Persistent fifth aortic arch diagnosed by echocardiography and confirmed by angiography: Case report and literature review

Persistent fifth aortic arch is a rare congenital anomaly that can be discovered incidentally or at postmortem exam. It can be associated with major congenital heart malformations involving the systemic or the pulmonary circuits. It usually has no clinical significance but can be either, beneficial as in systemic outflow tract obstructions or cause hemodynamic compromise when associated with a significant left to right shunt. We report an infant with persistent fifth aortic arch associated with Shone’s complex diagnosed accurately by transthoracic echocardiography and confirmed by cardiac catheterization and computed tomography.     

胸主动脉腔内治疗相关的主动脉弓形态研究

目的 通过对主动脉弓CTA资料的回顾性研究,了解正常主动脉弓的解剖形态学特点,为腔内治疗技术以及人工血管和新型支架的设计建立解剖形态学基础。方法 2009年1月至2012年10月年符合入组标准的正常型主动脉弓CTA资料共217例,使用GE公司AW4．5工作站测量升主、弓上分支的直径及分支之间距离,运用统计学软件进行分析。结果 冠状动脉开口上2cm处主动脉直径：34.56±4.72 mm,分支动脉开口直径：无名动脉12.50±1.95 mm,左颈总动脉8.28±1.51 mm,左锁骨下动脉10.41±2.20 mm。主动脉弓横断面上无名动脉与左锁骨下动脉连线与矢状面的成角50.30±13.79°。无名动脉-左颈总动脉距离为4.39±2.49 mm,左颈总动脉-左锁骨下动脉距离为6.43±3.98 mm。主动脉直径在不同年龄中的差异有统计学意义,61岁以上年龄组主动脉直径明显大于18-40岁和41-60岁年龄组。结论 1.在主动脉进行腔内修复术时,应充分考虑主动脉直径在61岁以上有明显增大趋势来进行支架的选择。2.由于近端锚定区距离过小,涉及左锁骨下动脉的主动脉病变大部分是不可能通过简单地扩展胸主动脉腔内支架来成功处理。     

Complex atheromatosis of the aortic arch in cerebral infarction

In many stroke patients it is not possible to establish the etiology of stroke. However, in the last two decades, the use of transesophageal echocardiography in patients with stroke of uncertain etiology reveals atherosclerotic plaques in the aortic arch, which often protrude into the lumen and have mobile components in a high percentage of cases. Several autopsy series and retrospective studies of cases and controls have shown an association between aortic arch atheroma and arterial embolism, which was later confirmed by prospectively designed studies. The association with ischemic stroke was particularly strong when atheromas were located proximal to the ostium of the left subclavian artery, when the plaque was ≥ 4 mm thick and particularly when mobile components are present. In these cases, aspirin might not prevent adequately new arterial ischemic events especially stroke. Here we review the evidence of aortic arch atheroma as an independent risk factor for stroke and arterial embolism, including clinical and pathological data on atherosclerosis of the thoracic aorta as an embolic source. In addition, the impact of complex plaques (≥ 4 mm thick, or with mobile components) on increasing the risk of stroke is also reviewed. In non-randomized retrospective studies anticoagulation was superior to antiplatelet therapy in patients with stroke and aortic arch plaques with mobile components. In a retrospective case-control study, statins significantly reduced the relative risk of new vascular events. However, given the limited data available and its retrospective nature, randomized prospective studies are needed to establish the optimal secondary prevention therapeutic regimens in these high risk patients.     

Endovascular coil embolization of a complex aortic arch pseudoaneurysm following arch stenting

Pseudoaneurysm formation is a life‐threatening complication of thoracic aortic stenting due to the high risk of rupture. When located in the aortic arch, anatomic features may pose difficulties in choosing the optimal treatment strategy. Here, we describe the first poststenting aortic arch pseudoaneurysm treated by endovascular coil embolization. This approach, which we performed in a multidisciplinary setting, may be a feasible alternative in patients not considered suitable for open repair or stent‐grafting. As an acute pseudoaneurysm may develop and rapidly expand during the first days after aortic stenting, early follow‐up imaging is preferable.     

3-D echocardiographic imaging of double aortic arch.

Delineation of arch abnormalities is difficult by conventional 2-D echocardiography and MRI has been the investigation of choice. 3-D echocardiography is increasingly used in congenital heart disease for both functional anatomy and morphology. This case report demonstrates that 3-D echocardiography can be used in delineating arch anomalies, which can avoid further imaging that needs a general anaesthetic.     

Safety and durability of single-stage type I hybrid total aortic arch repair for extensive aortic arch disease: early- and long-term clinical outcomes from a single center and our 10-year of experience

BackgroundSingle-stage type I hybrid total aortic arch repair is a surgical treatment for extensive aortic arch disease, but the clinical outcomes were distinguishing. The purposes of this study were to share our experience and evaluate the perioperative safety and long-term durability.MethodsThirty-six patients who underwent single-stage type I hybrid total aortic arch repair in Fuwai Hospital between January 2010 and June 2020 were respectively reviewed. Early primary endpoint was defined as early composite adverse events, including mortality, multiple organ dysfunction syndrome (MODS), unplanned reoperation, stroke, paraplegia, acute renal failure (ARF) necessitating continuous renal replacement therapy (CRRT), respiratory failure and stents related complications. Long-term endpoints included late mortality, late aortic related reintervention and late adverse aortic events. When evaluating the early- and long-term outcomes, all patients were stratified into two subgroups by age (65 years).ResultsAll patients acquired technical success. Early composite adverse events rate was 11.1% (4/36), in-hospital mortality was 8.3% (3/36). Average follow-up period was 48.0±35.3 months. Overall survival rate was 83.3% and 51.9% at 5 and 10 years respectively. Late aortic related reintervention occurred at one (3.0%, 1/33) patient and this patient died after reintervention. Overall freedom from adverse aortic events was 79.2% and 47.5% at 5 and 10 years respectively. Significant difference was not observed between the elderly and young subgroups, no matter in early- and long-term outcomes.ConclusionsSingle-stage type I hybrid total aortic arch repair has achieved desirable outcomes in our center, which does not increase perioperative risk in the elderly patients, meanwhile, also acquire acceptable durability in the young patients. In conclusion, this surgery is a practical mini-invasive treatment for extensive aortic arch disease with strict and limited indications.     

复合手术治疗老年主动脉弓部疾病

目的探讨“复合”手术治疗老年主动脉弓部病变患者的临床经验及早期随访结果。方法2009年2月至2012年2月,11例主动脉弓部疾病、无法耐受开胸体外循环／深低温停循环下外科手术而接受复合手术的老年患者,其中包括主动脉瘤6例、主动脉溃疡3例和Stanford B型夹层2例。男8例,女3例;平均年龄70岁。患者先行右腋动脉至左颈总动脉和左腋动脉转流手术,然后同期进行主动脉覆膜支架腔内隔绝术覆盖左颈总动脉及左锁骨下动脉。于术后第3、6、12个月及每年进行CT血管造影或MRI复查。结果手术成功率为100％。1例于术后8天死亡、脑卒中发生率为0．0％、截瘫发生率为0．0％,仅有2例术后早期出现少量Ⅰ型内漏。随访时间（15±10）个月,无新的死亡病例发生,无迟发主动脉相关事件,发生Ⅰ型内漏者分别于术后3个月及6个月内漏消失。结论复合手术治疗老年患者主动脉弓部疾病的近期结果满意。     

Interrupted aortic arch in an adult female

Interrupted aortic arch is a rare and usually lethal malformation, representing approximately 1% of congenital heart disease. This presents as a missing segment of the aortic arch and is divided into three types: A-called extreme form of coarctation, and is characterized by disruption of aorta's continuity distal to the left subclavian artery (30-40%), B-disruption between the left subclavian and the left carotid arteries (55-60%), and C-the most uncommon type, interruption proximal to the left common carotid artery. The suspicion of coarctation of the aorta can be made from a combination of physical findings including systolic ejection murmur, the murmurs of collateral blood vessels, diminished or absent femoral pulse, and difference in blood pressure between arms and legs. Interrupted aortic arch is an extremely rare anomaly in adult patients. To our knowledge, the world medical literature contains only about 13 reports of interrupted aortic arch diagnosed in adults.     

Taussig-Bing畸形合并主动脉弓病变的外科治疗

目的：总结Taussig-Bing畸形合并主动脉弓病变的手术治疗经验。方法：2012年3月至2017年5月手术治疗5例Taussig-Bing畸形合并主动脉弓病变的患儿,其中主动脉弓中断（Interrupted aortic arch,IAA）3例,主动脉缩窄（Coarctation of aorta,COA）2例。3例患儿采用一期大动脉调转术(Arterial switch operation,ASO)+IAA矫治术（降主动脉与主动脉弓端侧吻合）;1例患儿分期手术（一期行COA矫治术,二期行ASO手术）;1例患儿行ASO手术+COA矫治术。结果：全组患儿均顺利出院。气管插管时间平均（158.20±55.64）h;ICU停留时间平均（13.20±3.42）d;住院时间平均（42.00±11.04）d;术后早期并发症包括低心排血量综合征1例、术后出血1例、少尿和腹膜透析1例、肺不张1例。术后随访患儿无因残余解剖问题,无再次手术者。结论：Taussig-Bing畸形合并主动脉弓病变病理解剖比较复杂,手术方式与合并主动脉弓病变畸形的类型对治疗效果有影响,合并IAA患儿应一期矫治,合并COA的个别患儿可选择二期手术矫治。     

Balanced double aortic arch with tetralogy of Fallot

We present a rare case of balanced double aortic arch in a 19 year old cyanotic boy, a known case of tetralogy of Fallot. This was suspected on chest X-ray and confirmed on further imaging by cardiac catheterization and computed tomography. We discuss the clinical and surgical implications of this condition.     

Changes in aortic arch geometry and the risk for Stanford B dissection

BackgroundThe increase in aortic diameter is not closely associated with type B aortic dissection (TBAD); morphological risk factors other than aortic diameter may help to better identify patients at risk for TBAD. The purpose of this study was to investigate possible morphological factors associated with the occurrence of TBAD.MethodsThis study was a retrospective, multicenter, cross-sectional study. We collected 94 patients with TBAD as the TBAD group and 534 patients with healthy aortas as the healthy control group. Morphometric data were collected on three-dimensional models of the thoracic aorta. A propensity score matching (PSM) analysis was conducted to reduce the potential for confounding by baseline factors.ResultsThe number of patients in the TBAD group was 75 after PSM. Longer lengths of the aortic arch (28.00±7.42 vs. 25.14±7.11 cm) were observed in patients with TBAD. The width (80.04±17.27 vs. 71.73±15.55 mm) and height (24.92±11.39 vs. 19.37±10.10 mm) of the aortic arch in patients with TBAD were both larger than those of healthy controls. The morphological changes associated with the occurrence of type B acute dissection were most pronounced in the geometry of the aortic arch.ConclusionsThis study demonstrates that TBAD was associated with longer lengths of aortic arch and with larger arch height and width.     

先天性双主动脉弓畸形的外科诊治

目的 总结先天性双主动脉弓畸形患儿的临床特点和手术治疗经验。方法 回顾性分析2013年3月-2015年8月,共5例双主动脉弓患儿临床资料。5例患儿均为男性。年龄7 -18月,均数(11.60±4.21)月;体质量8-9.5Kg,均数(8.50±0.50)Kg。左右弓均衡型1例,右弓优势型4例。临床表现为气喘、喉鸣、吞咽困难。5例患儿手术经左后外侧切口进胸,切断左弓,同时切断动脉导管或动脉韧带,松解气管、食管周围组织,解除对气管、食管的压迫。 结果 5例患儿均顺利完成手术,未出现与手术相关并发症,无围术期死亡病例。呼吸机使用时间2-4h,均数(3.30±0.83)h,住院时间9-20天,均数(12.40±4.39)天。5例患儿均得到随访,1例患儿术后半年行肺动脉闭锁、室间隔缺损、房间隔缺损根治手术,术后痊愈出院。1例患儿术后半年行双侧隐睾下降固定术。其余患儿临床症状均缓解,生长发育、活动量较正常儿童无明显差异。结论 DAA是一种少见的心血管畸形,易引起气喘、喉鸣、吞咽困难等症状,心脏大血管CT检查可确诊,早发现、早期手术治疗效果良好。     

主动脉弓离断的临床X线表现（附7例分析）

目的探讨先天性主动脉了断（ＩＡＡ）的临床Ｘ线特点及Ｘ线平片诊断价值。材料和方法回顾性分析７例（男３例,女４例,年龄３０天～１１岁,平均４岁）经心血管造影（ＡＣＧ）和（或）手术证实的ＩＡＡ患儿的临床、Ｘ线和ＡＣＧ表现及相互关系。结果７例全属Ａ１型,无Ｂ、Ｃ型,均合并心内畸形,术前Ｘ线平片符合诊断３例。主要的Ｘ线征象有：①气管居中（７例）;②主动脉结消失（７例）;③主动脉弓低位征（２例）;④升主动脉发     

Right aortic arch with situs solitus

Patients : We reviewed all patients evaluated at our center with situs solitus, levocardia, and a right aortic arch that were born between January 2000 and January 2018. Results : From our databases, we identified 204 patients. We excluded patients with a double aortic arch from analysis. Of the 204 patients, 103 (50%) were male. Of the 204 patients, 95 (47%) had an isolated right aortic arch. Of the 95 with an isolated right aortic arch, 4 (4%) had chromosome 22q11.2 deletion syndrome, and 89 (94%) had a vascular ring. Of the 204 patients, 109 (53%) had a surgical intracardiac malformation. Of the 109, with an associated surgical intracardiac malformation, 38 (35%) had chromosome 22q11.2 deletion syndrome, and 28 (26%) had a vascular ring. Overall, of the 204 patients, 200 (98%) had a vascular ring, an associated intracardiac malformation, or both. Prenatal detection was 85% (40/47) for the subset of patients born in Southern Nevada between January 2015 and January 2018. Conclusion : this review, to best of our knowledge, reports one of largest series of right aortic arches in the literature. Data from our cohort demonstrates that a right aortic arch in situs solitus is almost always associated with pathology. Further, our center's right aortic arch prenatal detection rate exceeds previous reports.     

Relapsing polychondritis with aortic arch aneurysm and aortic arch syndrome

Summary A case of relapsing polychondritis with aortic arch aneurysm and aortic arch syndrome is described. The pattern of vascular involvement supports the inclusion of relapsing polychondritis within the spectrum of systemic vasculitides.     

Percutaneous occlusion of posttraumatic aortic arch pseudoaneurysm by catheter-based delivery of thrombin.

PURPOSE: To report a patient in whom a posttraumatic pseudoaneurysm of the aortic arch was successfully treated by catheter-based delivery of thrombin with balloon occlusion of the entry tear. CASE REPORT: A 63-year-old male presented with pseudoaneurysm of the aortic arch after blunt chest trauma which was detected incidentally during routine chest X-ray. Using a percutaneous transfemoral approach, a 5 French Swan-Ganz catheter could be positioned into the aneurysm sac. Under balloon occlusion of the entry tear in order to prevent systemic embolization, 1,000 units of bovine thrombin were injected into the aneurysm sac resulting in complete occlusion of the aneurysm. Contrast-enhanced computed tomographic control showed complete thrombosis of the aneurysm over a follow-up period of 12 months. CONCLUSION: Percutaneous catheter-based delivery of thrombin is a feasible and effective minimally-invasive treatment alternative for patients with thoracic aortic pseudoaneurysms.     

Successful management of esophageal perforation due to an aortic arch aneurysm replacement

SUMMARY.  We present the successful management of an esophageal perforation after aortic arch aneurysm replacement in a 64-year-old patient. Four weeks after surgical repair of a perforated aortic arch aneurysm, a contained perforation of the thoracic esophagus on the prosthesis was detected. A subtotal esophagectomy and reconstruction by pull-up of the stomach together with the greater omentum and high intrathoracic esophagogastrostomy was performed. The aortic prosthesis was covered by omentum. After a prolonged postoperative course, the patient was discharged from the hospital on a full oral diet. She is well after 1 year without signs of infection.     

Double aortic arch and d-transposition of the great arteries

We present a rare case of D-transposition of the great arteries (D-TGA) and double aortic arch (DAA). The anatomy was prospectively and preoperatively diagnosed by echocardiography and confirmed by ultra-fast computed tomography. The patient underwent successful arterial switch operation and division of the vascular ring at a single procedure.