A case of multiple immunoglobulin G4-related periarteritis: a tumorous lesion of the coronary artery and abdominal aortic aneurysm

Immunoglobulin G4 (IgG4)-related disease can occur in various organs, most of which are glandular or ductal tissues. Here, we report a case of multiple IgG4-related vascular lesions. A 63-year-old patient was found to have an abdominal aortic aneurysm and a tumorous lesion around the right coronary artery. The surgically resected aneurysmal wall and a tumorous lesion of the right coronary artery showed similar histologic features including diffuse lymphoplasmacytic infiltration, occasional eosinophils, and obliterative phlebitis. Immunohistochemically, numerous IgG4-positive plasma cells were evident within the lesions. The serum concentrations of IgG4 in the preoperative period was 456 mg/dL (reference range, <135), which decreased to 242 mg/dL 2 weeks after surgery. We made a diagnosis of multiple IgG4-related periarteritis manifesting as an abdominal aortic aneurysm and a tumorous nodule of the coronary artery. This case report suggested that IgG4-related disease can occur in the vascular system and manifest as an aneurysm or a periarterial mass lesion.     

基于冠状动脉CT血管成像及三维重建的室间隔膜部膨出瘤的影像学特征观察

目的 探讨基于冠状动脉CT血管成像（CTA）及三维重建的室间隔膜部膨出瘤（IVMSA）的影像解剖学特征及其临床意义。方法 横断面研究。纳入2013年1月—2022年5月山东大学齐鲁医院德州医院放射科经冠状动脉CTA检出的50例IVMSA,其中男15例、女35例,年龄35~80（59±11）岁。冠状动脉CTA横断面图像上找到室间隔膜部的位置,采用容积再现（VR）、多平面重组（MPR）和最大密度投影（MIP）等后处理技术进行三维重建,分析IVMSA在形态、大小、膨出方向、瘤壁及瘤腔血栓形成情况等方面的CT影像学特征。结果 （1）IVMSA形态：50例IVMSA中多叶状47例,呈分叶大小不均的多分叶状;半球形2例,其表面较光滑,瘤颈直径大于瘤体长径;隧道样1例,表现为弯曲长条形瘤腔,游离于右心室内,瘤体长径超过瘤颈直径4倍。（2）IVMSA大小：瘤颈直径为1.95~18.95（8.36±3.84）mm,瘤体长径范围为2.39~20.70（9.67±3.77）mm。（3）IVMSA膨出方向：49例患者IVMSA膨向右室流入道,1例膨向右室流出道。（4）IVMSA瘤壁：1例室间隔缺损修补患者术后合并之IVMSA瘤壁破裂,其余49例未见瘤壁破裂迹象。（5）IVMSA瘤腔：50例患者均未见IVMSA瘤腔内血栓形成。结论 IVMSA形态上以多叶状最多见,大小不一,多数膨向右室流入道,极少数可出现瘤壁破裂,瘤腔内血栓形成较为罕见。冠状动脉CTA结合三维重建技术,可以对IVMSA的影像形态进行综合观察,为临床诊疗提供可靠的依据。     

Asymptomatic Continuous Murmur Due to Acquired Coronary Artery to Right Ventricular Fistula

An unusual case of an acquired coronary artery to right ventricular fistula is reported. A new continuous murmur was noted one month following a puncture wound to the precordium. Cardiac catheterization documented a left anterior descending coronary artery to right ventricular communication with a small left to right shunt and normal cardiac pressures. The angiographic appearance was that of a false aneurysm of the anterior descending artery with rupture into the right ventricle. The patient has been followed for 36 months and is asymptomatic with no evidence of myocardial ischemia or infarction. Because of the small size of the fistula and its intracardiac position, conservative treatment has been employed.     

Dissecting aneurysm of a coronary artery due to percutaneous transluminal balloon angioplasty

Percutaneous transluminal balloon angioplasty (PTCA) is utilized successfully to dilate stenotic coronary arteries. The procedure is a frequent nonsurgical method to restore coronary artery blood flow to ischemic myocardium. In this paper, we report a case of intimal tear and medial dissection of the right coronary artery leading to a dissecting aneurysm and occlusion of the coronary artery. The patient died following coronary artery bypass grafts, and the autopsy demonstrated the dissecting aneurysm of the coronary artery.     

Prevalence of fenestrated basilar artery with magnetic resonance angiography: a transversal study

Fenestration of the basilar artery (BA) is a rare anatomical variation in comparison to those of the other intracranial arteries constituting the cerebral arterial circle. The incidence is difficult to ascertain and data vary according to type of series and modalities of detection. Basilar artery fenestration (BAF) has been reported in association with arteriovenous malformations, vascular variants, other developmental anomalies and neurovascular conflicts as a consequence of relations between the arterial branches of the BA and the nerves and other structures in the posterior cranial fossa. However, the real clinical interest of BAF is due to the possible formation of an aneurysm at the junction of the fenestrated segment and less frequently to the thrombosis of the vessels. With the aim to establish the prevalence of BAF in our population, we made a transversal pilot study of the first 200 MR angiographies performed on patients attending for the first time to control their base pathology (vascular or not). We have described three patients with this condition (representing a prevalence of 1.5 % on MR angiography) to shed additional light on this anomaly, two cases located at 1/3 proximal end (type 1-BAF) and one case located at joint 1/3 medium–1/3 distal end, locating distal to the anterior inferior cerebellar artery (type 4-BAF). In neither case was any other lesion found (i.e. aneurysm, infarctions, ischemia or thromboembolism). The pertinent clinical anatomy and embryological basis for this variation are reviewed, and the possible clinical implications and associated findings are discussed.     

Postmortem findings in morbidly obese individuals dying after gastric bypass procedures

Mortality has been reported to complicate gastric bypass, with common causes of death attributable to anastomotic leaks, sepsis, hemorrhage, and bowel obstruction. We evaluated autopsy reports from 10 patients having undergone gastric bypass. Medical records were reviewed to identify comorbidities. Data of interest included preoperative electrocardiogram (EKG) abnormalities, cause of death, body weight, anastamosis appearance, heart weight, extent of coronary artery disease, ventricular size, liver weight, and gall bladder status. A total of 7 men and 3 women were autopsied. Average age was 40 years (range, 30-49 years), and mean body mass index at autopsy was 60.3 kg/m(2) (range, 33.2-80.9 kg/m(2)). Evidence of anastomotic leaks was present in 7 cases, resulting in 4 deaths. Death was attributed to pulmonary embolism in one case. There were 5 cardiac-related deaths, all attributed to arrhythmias. Microscopic evidence of coronary artery disease was observed in 6. Cardiomegaly was seen in all patients, left ventricular hypertrophy in 8, right ventricular hypertrophy in 3, and hepatomegaly in all 10. Nine patients were status post cholecystectomy. Of the 8 preoperative EKG available, abnormalities were identified in 5. After gastric bypass, death was attributed to cardiac-related causes, pulmonary embolism, and operative complications. A significant proportion of cardiac-related deaths occured in the absence of atherosclerosis. Most patients had preoperative EKG abnormalities. As a high incidence of cardiomegaly was observed, operative stress associated with the procedure may increase the risk of arrhythmia in morbid obesity. Consequently, in morbidly obese patients, a detailed preoperative cardiovascular evaluation is warranted to reduce postoperative mortality.     

A case of intravenous immunoglobulin-resistant Kawasaki disease treated with methotrexate

Kawasaki disease, an acute febrile vasculitis of unknown etiology, is usually treated with high doses of immunoglobulin (IVIG) and aspirin. However, 20% of children show persistent or recurrent fever despite IVIG, and coronary artery aneurysm progression. In such cases of resistance to IVIG treatment, repeated IVIG administration or the initiation of steroid therapy, and the use of cyclophosphamide have been reported. We aimed to show in this study that methotrexate (MTX) may be used as a treatment for Kawasaki disease resistant to IVIG treatment. We report the case of a 6-year old boy who was admitted at another hospital with an initial complaint of a fever for 5 days and skin rashes for 3 days. The patients fever persisted despite three courses of IVIG (2 gm/kg, 1 gm/kg, 1 gm/kg, respectively) over a 14-day period. On day 14 of his illness he showed a dilated right coronary artery, and on day 19 dexamethasone, at a daily dose of 0.3 mg/kg, was given but this resulted in defervescence. However, upon stopping the dexamethasone treatment, his fever recurred and he was transferred to our hospital. On days 31 and 38 of his illness, IVIG (400 mg/kg for 5 days, twice) was administered and from day 38 onwards the patient was given dexamethasone (0.6 mg/kg, daily) and MTX (10 mg/BSA, once weekly) whereupon his fever subsided and did not recur. On day 48 dexamethasone was replaced with prednisolone, which was subsequently tapered. The patient is now taking MTX and being observed on an outpatient basis. We report the case of a boy with IV-globulin resistant Kawasaki disease, who after repeated infusions of IVIG and steroid therapy showed fever recurrence, which that subsided after MTX treatment.     

Uncommon associations and catastrophic manifestation in Takayasu arteritis: an autopsy case report

Takayasu arteritis, a chronic inflammatory vasculitis affecting aorta and its major branches, is complicated by stenosis, occlusion, and aneurysm formation. The aneurysm formation and subsequent complications such as heart failure, aortic regurgitation, and aneurysm rupture can be fatal. The aortic aneurysm rupture is a rare and fatal complication with only a few cases reported in the English literature. The involvement of coronary artery in Takayasu occurs in about 10% patients, and the coronary artery aneurysm is the least common manifestation. Here, we describe a case of Takayasu arteritis with abdominal aortic aneurysm rupture and coronary artery aneurysm. This patient also had associated systemic inflammatory diseases like sarcoidosis and Hashimoto's thyroiditis.     

Aneurysm of the great cardiac vein

Anatomical variations in the cardiac veins have the potential to cause iatrogenic injuries during cardiac surgical procedures or cardiac resynchronization therapy. We present a case of an 86-year-old man, which presented with a great cardiac vein aneurysm. The great cardiac vein arose near the apex of the interventricular sulcus to the right of the anterior interventricular branch (AIB) of the left coronary artery and crossed the AIB anteriorly to the left. The great cardiac vein aneurysm appeared to be due to a possible distal constriction of the great cardiac vein by a small muscular branch of the circumflex branch and a possible proximal constriction by the left marginal artery. Cardiologists who interpret imaging of the cardiac veins and cardiac surgeons who operate close to the great cardiac vein should be aware of such a variation.     

Evidence at autopsy of spasm in the distal right coronary artery in persons with coronary heart disease dying suddenly

The aim of this study was to examine whether there are morphological signs in spasm in the coronary arteries at autopsy in persons with coronary artery disease dying suddenly. From a forensic autopsy service, 48 cases of sudden and unexpected deaths were selected: 24 cases with a preliminary diagnosis of coronary heart disease and 24 cases involving persons dying of noncoronary causes. A complete autopsy according to a preset protocol was followed with particular emphasis on the heart examination. The myocardium and the coronary arteries were sampled and examined without knowledge to which group the case belonged. The degree of folding of the internal elastic lamina of the proximal and distal parts of the coronary arteries was measured by picture analysis of elastin-stained cross sections of the arteries. The degree of folding was significantly greater in the distal section of the right coronary artery in cases of the coronary group compared to the folding in the same section in cases of the noncoronary group. In the proximal part of the right coronary artery and in the left coronary artery with its two branches, there were no differences in the folding of the internal elastic membrane between the groups.

Our findings indicate that a spasmic contracture of an artery may be diagnosed postmortem. The spasm of the distal part of the right coronary artery may have caused focal ischemia in the central parts of the cardiac conducting system, precipitating a lethal arrhythmia.     

Cardiac tamponade due to a rupture of the coronary arteriovenous aneurysm--a case report.

We experienced an unusual case of cardiac tamponde caused by a rupture of the coronary arteriovenous aneurysm in a 54-year-old woman. The patient was suffered from sudden chest pain and syncope, and was initially managed by pericardiocentesis following an echocardiogram which revealed a massive pericardial effusion with signs of cardiac tamponade. She was referred to our hospital under the impression of aortic dissection with cardiac tamponade. She underwent an emergency operation and was found to have a 2 x 2 cm sized bleeding cystic mass protruding from the proximal anterior descending coronary artery. The aneurysm was excised and the openings connected with the coronary artery and right ventricular outflow tract were closed with sutures from the inside of aneurysm. Subsequent coronary arteriography supported the diagnosis.     

In vivo validation of the design rules of the coronary arteries and their application in the assessment of diffuse disease

The conventional rationale that uses per cent diameter reduction to assess diffuse coronary artery disease is not appropriate because no normal reference segments exist. In a recent publication, we have proposed a theoretical model based on physical principles that relate the various morphological and haemodynamic parameters (cross-sectional area, length, volume and flow) of the normal coronary arterial tree. The model was validated using haemodynamic simulations based on detailed morphological data of the pig coronary arterial tree. This paper extends the model validation to in vivo swine studies. Coronary arteriography was performed in five swine (15-18 kg body weight) after power injection of contrast material into the coronary artery. Coronary arterial length was obtained using a 3D reconstruction technique. The arterial volume, cross-sectional area and blood flow were measured using videodensitometry. The proposed relationships between these quantities were validated. Furthermore, a sensitivity analysis was demonstrated based on a simulation of diffuse coronary artery disease (approximately 40% reduction in cross-sectional area). The results of a sensitivity analysis based on a simulation of diffuse coronary artery disease suggest that the relationships between arterial volume, cross-sectional area, blood flow and the distal arterial length can be utilized to quantify moderate levels of diffuse coronary artery disease.     

A giant coronary artery aneurysm in the right coronary artery.

Aneurysms of the coronary arteries are uncommon occurrences that usually develop secondary to atherosclerosis and are often asymptomatic. They are usually diagnosed incidentally during investigation for ischemic heart disease or at autopsy for sudden death. We present a case of a "giant" right coronary artery aneurysm (CAA) discovered incidentally at surgery. Pathological examination confirmed that this was a true aneurysm showing marked thinning of the media and fibrocalcific plaques with small, multifocal areas of lymphocytic infiltrates.     

Deep femoral artery aneurysm presenting as lower limb swelling: a case report

We report a rare case of lower limb swelling due to compression of the superficial femoral vein by a solitary deep femoral artery aneurysm. The patient was a 58-year-old man presenting with acute swelling of the right lower limb caused by deep venous thrombosis. A multi-detector computed tomographic scan (CT) confirmed the diagnosis of a deep femoral artery aneurysm and revealed no evidence of aneurysms or occlusive lesions in the other arteries. Aneurysmectomy and ligation of the deep femoral artery were successfully performed. Preoperative multi-detector CT scanning is a valuable, non-invasive diagnostic tool to delineate not only the state of the aneurysm, but also that of the distal arterial tree.     

In vitro performance of the novel coronary sinus AutoRetroPerfusion Cannula

Myocardial salvage through coronary sinus intervention has been documented. The AutoRetroPerfusion Cannula is a novel device that is able to perfuse the coronary bed retrogradely through the coronary sinus with arterial blood generated from a peripheral artery with no need for a pump. The cannula consists of a distal end that, once secured in the coronary sinus, opens an umbrella-like membrane to create pressure in the coronary sinus, and at the same time has small channels directed backwards to the right atrium to provide pressure relief. The cannula is introduced from the axillary vein under local anesthesia and the proximal end, which consists of a graft, is anastomosed to the axillary artery to start autoperfusion once the distal end is secured in the coronary sinus and the occluding membrane is open. The AutoRetroPerfusion Cannula was tested in the in vitro mock loop under 50-120 mm Hg of proximal pressure and 50, 100, and 150 ml/min of total flow in the cannula. We were able to achieve the nominal design point of 40-80 mm Hg of distal pressure and 50-150 ml/min of distal flow by adjusting the number, diameter, and length of the small backwards channels.     

A case of an aneurysm of the coronary artery

A rare case of the right coronary artery aneurysm is described. The condition had a clinical course of the heart ischemic disease. The death occurred due to the aneurysm rupture and haemopericardium.     

Sudden death due to left coronary artery occlusion in infective endocarditis

A 44-year-old man died suddenly, shortly after admission to the hospital with complaints of abdominal pain. Medical history was significant for chronic alcoholism and homozygous hemoglobin C disease. Autopsy revealed vegetations on the aortic valve, especially on the left coronary cusp. There was anomalous origin of the coronary arteries from the left sinus of Valsalva. The large vegetation on the left coronary cusp had extended into the left main-stem coronary artery and obstructed it. There was evidence of prior embolization to the right coronary artery with mycotic aneurysm formation and myocardial infarction. Other lesions included a cerebral artery mycotic aneurysm and metastatic abscesses within the myocardium and spleen. Although the aortic valve was free of underlying chronic pathology, the causative organism was Streptococcus viridans. This case illustrates several unusual, and, in some instances, unique findings in infective endocarditis.     

Complex anomalies of type 1 proatlantal intersegmental artery and aortic arch variations

Purpose

We report a case of type 1 proatlantal intersegmental artery (PIA) associated with multiple anomalies of the aortic arch, and discuss the possible embryonic mechanism and clinical importance of the multiple cerebrovascular variants in this patient.

Methods

A 65-year-old woman with dizziness underwent cerebral magnetic resonance (MR) imaging and head and neck MR angiography using a 3-tesla scanner and computed tomography (CT) angiography using a 64-slice multidetector CT scanner.

Results

MR and CT angiography demonstrated an aneurysm of the distal end of the azygos anterior cerebral arteries and hypoplasia of the proximal right vertebral artery (VA) with an anastomotic artery, between the right internal carotid artery (ICA) and distal right VA that passed through the foramen magnum, indicating a type 1 PIA. She also demonstrated an aberrant right subclavian artery (ARSA) with hypoplasia of the right VA, and the left VA arose directly from the aortic arch.

Conclusion

To our knowledge, this is the first report of a type 1 PIA associated with multiple vascular anomalies of the aortic arch, such as ARSA and origin of the left VA from the arch. In cases of persistent anastomoses between the carotid and vertebrobasilar arteries, such as PIAs, imaging examination should include the aortic arch to identify associated vascular variations.     

Prostacyclin and regional coronary blood flow in experimental myocardial infarction

Infusion of prostacyclin (PGI2) has been reported to affect infarct size and myocardial blood flow favourably in various animal models of myocardial ischaemia. Recent data suggest that a similar effect of PGI2 may occur also in humans with acute myocardial infarction. We addressed the hypothesis that PGI2 redistributes myocardial blood flow following coronary ligation, and that this effect favours perfusion of myocardium at risk and thereby limits infarct size. Following ligation of a distal branch of the left coronary artery in anaesthetized dogs, PGI2 (2-4 ng/kg/min) was infused for 72 h. Regional myocardial blood flow was assessed immediately after the coronary ligation and at the end of the drug infusion, by injection of 57Co- and 113Sn-labelled microspheres, respectively. Coronary ligation reduced regional coronary blood flow by 40-70%. During the subsequent 72 h the blood flow increased, being at the end of the period 50-70% of the flow in the non-ischaemic myocardium. PGI2 did not affect the spontaneous improvement of regional myocardial blood flow, as assessed at the end of the infusion. PGI2 also failed to affect infarct size, either when expressed in relation to total left ventricular mass, or in relation to area at risk. We conclude that PGI2, when infused immediately after coronary ligation in dogs in a clinically relevant dose, neither affects regional myocardial blood flow in the ischaemic regions, nor the size of the myocardial infarction.     

锁孔择期手术治疗破裂的大脑中动脉瘤32例

目的探讨锁孔神经外科手术治疗后交通动脉瘤的手术技巧和适应证。方法回顾性分析32例破裂的大脑中动脉瘤病例,全部经DSA或CTA确诊,出血2周后行眉弓上锁孔开颅,显微神经外科手术动脉瘤夹闭。结果 32例全部夹闭,无1例死亡,治愈率100%。术后1周内头部CTA,动脉瘤消失,载瘤动脉通畅,动脉瘤夹固定良好。10例于术后3～6个月进行了随访（CTA或DSA）,未见复发。结论锁孔手术是治疗大脑中动脉瘤的微创治疗技术,减少了手术创伤及并发症,恰当地选择适应证和手术时机及应用显微外科技术是治疗的关键。