全脑血管造影60例的护理体会

目的 探讨数字减影全脑血管造影(digital substraction angiography,DSA)的护理方法及注意事项.方法 对60例行全脑血管造影的神经疾病患者进行术前、术中及术后观察和护理.结果 DSA诊断颈动脉狭窄27例,颅内动脉狭窄13例,颈部或颅内血管闭塞8例,颅内动脉瘤7例,颅内占位3例,动静脉畸形2例.并发症:穿刺部位皮下血肿2例,抽搐2例,排尿困难3例,并发症发生率为11.67%,以上并发症给予相应的处理后,症状均缓解,未遗留后遗症,手术成功率为100%.结论 脑血管造影术是一种有创检查,术前准备妥善是造影术顺利进行的关键,术后准确及时地观察病情并完善各项护理是减少并发症的有效保证.     

120例颅内外脑供血动脉严重狭窄或闭塞患者全脑血管造影分析

目的 探讨颅内外脑供血动脉狭窄或闭塞患者的全脑血管造影特点.方法 对经全脑血管造影证实的120例颅内外脑供血动脉严重狭窄或闭塞病例的血管造影资料进行分析.根据年龄将患者分为青年组(6～44岁,48例)、中年组(45～59岁,41例)和老年组(≥60岁,31例).结果 全组病例共有狭窄或闭塞324处,单纯颅内动脉狭窄占47%,显著高于单纯颅外动脉狭窄占35%和颅内外狭窄并存占18%.青年组单纯颅内动脉狭窄比例为79%,显著高于中年组(29%,P<0.01)和老年组(19%,P<0.01);青年组单纯颅外血管狭窄比例为10%,显著低于中年组(46%,P<0.01)和老年组(58%,P<0.01);青年组前循环狭窄比例为97%,显著高于中年组(75%,P<0.01)和老年组(56%,P<0.01).脑梗死患者单纯颅内动脉狭窄占35%,单纯颅外动脉狭窄占42%,颅内外动脉狭窄并存占23%;短暂性脑缺血发作(transient ischemic attack,TIA)患者单纯颅内动脉狭窄占44%,单纯颅外动脉狭窄占39%,颅内外动脉狭窄并存占17%.82%的脑(室)出血发生于单纯颅内动脉狭窄患者.共107例患者存在梗死灶,其中99例梗死灶与动脉狭窄部位存在相关性.部分患者存在高血压、吸烟、高脂血症、高龄和糖尿病等危险因素,与狭窄分布模式有一定关系.结论 脑供血动脉狭窄的最多发部位是颈内动脉.单纯颅内动脉狭窄以青年人多见,随年龄的增长颅外动脉狭窄比例逐渐增高,颅内外动脉狭窄并存情况逐渐增多,前循环狭窄比例逐渐降低,而后循环狭窄比例逐渐升高.脑梗死、TIA和脑(室)出血患者动脉狭窄好发部位各不相同.缺血性脑血管病患者梗死灶与动脉狭窄部位密切相关.高血压对患者形成颅内外动脉狭窄影响最大,高龄和糖尿病对颅外动脉狭窄影响较大.     

缺血性脑卒中患者颅内外脑动脉狭窄的分布及其危险因素分析

目的探讨缺血性脑卒中患者颅内外脑动脉粥样硬化性狭窄的分布特征及其危险因素。方法选择存在颅内外脑动脉粥样硬化性病变的缺血性脑卒中患者155例,按年龄分为中青年组(年龄33~59岁,51例)和老年组(年龄≥60岁,104例)。通过全脑血管造影检查,对颅内外脑动脉狭窄的分布特征及危险因素进行分析。结果155例患者中共有狭窄或闭塞472处,颅内外病变分别以大脑中动脉狭窄及颈内动脉颅外段最易发生。单发脑动脉病变80例(51.6%),其中单纯颅内动脉病变44例(55.0%),单纯颅外动脉病变36例(45.0%);颅内外动脉并存病变75例(48.4%),其中颅内外动脉串联病变27例(36.0%)。单纯前循环狭窄58例(37.4%),单纯后循环狭窄19例(12.3%),前后循环并存狭窄78例(50.3%)。与中青年组比较,老年组单纯颅内动脉狭窄和前循环狭窄明显降低(22.1%vs 41.2%,31.7%vs 49.0%,P<0.05)。部分患者存在高血压、高脂血症等危险因素与颅内外动脉狭窄分布模式存在相关性。结论颅内外脑动脉狭窄的分布有年龄特征性,随着年龄增长,逐渐以颅内外动脉及前后循环并存狭窄为主。高血压和高脂血症与颅内外动脉并存狭窄有关。     

缺血性脑血管病患者脑动脉狭窄与ESSEN卒中风险评分的关系

目的探讨缺血性脑血管病患者脑动脉狭窄与Essen卒中风险评分(ESRS)的相关性。方法连续纳入482例行脑血管造影的症状性脑动脉狭窄患者,根据患者的临床资料对其进行ESRS评分,并记录脑动脉狭窄的部位(前循环、后循环及颅内动脉、颅外动脉)。按ESRS评分将患者分为卒中复发低危组(0~2分)及高危组(≥3分)。分析ESRS评分与脑血管狭窄程度及部位的相关性。结果 482例中,低危组198例,高危组284例。(1)高危组的年龄及高血压、糖尿病、心肌梗死病史、其他心脑疾病史、外周动脉疾病的比率高于低危组,差异有统计学意义(P0.05或P0.01);而性别、吸烟差异无统计学意义。(2)血管轻度狭窄64例,中度狭窄111例,重度狭窄或闭塞307例。Pearson直线相关分析显示,ESRS评分与血管狭窄程度呈正相关(r=0.095,P=0.037)。(3)482例中,前、后循环均有狭窄者占52.1%(251例),前循环狭窄者占29.0%(140例),后循环狭窄者占18.9%(91例)。高危组前、后循环均有狭窄者占61.6%(175/284),高于低危组的38.4%(76/198);而低危组的前循环狭窄患者较多,占40.4%(80/198),差异有统计学意义(P0.01)。(4)482例中,颅外动脉狭窄者占45.9%(91例),颅内-外动脉均有狭窄者占39.4%(190例),颅内动脉狭窄者占14.7%(71例)。高危组颅外动脉狭窄及颅内-外动脉均有狭窄者共占90.5%(257/284),高于低危组的77.8%(154/198),差异有统计学意义(P0.01)。结论缺血性脑血管病患者的脑动脉狭窄程度与ESRS评分相关,ESRS不同时脑动脉病变的部位分布有区别。     

缺血性脑血管病患者脑动脉狭窄的分布

目的探讨不同年龄缺血性脑血管病患者脑动脉狭窄的分布规律。方法缺血性脑血管病患者340例行全脑血管造影,其中短暂性脑缺血发作(TIA)患者105例(TIA组),脑梗死患者235例(脑梗死组);又根据年龄分为青年组(54例)、中年组(137例)、老年组(149例)。结果脑梗死组吸烟、饮酒明显高于TIA组(P<0.05);老年组颈动脉狭窄、颅外动脉狭窄高于中年组和青年组,中年组高于青年组(P<0.05);青年组颅内动脉狭窄高于颅外动脉狭窄,老年组颅外动脉狭窄高于颅内动脉狭窄(P<0.05);缺血性脑血管病和脑梗死患者前循环动脉狭窄高于后循环(P<0.01);老年组前后循环及后循环动脉狭窄明显高于中年组和青年组(P<0.01);中年组前后循环及后循环动脉狭窄高于青年组(P<0.01)。结论动脉粥样硬化性缺血性脑血管病患者脑动脉狭窄的分布有疾病和年龄特征性。     

缺血性脑血管病患者脑动脉狭窄的分布及特征

目的　探讨动脉粥样硬化性缺血性脑血管病患者脑动脉狭窄的分布特征。方法　选择连续行主动脉弓 +全脑血管造影检查的成年缺血性脑血管病患者 196例 ,将其中 171例存在脑动脉狭窄的患者按年龄分为青年组 (18～4 4岁 )、中年组 (45～ 5 9岁 )及老年组 (≥ 6 0岁 ) ,分析脑动脉狭窄随年龄变化的分布规律。结果　 171例脑动脉狭窄的患者中颅内动脉狭窄的发生率 (80 .7% )明显高于颅外动脉 (5 6 .1% )。青年组单纯颅内动脉狭窄的比例较高 ,主要发生在大脑中动脉。中年组及老年组颅内、外动脉狭窄并存的比例较高。颅外动脉病变数目随年龄不断增加(P <0 .0 0 1)。结论　动脉粥样硬化性缺血性脑血管病患者颅内动脉狭窄的发生率高于颅外。脑动脉狭窄的分布有年龄特征性     

脑血管造影术并发症发生原因分析及防治措施

目的分析脑血管造影术并发症的发生原因及防治措施。方法选择2008年12月—2011年12月在我院实施脑血管造影术的患者150例,其中9例发生脑血管造影术并发症,分析其发生原因并提出防治措施。结果脑血管造影术并发症发生率为6.0%(9/150),其中局部并发症3例、神经系统并发症2例、其他并发症4例。结论神经系统并发症是脑血管造影术常见的严重并发症,提高穿刺技术和熟练掌握造影术操作有利于减少并发症的发生,提高造影术成功率。     

老年人自发性蛛网膜下腔出血诊治临床分析

目的 研究老年人自发性蛛网膜下腔出血患者的诊治策略及疗效. 方法 68例60岁以上自发性蛛网膜下腔出血患者,均行全脑血管造影,共发现颅内动脉瘤72个,47例患者(59个动脉瘤)接受栓塞治疗,6例患者(6个动脉瘤)行显微神经外科手术夹闭,7例患者选择保守治疗.结果 57个动脉瘤被成功栓塞,技术成功率为96.6%,其中致密栓塞40个(占70.2%),不完全栓塞17个(占29.8%);47例患者中,出院时根据格拉斯哥预后评分(Glasgow outcome scale,GOS)良好39例(占83.0%),较差7例(占14.9%),死亡1例. 结论 对于老年自发性蛛网膜下腔出血患者特别是存在颅内动脉瘤者,积极的干预措施如血管内栓塞治疗能取得较好的效果.     

自发性蛛网膜下腔出血急诊脑血管造影

作者对该院最近20年收治的592例自发性蛛网膜下腔出血(SAH)患者的临床特征和病因进行了分析和探讨.572例SAH患者脑血管造影结果显示,脑动静脉畸形是SAH的最常见病因(395/572例),其次为颅内动脉瘤(98/572例).脑动静脉畸形和颅内动脉瘤患者在脑血管造影后均行血管内栓塞治疗和动脉瘤夹闭术.其中绝大部分患者取得良好效果.因此,作者认为SAH急诊脑血管造影不仅可以迅速明确病因,而且可以采取及时有效的治疗措施,降低SAH的复发率和病死率;急诊脑血管造影的最佳时机在SAH发病后6～72h.     

急性缺血性脑卒中诊断的MRA和DSA分析

目的探讨急性缺血性脑卒中诊断中MRA和DSA分析的应用价值。方法选择苏州市市立医院北区神经内科于2010年5月—2012年4月收治的236例急性缺血性脑卒中患者经MRA检查以诊断脑血管狭窄程度,合并30例经MRA诊断为脑血管狭窄患者经DSA检查以进一步分析病变原因和病变部位。结果 236例急性缺血性脑卒中经MRA分析,脑血管正常71例,占30%;脑血管狭窄(含闭塞)165例,占70%。30例经MRA诊断为脑血管狭窄患者实施DSA检查,其病因分布情况为动脉粥样硬化脑血管病21例,非动脉粥样硬化脑血管病9例。21例动脉粥样硬化脑血管病的病变部位分布为颅内病变11例,颅外病变6例,颅内外联合病变4例。结论临床应用上优先采用磁共振血管造影术为急性缺血性脑卒实施诊断,对诊断为重度狭窄(>70%)以上的患者实施DSA,以进一步分析病变部位和病变原因,采取合理有效防治措施。     

Angiography diagnosis in the elderly--current indications in reduced complication rates and expanded therapeutic modalities

A potential higher rate of complications, recent developments in the technical procedures, and expanding modalities of interventional or operative therapy must be considered in evaluating angiographies in the aged patient. Most frequently, imaging of the pelvic and femoral arteries as well as the cerebral vessels was performed in patients older than 70 years, in 40% as out-patient angiography. The risk of puncture-site, vascular and catheter complications, as well as of neurological deficits was not found increased. A higher rate of cardiovascular problems however requires exact internal investigations and evaluation of the biological age. Angiographic information about vascular morphology as a basis for interventional therapy can be achieved today with an acceptable risk in the aged patient.     

Neurologic manifestations of infectious endocarditis

Thirty out of 287 patients (10.4%) admitted to hospital for infective endocarditis between December 1970 and January 1990 had neurological complications. Twenty-three patients had native valve infectious endocarditis and 7 had prosthetic valve endocarditis. The clinical features were characterized by the frequency of aortic valve involvement (23 out of 30) and other complications, especially cardiac failure (16 cases) and peripheral vascular manifestations (7 cases). The commonest organism was the staphylococcus (53% of identified organisms) but the number of negative blood cultures was high (50% of cases). The neurological complication was often the presenting symptom of the endocarditis (19 cases) but it occurred after bacteriological cure in 4 cases. The complications observed were cerebral ischemia (16 cases), cerebral haemorrhage (11 cases), coma (2 cases), and one peripheral neuropathy causing a Claude Bernard Horner syndrome. These complications presented with hemiplegia in 17 cases, a meningeal syndrome in 8 cases, a convulsion in 1 case, a Von Wallenberg syndrome in 1 case, and a Claude Bernard Horner syndrome in 1 case. Twelve patients had a transient or permanent neurological coma. Cerebral CT scan showed ischemic lesions in 7 cases and haemorrhagic lesions in 10 cases. Carotid angiography demonstrated mycotic aneurysms in 6 patients. Twelve patients died: the cause of death was neurological coma (7 cases), low cardiac output (4 cases) and haemorrhagic shock (1 case). Four patients underwent neurosurgery: 3 for clipping a mycotic aneurysm and 1 for drainage of an intracerebral haematoma. Poor prognostic factors were: coma, cardiac failure, cardiac valve prosthesis and, above all, the extent and multiplicity of the neurological lesions. The authors propose the following measures to improve the prognosis: early surgery in cases of large and/or mobile vegetations especially when the infecting organism is a staphylococcus and when a systemic embolism has occurred; routine CT scanning and/or digitised cerebral angiography in all patients with infective endocarditis to detect surgically accessible mycotic aneurysms.     

Effects of coronary insufficiency on the early and late results in patients surgically treated for aneurysm of the sub-renal abdominal aorta

Between 1973 and 1988, 200 patients underwent repair of unruptured aortic aneurysm located distal to the renal arteries. There were 181 men (90%) and the mean age was 68.8 years. The most serious associated disease was arteriosclerotic heart disease which was present in 102 patients (51%): 48 patients had angina pectoris; 59 patients had previous myocardial infarct; 8 patients had ischemic myocardiopathy. Associated cerebrovascular disease was found in 29 patients (15%). Of these 200 patients, 36% had no symptoms relating to the aneurysm. The aneurysm was associated with iliac aneurysm (19%), iliac occlusion (14%), distant femoral occlusion (14%). In patients with history of coronary arteries disease (102), 39 (18%) had a coronary angiography prior the elective resection, 18 (9%) coronary artery bypass surgery underwent elective myocardial revascularisation prior to elective resection of their aneurysm. The treatment was by graft replacement and exclusively by graft inclusion. RESULTS: Death occurred within 30 days of treatment in 5 patients (2.5%). The first cause of early death was myocardial infarct (3). Early peripheral vascular complication occurred within 30 days in these 200 patients and were thromboembolism in 12 patients and colic ischemia in 8 patients. Of the 83 patients (1975-1983) who survived operation, follow up information regarding survival was obtained in 79 patients. The overall 5 and 8 years survival rates in percentage in the series were 69% and 50%. The survival rate was greatest in patients free of associated disease and worse in patients with myocardial infarctus. Subsequently 24 vascular operations were performed in these patients: 7 iliac aneurysms, 16 occlusive lesions and 2 false aneurysms. DISCUSSION: Young (15) and associates reported an operative mortality rate of 6.3% for elective aneurysm resection but found that 20% of the patients with pre-operative evidence of coronary artery disease had post-operative myocardial infarct of which 58% were fatal. Hertzer and colleagues (6), using routine coronary angiography prior to elective aortic reconstruction, have documented a 59% incidence of significant anatomic coronary artery disease. This incidence increased to 95% in patients with abdominal aortic aneurysm and suspected coronary artery disease. Only one patient of the 68 patients with an abdominal aortic aneurysm had normal coronary arteries in their series. Thus, considering the omnious implications of coronary artery disease in patients with abdominal aortic aneurysms, routine preoperative coronary angiography has been recommended. For Brown and coll. (1), it would appear that the risk of prophylactic coronary artery revascularisation may be greater than that for elective abdominal aortic aneurysm resection alone in the older age group. For the authors, only patients which instable angina pectoris or angina pectoris with a myocardial infarct had a coronary angiography. The coronary artery bypass is recommended for left maintrunk obstruction or diffuse multivessel coronary artery disease.     

Atteintes cardiovasculaires de la maladie de Behçet

Vascular involvement is a common complication of Behçet's disease (BD) and affects up to 40% of BD patients. These complications worsen the prognosis of BD. The concept of vasculo-Behçet has been adopted for cases in which vascular complications dominate the clinical features. Vascular manifestations affect particularly young men, during the first years following onset of the disease. Venous complications are the most frequent vascular complications, affecting 14 to 40% of BD patients. Superficial and deep lower limb thrombosis is the most frequent venous complications but one third of venous thrombosis concern large vessels (such as cerebral venous thrombosis, pulmonary embolism, and inferior or superior vena cava, etc.). Budd-Chiari syndrome is the worst prognostic factor increasing mortality by 9 times. Arterial complications (2 to 17% of BD patients) include aneurysms and occlusions/stenosis. Main locations of arterial lesions are aortic (abdominal and thoracic), femoral, pulmonary and iliac arteries. Aneurysms are the most severe arterial complications, particularly pulmonary aneurysms associated with a high risk of massive bleeding. Cardiac complications (up to 6% of BD patients) include pericarditis, endocardial lesions (aortic regurgitation and less often mitral insufficiency), myocardial lesions (myocardial infarction, myocarditis and endomyocardial fibrosis) and intracardiac thrombosis (right ventricle and atrium). Coronary lesions complicated to myocardial infarction are the most severe cardiac complications. Treatment is based on corticosteroids and immunosuppressive drugs. The use of anticoagulation in venous thrombosis is still controversial.     

脑动脉狭窄分布特征及其危险因素的分析

目的探讨缺血性脑血管病患者的脑动脉狭窄病变的分布,以及与动脉粥样硬化危险因素的关系。方法回顾性分析377例经脑血管造影检查的脑缺血患者的临床资料,由两名神经科医师分别对血管狭窄病变的部位、程度进行统计,并按照病变发生的部位及程度,分为单纯颅内动脉病变组、单纯颅外动脉病变组、重度狭窄及闭塞组以及多发性血管病变组,对其动脉粥样硬化的危险因素进行分析。结果（1）377例患者中,存在脑血管病变的患者有285例,共检出病变数892处,其中颅外动脉病变438处（49．1％）,颅内动脉病变454处（50．9％）。前者最常见于颈内动脉颅外段（19．6％,175／892）,后者最常见于颈内动脉颅内段（15．0％,134／892）及大脑中动脉（12．4％,111／892）。②对血管狭窄程度的分析表明,颅外动脉中,以椎动脉及颈内动脉颅外段的狭窄程度最为严重,与颈总动脉比较,差异有统计学意义（P〈0．05）;颅内动脉则以大脑前动脉及大脑中动脉的狭窄程度严重。③单纯颅内动脉病变的患者合并有高血压病的比率较单纯颅外动脉病变者多（80．39％,59．42％）,差异有统计学意义（P=0．015）。存在严重血管病变患者的多项动脉粥样硬化危险因素较造影正常的患者增多。结论缺血性脑血管病患者颅内动脉病变略多于颅外动脉。椎动脉及颈内动脉颅外段、大脑前动脉、大脑中动脉狭窄程度较为严重。单纯颅内动脉病变的患者合并有高血压病的比率较单纯颅外动脉病变者明显增高;动脉硬化危险因素增多,脑血管造影出现血管重度狭窄的概率增大。     

老年脑动脉瘤患者血管内治疗的疗效分析

目的探讨经血管内治疗老年脑动脉瘤的疗效。方法回顾性分析38例经血管内治疗的60岁以上脑动脉瘤患者,总结老年脑动脉瘤患者的临床特点、介入治疗方式及临床并发症,按照改良Rankin量表评分标准评价预后。结果本组共计脑动脉瘤42个,41个被成功栓塞。71%以上患有高血压或糖尿病等基础疾病。术后3个月预后佳者33例,占86.8%。结论老年脑动脉瘤患者血管条件差,基础疾病多,对老年人应用血管内治疗安全有效。     

Feasibility and application of single 5F multipurpose catheter in coronary and peripheral angiography via a transradial approach

Objective

Atherosclerosis is a systemic disease, in which coronary and peripheral angiographies are required to be done at the same time in a large number of patients. To shorten the procedure time, and reduce complications, we tested the feasibility and the safety of using a single 5F multipurpose catheter, via transradial approach, for coronary, cerebral and renal angiographies.

Methods

One thousand and ninety-two patients were enrolled in the study. The procedure time, local vessel complications, duration of hospitalization, and costs were evaluated.

Results

Among 1092 patients, the radial artery puncture was successful in 1081 patients, a successful coronary angiography via the radial artery was done in 1074 patients, and the remaining 18 patients had to be accessed via the femoral artery. Thus, successful angiography rate was 97.7% for the right coronary artery, 95.8% for the left coronary artery, 100% for the right cerebral artery, 95.2% for the left subclavian artery, 96.1% for the left carotid artery and 83.1% for the renal artery. The failures were caused by abnormal curvature of the aortic arch and abnormal origins of the above-mentioned arteries. There were 1460 artery stenosis lesions found in 661 patients and 624 lesions (93.3%) needed stents via the transradial approach. The mean procedure time was 20.9 ± 9.3 min including puncture, angiography and hemostasis time. There were 4.35% complications. No local hematoma, hand ischemia, or cerebral infraction was found in this study.

Conclusion

Angiography using a single 5F multipurpose catheter, via transradial approach, is associated with a short procedure time and a low rate of complications without affecting the angiography success rate.     

Advantages of multislice spiral computed tomography for evaluation of serious coronary complications after Kawasaki disease

OBJECTIVES: Novel multislice spiral computed tomography (MSCT) findings were identified in patients after Kawasaki disease that could not be detected by coronary angiography (CAG). METHODS: Eighteen patients had suffered from serious coronary arterial lesions after Kawasaki disease (mean age 21.7 years, range 13-34 years). Seventeen patients had stenotic lesions, and all of them had coronary aneurysms. MSCT was performed using a Siemens SOMATOM Volume Zoom (4-detector row) or a Toshiba Aquillion 16 (16-detector row). Findings of coronary calcification, stenotic lesion, and intimal hypertrophy in all coronary arteries were compared to those of CAG. RESULTS: Eleven of the 18 patients (61%) had novel findings detected by MSCT. Coronary calcifications were found in 11 of the 18 patients (61%). Five patients had concentric calcified aneurysms, four had eccentric calcified aneurysms, and two had mixed calcified aneurysms. Coronary stenotic lesions were present in 6 of the 18 patients (33%) with calcified aneurysms. Two patients had intimal hypertrophy (11%). One patient had intimal hypertrophy along the left main trunk with a giant calcified aneurysm along the left anterior descending artery. Two patients had severe stenoses just distal to giant calcified aneurysms that were regarded as false positive findings, and were identified as mild stenoses by CAG. CONCLUSIONS: MSCT offers advantages over CAG in the evaluation of calcified aneurysms and intimal hypertrophy, and is a potential diagnostic modality for coronary intervention in patients after Kawasaki disease.     

Coronary Endothelial Dysfunction After Kawasaki Disease: Evaluation by Intracoronary Injection of Acetylcholine

Objectives. This study sought to assess the endothelial function of long-term coronary artery lesions in patients with Kawasaki disease (KD).Background. The vascular function of the coronary arteries in children with long-term KD remains uncertain. We report our findings of the vascular response of the coronary arteries to intracoronary injection of acetylcholine (ACh) in patients with KD.Methods. A total of 35 patients (25 patients with KD and 10 control subjects) were examined using coronary angiography. Individual arteries were divided into four groups according to the type of the coronary artery lesion: group 1 consisted of 25 sites with regressed aneurysms. These aneurysms had developed in the acute stage but had subsequently regressed and demonstrated normal findings on the follow-up coronary angiogram. Group 2 consisted of 24 sites with persistent aneurysms. Group 3 involved 60 angiographically normal sites in the same patients as those in group 1 or 2. Group 4 consisted of 30 sites in control subjects who had congenital heart disease with normal coronary arteries. During coronary angiography we infused 15 μg of ACh chloride into the coronary artery. The lumen diameters were measured using a cine videodensitometric analyzer to study the distensibility of the coronary artery wall.Results. The mean (±SD) change in diameter was an increase of 11.71 ± 12.34% in group 3 (coronary arteries without lesions in patients with KD) and 12.21 ± 9.71% in the control group, demonstrating marked vasodilation in both groups. In contrast, the changes in the regressed aneurysms of group 1 and in the persistent aneurysms of group 2 were −2.65 ± 12.12% and −0.08 ± 6.51%, respectively, demonstrating no change or mild vasoconstriction. The change in groups 1 and 2 was significantly less than that in group 3 or in the control group. Group 3 showed no significant difference from the control group.Conclusions. These findings suggest that long-term coronary artery lesions, even after aneurysm regression, may have impaired endothelial function. A long-term follow-up study for those patients is essential.     

Pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: a series of 126 patients

Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder characterized by epistaxis, telangiectasia, and visceral vascular manifestations. Infectious and ischemic central nervous system (CNS) manifestations due to embolism through pulmonary arteriovenous malformations (PAVMs) represent the main causes of morbidity. To improve the phenotypic characterization of HHT with PAVM, we conducted a retrospective multicenter study of patients with HHT and at least 1 PAVM detected by chest computed tomography (CT) and/or pulmonary angiography, with particular attention to CNS and infectious manifestations. The study included 126 patients (47 men, 79 women), with a mean age of 43.1 +/- 17.4 years; 45 patients had a mutation of the ENG gene and 16 had a mutation of ACVRL1. PAVMs were diagnosed as a result of systematic screening procedures (29%), incidental imaging findings (15%), dyspnea (22%), or CNS symptoms (13%). The PAVMs were diagnosed at a mean age of 43 +/- 17 years, with a linear distribution of diagnosis between 20 and 75 years. Dyspnea on exertion was present in 56% of patients. Four patients had a hemothorax, including 1 during pregnancy. Fifty-three CNS events directly related to HHT (excluding migraine) were observed in 35% of patients: cerebral abscess (19.0%), ischemic cerebral stroke (9.5%), transient cerebral ischemic attack (6.3%), and cerebral hemorrhage (2.4%). The median age of onset was 33 years for cerebral abscesses (range, 11-66 yr), and 53.5 years for ischemic cerebral events (range, 2-72 yr). Migraine was reported in 16% of patients. The diagnoses of PAVM and HHT were made at the time of the cerebral abscess in 13 cases (54%). Forty-three percent of patients were hypoxemic at rest. Contrast echocardiography showed intrapulmonary right-to-left shunting in 87% of tested patients. PAVMs were seen on chest radiograph in 54% of patients, and on the CT scan in all patients. One hundred five patients (83%) underwent treatment of the PAVM, by percutaneous embolization (71%) and/or by surgical resection (23%). A high frequency of CNS and infectious complications was observed in this large series of patients with HHT-related PAVM. Physicians may not be sufficiently aware of the clinical manifestations of this orphan disorder. Patients diagnosed with HHT should be informed by physicians and patient associations of the risk of PAVM-related complications, and systematic screening for PAVM should be proposed, regardless of a patient's symptoms, familial history, or genetic considerations.