriage in these patients according to level of obstruction. METHODS: A total of 202 female patients, aged 18-49 years and 466 healthy control women from a population based case-control study, donated venous blood samples and filled out a structured questionnaire concerning classical cardiovascular risk factors and obstetrical history. In all patients, diagnosis of peripheral arterial occlusive disease was confirmed by intra-arterial angiography. Patients were classified into two groups: those with and those without stenosis of the aortoiliac segment (aortoiliac disease). RESULTS: In 77 of the 202 patients (38%) with peripheral arterial occlusive disease, the obstruction was confined to the aortoiliac segment. The occurrence of miscarriage was high (42%) in young women with aortoiliac disease. Compared to healthy controls, the risk of miscarriage increased 3-fold (OR 3.1; 95% CI 1.8-5.6) in these patients. Adjustment for obstetrical and vascular risk factors did not affect the risk estimate. CONCLUSION: This is the first study that identifies aortoiliac disease as a risk factor for pregnancy loss in young women. The risk of miscarriage is increased 3-fold in women with aortoiliac disease. The presence of vascular and obstetrical risk factors did not affect the strength of the association. Pregnancy loss could be the first sign of insufficient aortic circulation in these patients.     

Percutaneous intraaortic balloon insertion in patients with aortoiliac disease

A new technique for percutaneous insertion of intraaortic balloon catheters in patients with tortuous or narrowed external iliac arteries is described. This method uses a long (45 cm) 12F sheath, which extends up to the descending aorta and protects the iliac artery and aorta from injury during insertion of the balloon catheter. Insertion of intraaortic balloon catheters was not possible by conventional percutaneous technique in six patients with severe acute myocardial ischemia. Use of the long sheath approach permitted rapid and successful institution of circulatory support in these patients.     

主髂动脉硬化闭塞症的腔内治疗

目的:探讨主髂动脉硬化闭塞症的腔内治疗方法和初步临床经验。方法:2002年1月至2010年6月主髂动脉硬化闭塞症腔内治疗患者161例,其中4例行单纯髂动脉球囊扩张,157例患者行髂动脉球囊扩张及支架植入术,共植入支架207枚,其中累及腹主动脉末端的10例患者采用对吻技术行支架植入。结果:161例患者均成功完成手术。术后患者踝臂指数(ABI)由术前的0.46±0.28提高至0.89±0.26,二者差异有统计学意义(P0.01)。间歇性跛行及静息痛症状均较术前有明显改善。围手术期并发症包括急性血栓形成2例,穿刺部位血肿4例,肺部感染2例,脑梗死2例,急性心肌梗死1例,消化道出血2例。其中1例患者术后出现应激性溃疡消化道出血,后并发多脏器功能衰竭死亡,围手术期死亡率为0.62%,随访12个月的一期通畅率为91.8%,二期通畅率为100%。结论:主髂动脉病变的腔内治疗有良好的效果,部分复杂病变使用杂交技术可以提高成功率及通畅率。     

Gadolinium accumulation after contrast-enhanced magnetic resonance imaging: Which implications in patients with Crohn’s disease?

Crohn’s disease (CD) is a chronic inflammatory condition of the bowel, characterized by an alternation of remission and relapse phases, leading to a progressive intestinal damage with loss of function. Magnetic resonance enterography has been widely used in the past for the evaluation of fistulizing disease, but its use increased over time, being considered helpful in different moments of disease course. Intravenous injection of Gadolinium-based contrast agents has been demonstrated to be crucial to assess mucosal inflammation, transmural involvement, and extraintestinal disease. Recently, Gadolinium accumulation in human tissues has been increasingly reported, although clinical implications of this event are still unclear. In the present paper, we review the main evidence on the topic, focusing on the potential implications for gastroenterological practice.     

Minilaparotomy for aortoiliac occlusive disease: preliminary results

This prospective study was designed to establish the feasibility of minilaparotomy for aortobifemoral bypass, and its effect on intraoperative and postoperative variables. A minilaparotomy has potential benefits for the patient, including smaller size of the surgical wound, reduced risk of infection, shorter postoperative intubation, decreased postoperative pain, earlier discharge, and a smaller, aesthetically more acceptable postoperative scar. Moreover, reoperation is less hazardous, because the peritoneum is not completely dissected. From the beginning of June 1999 through the end of September 2001, we used a minilaparotomy in 33 patients with aortoiliac occlusive disease. Obesity and prior abdominal surgery were regarded as contraindications to the operation. One patient required conversion to a full laparotomy because of intraoperative bleeding. Another patient developed wound infection. There were no deaths. The technique has proved safe, effective, and aesthetically acceptable to the patient. To date, no study has compared a median laparotomy with a minimally invasive alternative for the surgical treatment of aortoiliac occlusive disease. Prospective randomized trials are needed to determine whether minilaparotomy is the superior technique for treatment of aorto-occlusive disease.     

Angiographic concurrence of coronary artery disease and aortoiliac lesions

The importance of recognizing the association between aortoiliac disease and coronary artery disease includes the following: (1) Long-term morbidity is higher and survival ratio poorer in patients with coronary heart disease compared with isolated lower extremity revascularization surgery. (2) Coronary artery bypass grafting is a relatively high-risk procedure in patients with severe vascular disease. (3) There is the prospect that the patient will eventually face simultaneous coronary artery and vascular surgery, or coronary artery angioplasty previous to aortoiliac surgery. The aim of this investigation is to know the frequency of the association of coronary artery disease with aortoiliac lesions and to stratify the risk factors related to such an association. In total, 65 men and 19 women (30 to 76 years of age) with a history of coronary heart disease underwent abdominal aortography after selective coronary artery and left ventricle angiography. Aortoiliac lesions were identified at angiograms. Relevant coronary artery disease was diagnosed when at least 1 coronary artery was obstructed > 50%. The frequency of association between aortoiliac and coronary artery lesions was established, as well as the relationship of these lesions to the following clinical variables: age, weight, height, smoking habit, history of coronary heart disease, systemic arterial hypertension, diabetes mellitus, intermittent claudication, glycemia, uricemia, and triglyceridemia. There were 36 patients (42.9%) with aortoiliac lesions. In 34 patients (40.5%) coronary artery disease was associated with aortoiliac lesions. Abdominal aortic dilations were found in 10 instances, abdominal aortic stenosis in 13 patients, and stenosis of the iliac arteries or their branches on 28 occasions. The variables statistically related to the presence of aortoiliac lesions were smoking habit and a history of intermittent claudication. The number of affected coronary arteries was directly related to the frequency of aortoiliac lesions. In the entire sample, 11 patients (13%) had no coronary artery disease, and 15 (17.9%) had 1-vessel, 24 (28.6%) 2-vessel, and 34 (40.5%) 3-vessel disease. The extent of coronary disease was directly related to the frequency and extent of aortoiliac lesions. Frequencies of aortoiliac lesions were strongly related to a history of smoking habit and intermittent claudication and directly related to the extent of coronary artery disease.     

Endovascular treatment for obstructive disease of the aortoiliac bifurcation by the kissing stent technique

PURPOSE: To evaluate short and medium-term results of endovascular repair of the aortoiliac bifurcation for occlusive disease by the kissing stent technique. METHOD: Twenty patients were included in this retrospective study between May 1999 and November 2002 (13 men, mean age 58 years, range 42-86). Fifteen were treated for bilateral common iliac artery stenosis (75%), four for unilateral common iliac artery occlusion with or without controlateral stenosis (20%), and one for bilateral common iliac artery occlusion (5%). Each patient had a clinical examination and dupplex-scan prior to discharge, at three months, twelve months then yearly. RESULTS: All procedures were successful. Forty balloon expandable stents were deployed in the kissing position (mean length and diameter, 45 mm and 9 mm). Nine other stents were also deployed either in the abdominal aorta (one patient) either in the lower portion of the common iliac artery. Intra-operative complications were represented by one hematoma of the groin region treated surgically, one emboli of the femoral bifurcation treated by Forgarty balloon embolectomy, three dissections of the common iliac arteries treated by stenting, and one internal iliac artery occlusion not treated. Mean follow-up was 21 months (range 3-36). Three significant restenoses (>50%) were diagnosed during this period and one patient had an additional transluminal procedure. No intra-stent occlusion was found. Primary and secondary cumulative patencies at 12 and 36 months were respectively 94.7%, 84,4% and 100%, 89%. CONCLUSION: We confirm the feasibility, and the reliability of endovascular aorto-iliac kissing stent reconstruction in occlusive disease for selected patients.     

Gadolinium delayed enhancement cardiovascular magnetic resonance correlates with clinical measures of myocardial infarction

OBJECTIVES: The current study tested the hypothesis that gadolinium delayed enhancement assessment of infarct size correlates with clinical indices of myocardial infarction (MI) in humans. Acute infarct mass by cardiac magnetic resonance (CMR) was compared with peak troponin I, acute and chronic left ventricular (LV) systolic function, and chronic infarct mass in patients imaged after recent acute MI. BACKGROUND: Cardiac magnetic resonance accurately determines myocardial viability in patients with chronic ischemic heart disease but is not well validated for recent MI. METHODS: Patients with first acute MI (n = 33) or chronic MI (n = 10) underwent cine CMR followed by gadolinium delayed enhancement imaging. A follow-up CMR scan was performed on 20 of the 33 acute MI patients and all of the chronic MI patients. RESULTS: In patients with acute percutaneous coronary intervention, acute MI mass correlated with peak troponin I (r = 0.83, p < 0.001, n = 23). In the 20 acute infarct patients with follow-up CMR scans, the acute infarct size correlated well with the follow-up LV ejection fraction (r = 0.86, p < 0.001). The transmural extent of delayed enhancement imaged acutely correlated inversely with wall thickening measured acutely (p < 0.001) and at follow-up (p < 0.001). Although chronic infarct size was reproducible (11 +/- 4% vs. 12 +/- 7%, p = NS), acute infarct size decreased from 16 +/- 12% to 11 +/- 9% (p < 0.003). CONCLUSION: In humans imaged shortly after acute MI, gadolinium delayed enhancement acute CMR infarct size correlates with acute and chronic indices of infarct size but will appear to diminish in size on follow-up.     

Present role of extraanatomic bypass graft procedures for aortoiliac occlusive disease.

In a retrospective follow-up the long-term results and present role of axillofemoral and transverse femorofemoral bypass grafts are evaluated. During the period from 1970 until 1989 173 extraanatomic bypass operations were carried out on 150 high risk patients. There were 131 axillofemoral and 42 femorofemoral bypass grafts. In elective operations the mortality ranged from 5.3% for the axillofemoral bypass and 2.4% for the femorofemoral bypass. A five year postoperative follow-up showed a cumulative patency rate of 86.82% for the femorofemoral bypass, 73.74% for the axillo-bifemoral bypass and 43.18% for the unilateral modification. After ten years the results of femorofemoral bypass remained unchanged. In contrast nearly every second axillofemoral bypass showed one to five graft occlusions. Considering the low operative mortality, the short operating time, the late results and the high late mortality independent of the surgical procedure on the one hand femorofemoral bypass has presented more and more as surgical method of choice in cases of unilateral iliac artery occlusion and unilateral branch occlusion of aortoiliac bifurcation grafts as well. On the other hand in cases of bilateral occlusive disease in high risk patients axillofemoral bypass has been displaced more and more by anatomical surgical procedures as endarterectomy or unilateral bypass using retroperitoneal approach. Since 1986 axillofemoral bypass has no longer been performed in elective cases. Nowadays an indication does exist only for deep wound infection in retroperitoneal or inguinal space.     

The proximal form of mural thrombus in aortoiliac occlusive disease using computed tomography.

BACKGROUND: It is important to know the nature of proximal thrombus in patients with aortoiliac occlusive disease (AIOD) when juxtarenal aortic clamping is scheduled. This study was aimed to evaluate the shape and nature of thrombi at several sites of the abdominal aorta in patients with AIOD using enhanced computed tomography (CT). Final judgment was made according to the operative findings. METHODS: Between the years 1999 and 2001, 22 patients, who underwent aortobifemoral bypass, were enrolled. The shape and nature of their thrombi were examined at 4 points (superior mesenteric, suprarenal, juxtarenal and infrarenal arteries at the level of the 2 cm before the renal artery) and 88 slices of CT were examined retrospectively. RESULTS: There was mural thrombus in 31 slices, which could be classified into 4 shapes (crescent-shaped: 10 cases; magatama: 2; wavy: 12; circular: 6). The wavy and circular shaped thrombi were found to be atheromatous. Nine cases (40.9%) on operative findings were atheroma (wavy: 4; circular shaped: 5). The crescent shape might correspond to fibrin thrombus. Atheromatous thrombus clamping near the renal artery was thought to cause microthromboembolism to surrounding organs. CONCLUSIONS: It is recommended that the more proximal aorta or splanchnic arteries should be temporarily clamped during proximal procedures in patients with wavy or circular shaped thrombi at the juxtarenal aorta to prevent kidney or bowel infarction.     

Obesity and fat distribution as predictors of aortoiliac peripheral arterial disease in middle-aged men

Background

Peripheral arterial disease (PAD) in the aortoiliac segment is found in nearly a half of patients with PAD. The aim of this study was to estimate the influence of obesity and fat distribution on the occurrence of aortoiliac PAD in middle-aged men.

Methods

This case-control study included 204 middle-aged men (mean 58 ± 7 years; range 45-70 years): 102 patients with aortoiliac PAD and 102 healthy controls without PAD. Aortoiliac PAD was diagnosed by ankle-brachial index (ABI) and angiography. Body mass index (BMI) was categorized as: normal weight, overweight, and obese. Percents of body fat were grouped according to quartile distribution. The relationship between anthropometrics and aortoiliac PAD was estimated by multivariate logistic regression.

Results

Patients with aortoiliac PAD had higher body mass index, waist circumference, waist-hip ratio and percent of body fat. A strong correlation between all anthropometric parameters and ABI index and mean angiographic score was shown among patients with PAD. Multivariate regression, adjusted for blood pressure and cholesterol level, identified being overweight, body fat over 26.5% and WHR over 1.02 as predictors for aortoiliac PAD. Body fat over 26.5% and WHR over 1.02 remained significant after further adjustment for blood pressure, cholesterol and body mass index.

Conclusion

This study has identified the quantity of fat tissue (body fat over 26.5%) and its visceral distribution (waist-hip ratio over 1.02) as predictors of aortoiliac PAD in middle-aged men. Body mass index, a crude indicator of obesity, should be combined with these parameters when assessing the risk for aortoiliac PAD.     

The natural history of incidental renal artery stenosis in patients with aortoiliac vascular disease

PURPOSE: To examine the association between incidentally discovered renal artery stenosis and deterioration of renal function as determined by the change in serum creatinine concentration over time. SUBJECTS AND METHODS: We performed a retrospective review of consecutive patients who underwent aortography for aortoiliac vascular disease. Angiograms were reviewed for renal artery stenosis, defined as a narrowing of at least 20% compared with adjacent normal renal artery. For patients with at least 180 days of subsequent follow-up, the change in serum creatinine concentration per year was compared in patients who had or did not have renal artery stenosis. RESULTS: Of the 201 patients, 96 (48%) had some degree of renal artery stenosis in one or both renal arteries, including 53 (26%) who had at least one stenosis > or= 50% and 40 (20%) who had bilateral stenoses. The only clinical predictor of renal artery stenosis was a history of coronary artery disease (odds ratio = 2.0, 95% confidence interval: 1.2 to 3.8, P = 0.001). Among the 174 patients with > or =180 days of follow-up, there was no statistically significant difference (P = 0.88) in the mean change in serum creatinine concentration per year in the 78 patients with renal artery stenosis (0.06+/-0.33 mg/dL per year) as compared with the 96 patients without renal artery stenosis (0.06+/-0.22 mg/dL per year). Grouping the patients by the maximal percentage of stenosis did not reveal any difference in the mean changes in serum creatinine concentration per year. CONCLUSIONS: Although renal artery stenosis is a common incidental finding in patients with atherosclerotic vascular disease, it is an uncommon cause of progressive renal disease.     

深部脑磁刺激技术治疗阿尔茨海默病的临床研究

目的 探讨深部脑磁刺激技术(dTMS)治疗阿尔茨海默病患者(AD)的有效性及安全性. 方法 AD患者116例和年龄相匹配的健康对照组33例,AD患者随机分为(1)dTMS组dTMS真刺激治疗；(2)药物组给予盐酸多奈哌齐5 mg/d治疗；(3)联合治疗组同时给予dTMS和盐酸多奈哌齐药物治疗；(4)空白对照组伪刺激治疗.健康对照组dTMS真刺激治疗.疗程6个月.应用简明精神状态检查量表(MMSE)、蒙特利尔认知评估量表(MoCA)、汉密尔顿抑郁量表(HAMD)、缺血量表(HIS)、听觉词语测试、词语流畅性测试、数字广度测试、画钟测验(CDT)、Boston命名测试、韦氏积木图、日常行为能力量表(ADL)及神经精神问卷(NPI)评价dTMS治疗的疗效和安全性.结果 dTMS组、药物组和联合治疗组治疗6个月后MMSE、MoCA、ADL、NPI及认知亚项评分均较空白对照组改善(t值MMSE分别为2.49、2.46、2.20,t值MoCA分别为2.59、2.39、2.87,t值ADL分别为2.35、2.17、2.83,t值NPI分别为3.05、2.40、2.65,均P＜0.05),联合治疗组各量表评分较药物组和dTMS组认知功能和日常行为能力改善(均为P＜0.05),但dTMS组、药物组及联合治疗组治疗后各量表评分仍较健康对照组差(P＜0.05). 结论 dTMS可以有效安全地治疗AD患者的认知及非认知症状,为AD提供非药物治疗手段.