主动脉缩窄合并右弓右降及多囊状动脉瘤的影像诊断

目的 研究主动脉缩窄合并右弓右降及多囊状动脉瘤的的影像学表现。方法 回顾性分析2例主动脉缩窄合并右弓右降及多囊状动脉瘤x线胸片、超声心动图、心血管造影、三维动态增强磁共振造影(3DDCEMRA)资料并与手术病理结果对照。结果 两例均为镜面右位主动脉弓(Ⅰ型)、主动脉缩窄、主动脉弓部多囊状动脉瘤。结论 心血管造影是诊断该类复杂畸形最可靠的方法，三维动态增强磁共振造影(3DDCEMRA)在一定程度上可以替代心血管造影，能为临床手术提供明确的定位与定性诊断。     

Coexistence of a huge subaortic left ventricular aneurysm and a saccular descending aortic aneurysm

A huge subaortic left ventricular aneursym was diagnosed in an asymptomatic 22-year-old male patient. The walls of aneurysm were calcific and compressed the left atrium. Also, a saccular descending aortic aneursym and moderate mitral insufficiency associated with subaortic left ventricular aneurysm were found in the current case. Up to now, the coexistence of congenital left ventricular aneurysm and saccular aortic aneursym has not been reported in the literature. We present the first case of congenital left ventricular aneurysm combined with saccular aortic aneurysm in descending thoracic aorta in this case report.     

Multidirectional flow analysis by cardiovascular magnetic resonance in aneurysm development following repair of aortic coarctation

Aneurysm formation is a life-threatening complication after operative therapy in coarctation. The identification of patients at risk for the development of such secondary pathologies is of high interest and requires a detailed understanding of the link between vascular malformation and altered hemodynamics. The routine morphometric follow-up by magnetic resonance angiography is a well-established technique. However, the intrinsic sensitivity of magnetic resonance (MR) towards motion offers the possibility to additionally investigate hemodynamic consequences of morphological changes of the aorta.We demonstrate two cases of aneurysm formation 13 and 35 years after coarctation surgery based on a Waldhausen repair with a subclavian patch and a Vosschulte repair with a Dacron patch, respectively. Comprehensive flow visualization by cardiovascular MR (CMR) was performed using a flow-sensitive, 3-dimensional, and 3-directional time-resolved gradient echo sequence at 3T. Subsequent analysis included the calculation of a phase contrast MR angiography and color-coded streamline and particle trace 3D visualization. Additional quantitative evaluation provided regional physiological information on blood flow and derived vessel wall parameters such as wall shear stress and oscillatory shear index.The results highlight the individual 3D blood-flow patterns associated with the different vascular pathologies following repair of aortic coarctation. In addition to known factors predisposing for aneurysm formation after surgical repair of coarctation these findings indicate the importance of flow sensitive CMR to follow up hemodynamic changes with respect to the development of vascular disease.     

Bicuspid aortic valve stenosis complicated by descending aortic dissection mimicking coarctation of the aorta.

We report a rare case of bicuspid aortic stenosis complicated by an ascending aortic aneurysm and aortic dissection of DeBakey type IIIb. A 35-year-old woman was admitted to our hospital to examine her systolic murmur identified at birth. Severe aortic stenosis, dilatation of the ascending aorta, and the narrow color flow signal in the descending aorta were detected by transthoracic echocardiography. Initially, coarctation of the descending aorta was suspected, but aortic dissection, DeBakey type IIIb, was revealed by transesophageal echocardiography. Transesophageal echocardiography is indicated when only insufficient information is available on valve and aortic morphology in patients with bicuspid aortic valve.     

Endovascular stenting for aortic coarctation

Surgery had been the traditional treatment for native coarctation of the aorta, one of the most common cardiovascular congenital malformations. As a less invasive mode of treatment, balloon angioplasty has emerged as an alternative to surgery but has not gained universal acceptance due to its rates of restenosis secondary to vessel recoil and concerns over aortic wall injury resulting in aneurysm formation. To overcome these problems, endovascular stents were introduced in the management of this condition. The early- and intermediate-term results are encouraging, with low rates of restenosis and complications. In this article, the authors review the current evidence on coarctation stenting and discuss future trends in this area.     

Endovascular stenting for aortic coarctation

Surgery had been the traditional treatment for native coarctation of the aorta, one of the most common cardiovascular congenital malformations. As a less invasive mode of treatment, balloon angioplasty has emerged as an alternative to surgery but has not gained universal acceptance due to its rates of restenosis secondary to vessel recoil and concerns over aortic wall injury resulting in aneurysm formation. To overcome these problems, endovascular stents were introduced in the management of this condition. The early- and intermediate-term results are encouraging, with low rates of restenosis and complications. In this article, the authors review the current evidence on coarctation stenting and discuss future trends in this area.     

1期手术治疗主动脉缩窄合并心内畸形

目的探讨主动脉缩窄合并心内畸形的外科治疗方法。方法 2008年4月~2011年4月共手术治疗CoA合并其他心内畸形12例,其中合并VSD 2例,ASD 3例,VSD＋PDA 7例,合并主动脉弓发育不良2例。全组均行一期矫治,1例采用胸骨正中切口加左后外侧切口进行矫治,11例行胸骨正中单一切口。其中8例患者动脉缩窄段切除端端或端侧吻合,3例患者补片扩大成形,1例行人工血管植入。结果全组无手术死亡,均顺利出院。随访3~40月,术后恢复良好。超声复查无吻合口狭窄,无吻合处动脉瘤形成和主动脉瓣反流等并发症。结论主动脉缩窄合并心内畸形的外科手术策略主要根据心内畸形和缩窄病变特点决定,应视病变长短、并发症而采取不同的手术方法,并通过各种手段尽量避免复发及动脉瘤等并发症的发生。     

先天性主动脉缩窄的MRI诊断

目的：评价MRI在诊断主动脉缩窄中的作用和影像学表现。方法：主动脉缩窄患者16例，单纯型14例，复杂型2例，其中1例合并动脉导管未闭，4例合并动脉瘤，1例合并Debakey Ⅱ型主动脉夹层。16例均经手术证实。全部病例MRI检查包括SE序列、Cine MRI和三维动态增强磁共振造影(3DDCEMRA)。结果：16例MRI检查均清楚显示主动脉缩窄的部位、范围和程度，与手术所见完全一致。结论：MRI检查是诊断主动脉缩窄的可靠方法之一，可多方位、直观的显示病变部位及合并畸形，基本上可替代心血管造影，为临床手术提供明确的定位和定性诊断，MRI可作为诊断主动脉缩窄的首选方法之一。     

颅内巨大囊性动脉瘤的MRA诊断

目的 探讨MRA对颅内巨大型囊性动脉瘤的诊断价值。方法 分析8例颅内巨大型动脉瘤的MRA所见，其中5例行外科手术治疗，2例行DSA介入治疗而证实本病的诊断。结果 本组病例MRA均能显示囊状巨大瘤体，结合MRI得出本病的诊断。结论 MRA诊断颅内巨大动脉瘤简便易行，效果良好。     

产前超声诊断胎儿主动脉缩窄

目的 探讨产前超声诊断胎儿主动脉缩窄的临床价值.方法 回顾分析10例经尸体解剖证实的主动脉缩窄胎儿的超声心动图资料,总结其异常声像图特点.结果 10例胎儿主动脉缩窄中,7例主动脉弓失去正常柔和的弯曲状,呈细窄僵直状,2例足月胎儿主动脉峡部直径小于2.6mm,1例主动脉弓部血管显示不清;10例均显示右心房室增大,肺动脉及动脉导管增宽,4例动脉导管呈瘤样扩张,3例伴左心发育不良,4例伴室间隔缺损,2例合并右室双出口,1例合并二尖瓣闭锁;产前超声诊断胎儿主动脉缩窄9例,误诊主动脉弓离断1例.结论 产前超声检查对诊断胎儿主动脉缩窄,具有重要的临床价值.     

先天性主动脉缩窄的影像学表现

目的 提高对先天性主动脉缩穿表现,特别是ＭＲＩ表现的认识。方法 回顾分析２例主动脉缩窄的Ｘ线平片,主动脉造影和ＭＲＩ所见,并作文献复习,结果 Ｘ线平片和主动脉造影有特征性改变,但ＭＲＩ即能显示主动脉缩窄的部位、范围、程度及并发症,又能反应压力差的功能学变化。结论 ＭＲＩ是诊断先天性主动脉缩穿无创性最佳手段。     

Syphilitic aortitis complicated by multiple aortic aneurysms: findings of multidetector CT

We report a case of syphilitic aortitis complicated by multiple aortic aneurysms in a 50-year-old man with elevated rapid plasma reagin titer of 1:128 and positive Treponema pallidum particle agglutination test. 256-slice MDCT depicted two saccular aneurysms in the descending thoracic aorta with a markedly thick mural thrombus causing the trachea and esophagus to shift to the right. Thickening of the aortic wall was also noted. Stenting of the proximal descending thoracic aortic aneurysm and aorto-right common carotid artery bypass were performed. Operative findings revealed thickening of the descending thoracic aortic wall with a coarse luminal surface.     

超声心动图检测正常和主动脉缩窄胎儿主动脉及其意义

目的 探讨中晚孕期胎儿主动脉各段及分支内径的正常参考值范围,研究主动脉缩窄胎儿相应值的变化.方法 检测234例14~41孕周正常胎儿主动脉矢状断面上主动脉根部、升主动脉、主动脉横部、主动脉峡部、降主动脉、头臂干、左颈总动脉、左锁骨下动脉内径,并建立孕周与各项检测指标的直线相关方程.计算正常组与病例组主动脉各段与升主动脉的比值并比较.结果 主动脉各段及分支内径随孕周的增加而增加(P<0.01).主动脉缩窄组胎儿主动脉峡部/升主动脉及降主动脉/升主动脉比值与对照组比较明显降低(P<0.01).结论 监测中晚孕期胎儿主动脉峡部/升主动脉及降主动脉/升主动脉比值可作为主动脉缩窄的产前诊断筛查指标.

Abstract：

Objective To establish normal reference indexes of aorta during gestation and cut-points for detection of fetuses with coarctation of aorta.Methods From long-axis views of the aortic arch,the internal diameter of the aortic root,ascending aorta,transverse aortic arch,aortic isthmus,descending aorta,anonyma,left common carotid artery,left subclavian artery were measured in 234 normal fetuses at different time ranging from 14 to 41 weeks during gestation.Reference values of each aortic segment were constructed by linear regression analysis.The ratio of each aortic segment to the ascending aorta were calculated.ResultsThe internal diameter in each aortic segments increased as pregnancy progressed (P<0.01).In the prenatal diagnosis of fetus with coarctation of the aorta,the ratio of the aortic isthmus to the ascending aorta and descending aorta to the ascending aorta were significantly lower than the normal fetuses(all P<0.01).Conclusions The ratio of the aortic isthmus to the ascending aorta and ratio of descending aorta to the ascending aorta detected by echocardiography may be helpful in the prenatal diagnosis of coarctation of aorta.     

多排螺旋CT对儿童主动脉缩窄的诊断价值

目的 探讨多排螺旋CT以其后处理图像对儿童主动脉缩窄的诊断价值.方法 临床怀疑为主动脉缩窄的患者26例,平均年龄7.5 个月(5天～4岁),进行多排螺旋CT增强扫描.对所有病例的CT轴位图像按需要进行多平面重组及容积再现、最大密度投影.结果 26例患儿顺利完成MDCT成像检查,均确诊为主动脉缩窄,其中11例主动脉弓发育不良,CT诊断与手术结果相一致.主动脉靶重建直观地显示主动脉缩窄的部位及程度.结论 MDCT是一种有用的无创伤性检查方法,结合二维与三维图像有助于主动脉缩窄的诊断以及治疗方案的制定.     

Ruptured inferior vena cava aneurysm in the setting of mural vascular malformation: A case report

Aneurysm of the inferior vena cava is a rare anomaly with a very few reported cases worldwide. We report the case of a 26-years-old man with acute severe abdominal pain and hypovolemic shock following an episode of syncope. Ultrasonography showed a fusiform aneurysmal dilation of the infra-hepatic inferior vena cava (IVC), with a large saccular portion at its posterolateral wall and mural thrombosis. Abdominal computed tomography scan revealed extension to the right renal vein and adhesion to the right kidney. The saccular aneurysm and the right kidney were resected, and anatomopathological examination revealed a cavernous hemangioma. All symptoms disappeared after surgery. This is the first reported case of symptomatic congenital saccular aneurysm of the IVC due to mural vascular malformation and with involvement of the right kidney leading to nephrectomy.     

Vascular remodeling in adults after coarctation repair: impact of descending aorta stenosis and age at surgery

Background  

Patients after successful repair of coarctation of aorta (CoAo) are at risk of hypertension at rest and associated end-organ damage. The aim of the study was to assess arterial stiffness and function in adults after coarctation repair in relation to descending aorta (AoD) residual coarctation and patient’s age at operation.     

A case with coarctation of the aorta accompanied by coronary ectasia and dilatation of the ascending aorta

Coarctation of the aorta is one of the congenital heart diseases diagnosed primarily in childhood and early adolescence. A 67-year-old female was admitted to the hospital with chest pain. Angiography revealed a coarctation of the aorta, coronary ectasia and dilatation of the ascending aorta. This case attracted our attention because the first diagnosis was made at such an advanced age and coarctation was accompanied by coronary ectasia and dilatation of the ascending aorta. Therefore, we decided to report the case.     

Thoracoabdominal aorta coarctation with bilateral renal artery involvement: diagnosis with multidetector CT angiography (MDCTA)

Atypical coarctation of the descending thoracic and abdominal aorta is a very uncommon vascular disease. Congenital, acquired, inflammatory, and infectious etiologies have been proposed. Patients typically presents with uncontrolled secondary hypertension in the upper half of the body or hypotension in the lower extremities in the first three decades of their lives. We report the case of a 20-year-old man with severe hypertension. Diffuse coarctation of thoracoabdominal aorta associated with bilateral renal artery stenosis was demonstrated clearly by multidetector CT angiography. This is the first case of atypical aortic coarctation diagnosed by MDCTA.     

Aortic valve aneurysm: a novel cardiac manifestation of rheumatoid arthritis?

This report describes a 51-year-old man with rheumatoid arthritis, coronary artery disease, left ventricular failure, and saccular aneurysm of the left coronary cusp of the aortic valve. Rheumatoid arthritis can involve the heart in several ways; however, aortic valve aneurysm in rheumatoid arthritis has not been reported before. Absence of vegetation, lack of a history of infective endocarditis, and the preservation of aortic valve function suggested that this saccular aneurysm was not a result of infective endocarditis. An intimal flap, which strongly suggests aortic dissection, was not observed. To our knowledge, this may be a novel cardiac manifestation of rheumatoid arthritis, and this is the first published report.     

Sonographic sign suggesting the prenatal diagnosis of coarctation of the aorta

Coarctation of the aorta is a serious heart defect that can be successfully treated if identified early. Actual narrowing of the aortic isthmus is very difficult if not impossible to demonstrate on prenatal sonography; however, the indirect sign of discrepant ventricular sizes (right ventricle [RV] greater than left ventricle [LV]) is potentially useful to identify fetuses at risk for having coarctation of the aorta. We report on nine fetuses, 18 to 38 weeks gestation, in which the left ventricle was smaller than the right ventricle. After birth, four of the nine had coarctation of the aorta. One infant with Down's syndrome had a patent ductus arteriosus as well as foramen ovale. Another infant had a small left ventricle and parachute mitral valve but no coarctation. The last three infants had a normal cardiac workup at birth. We conclude that a small left ventricle compared to the right ventricle on prenatal sonography can be a sign of congenital heart disease and that one of the defects that can give this appearance is aortic coarctation. Careful neonatal follow-up is warranted.