Type B Interrupted Left Aortic Arch with Isolated Right Subclavian Artery

Interrupted aortic arch is a rare congenital heart malformation occurring in approximately three per 1 million births. Type B interrupted aortic arch (interruption between the second carotid artery and the ipsilateral subclavian artery) is the most common of three major types (A, B, and C). We report an extremely rare finding: a case of left‐sided type B interrupted aortic arch with isolation of the right subclavian artery (origin from the right pulmonary artery).     

Bovine Aortic Arch in Association with Double Inlet Left Ventricle and Pulmonary Atresia: A Novel Association

We report a newborn male who was diagnosed with a double inlet left ventricle with pulmonary atresia antenatally. Postnatally, it was difficult to determine his arch anatomy echocardiographically. Therefore, he underwent three‐dimensional computed tomography angiography, which confirmed the echocardiographic findings and demonstrated a bovine aortic arch. He additionally had a single coronary artery. To our knowledge, the association of a single ventricle with a single outlet of bovine morphology is novel.     

Interrupted Aortic Arch with Anomalous Origin of Left Pulmonary Artery from Aorta

We report a case of interrupted aortic arch and origin of the left pulmonary artery from the aorta with bilateral ductus arteriosus and discuss its possible embryological basis. To the best of our knowledge, this combination of anomalies has not been reported in the English medical literature.     

Right Aortic Coarctation and Ventricular Septal Defect: An Unusual Cause of Tracheal Compression in Infancy

Objective. Acyanotic congenital heart diseases may occasionally present with tracheobronchial obstruction. Increased pulmonary blood flow against a high‐resistance pulmonary bed may create significant pulmonary artery dilation. Methods. We report an unusual case of ventricular septal defect and right aortic arch coarctation, complicated with distal tracheal compression secondary to a pincer effect created by a right aortic arch and a massively dilated pulmonary artery. Results. High index of suspicion is required to anticipate tracheobronchial compression in acyanotic congenital heart diseases. Conclusion. Fiberoptic bronchoscopy is an invaluable tool to assess for tracheal compression relief following surgical repair and to identify tracheomalacia prior to extubation.     

Adult Presentation with Vascular Ring due to Double Aortic Arch

This is a case report on the use of cardiovascular magnetic resonance imaging to diagnose vascular ring due to double aortic arch in an adult presenting with an abnormal chest X‐ray. The experience in this case and the literature review identify the benefits of using cardiovascular magnetic resonance imaging to clarify complex aortic arch anatomy.     

Double Aortic Arch with Dominant Left Arch and Right Ligamentum Arteriosum

This case report describes a rare example of double aortic arch with a dominant left aortic arch, patent minor right aortic arch, left descending aorta, and right ligamentum arteriosum causing tracheobronchial compression in a twin baby girl with DiGeorge syndrome. She also had large right subclavian artery arising from right‐sided diverticulum of Kommerell, aplastic thymus, T cell lymphopenia with normal immunoglobulin, hypocalcemia, and hypomagnesemia. The diverticulum of Kommerell was resected and minor right aortic arch, right ligamentum arteriosum, and right subclavian artery were divided through right posterolateral thoracotomy. Aortopexy was performed under bronchoscopic guidance to relieve the airway compression. We strongly suggest a right‐sided approach in this type of vascular arrangement for easy access and better outcome.     

Adult Presentation of Interrupted Aortic Arch: Case Presentation and a Review of the Medical Literature

Interrupted aortic arch (IAA) is a rare congenital abnormality that presents in infancy. Isolated cases have been reported in adulthood, which are likely presentations of severe coarctation of the aorta. We present two such cases of adult presentation of IAA and a review of previously reported cases in the medical literature and current recommendations for management and follow‐up of these patients.     

A Late Presentation of Tetralogy of Fallot with Pulmonary Atresia and Coronary‐Pulmonary Artery Collateral and a Congenital Aortic Stenosis

We report a 40‐year‐old man with an unoperated tetralogy of Fallot with pulmonary atresia and congenital aortic valvular stenosis. A well‐developed coronary‐pulmonary arterial collateral arising from the left main coronary artery was also demonstrated. Although rare cases of tetralogy of Fallot with congenital aortic stenosis has been reported, this is the first particular case demonstrating a triad of these abnormalities.     

Silent Double Aortic Arch Coincidentally Found During Cardiac Catheterization in Elderly Man

We describe a case of an elderly man known for coronary artery disease (previous bypass surgery) hospitalized for ischemia in inferior wall. Since the operation, the patient underwent coronary angiographies. Due to the impossibility of selective engagement of left subclavian artery from femoral access, LIMA was always visualized through a right radial approach. Despite the suspicious of abnormal origin of left subclavian artery, aortic angiography was never performed. During the third angiography, the double aortic arch was coincidentally visualized by using a left Amplatz catheter. The diagnosis was confirmed by 64‐slice computed tomography scan.     

Right Aortic Arch and Coarctation: A Rare Association

Objectives. Understand anatomical and clinical correlatives to coarctation in right aortic arch. Background. Coarctation of the aorta is rare in patients with a functional right aortic arch. We reviewed a single institutional experience, examining associated diagnoses, diagnostic methodology, and surgical approaches. Methods. A retrospective study was performed of our echocardiographic, magnetic resonance imaging, catheterization, and surgical databases from 1988 to 2001. Results. Of 240 patients with right aortic arch, 10 (4.1%) had coarctation, constituting 1.9% of all native coarctations (n = 524). Nine (90%) had long‐segment hypoplasia. Six (60%) had an aberrant left subclavian artery or retroesophageal diverticulum, 3 (30%) had mirror image branching, and 1 (10%) had a double arch with an atretic left arch. Other congenital heart defects were seen in 6 (60%) comprising 3 with ventricular septal defects, and one each with double‐outlet right ventricle, cor triatriatum, and pulmonary valve abnormality. No patients with long‐segment hypoplasia had bicuspid aortic valve. Six (60%) had vascular rings, and 5 (50%) had other associated syndromes. Magnetic resonance imaging and/or echocardiography successfully diagnosed all of these patients. Although long‐segment right aortic arch coarctation courses behind the trachea posteriorly, only 2 needed an extra‐anatomic (jump) graft; the remainders were repaired with patch angioplasty. Conclusion. Coarctation with right aortic arch is rare, constituting 4.1% of all patients with right aortic arch, compared with 5–8% of patients with left aortic arch and congenital heart disease. Nearly all had long‐segment hypoplasia without bicuspid aortic valve, and half were part of other syndrome complexes. This association can be diagnosed noninvasively and can often be repaired by patch angioplasty.     

Persistent Fifth Aortic Arch Associated with Coarctation of Aorta: A Case Report

A 20‐year‐old man with persistent fifth aortic arch associated with coarctation of the aorta underwent catheterization and stenting of coarctation of the aorta.     

Aortic Arch Morphology and Late Systemic Hypertension Following Correction of Coarctation of Aorta

Objective. To reproduce in an adult population a pediatric study that found an association between aortic arch geometry and late systemic hypertension following successful repair of aortic coarctation. Design and Results. Fifty‐one patients with successful repair of coarctation of the aorta had blood pressure measurement at rest and during exercise. After cross‐sectional imaging of the aortic arch, patients were assigned to 1 of 3 previously defined morphological categories: normal, gothic, or crenel. The degree of residual stenosis and the ratio of the height/transverse diameter of the arch (A/T ratio) were calculated. No relationship was found between arch geometry and either resting‐ or exercise‐induced hypertension. Conclusions. We found the classification into 3 morphological types difficult and did not find an association between gothic arch or a high A/T ratio and hypertension.     

Right Ventricular Endocarditis in a Pregnant Woman with a Restrictive Ventricular Septal Defect

A 22‐year‐old woman with a restrictive unoperated perimembranous ventricular septal defect was diagnosed with staphylococcal endocarditis during her 14th week of pregnancy. Echocardiography revealed a long, thin, and mobile vegetation along the right ventricular free wall that increased to 8 cm in length, with systolic protrusion across the pulmonary valve. The vegetation subsequently embolized, resulting in a pulmonary abscess. She responded favorably to intravenous antibiotic therapy maintained for a total of 6 weeks, with resolution of the intracardiac mass and pulmonary abscess. The remaining peripartum and postpartum course was relatively unremarkable. Percutaneous closure of the ventricular septal defect was successfully performed postpartum.     

Successful Percutaneous Closure of a Secundum Atrial Septal Defect through Femoral Approach in a Patient with Interrupted Inferior Vena Cava

Percutaneous closure of secundum atrial septal defect (ASD II) is considered the treatment of choice in the majority of cases. Interrupted inferior vena cava with azygos continuation can make delivery of the occluder difficult or not possible. Transjugular, transhepatic approach or surgery can be the alternative. We present the case of a 53-year-old woman with ASD II and interrupted inferior vena cava, and describe successful atrial septal defect closure under transesophageal echocardiography guidance through transfemoral approach using a modification of the standard technique.     

中度低温停循环结合选择性脑灌注在婴儿主动脉弓部手术中的应用

目的 总结中度低温停循环(moderate hypothermia circulatory arrest,MHCA)结合选择性顺行脑灌注(sective antegrade cerebral perfusion,SACP)技术在婴儿主动脉弓重建手术中的应用经验.方法 回顾性分析上海市儿童医院心胸外科于2012年1月至2018年12月间完成的主动脉弓病变合并心内畸形矫正的患儿50例.依据中心温度将患儿分为深低温停循环(deep hypothermia circulatory arrest,DHCA)组及MHCA组,每组25例.所有患儿均在体外循环(cardiopulmonary bypass,CPB)下行一期手术治疗.主动脉弓重建过程中采用低温停循环技术,通过无名动脉SACP(25~40 ml·kg^-1·min^-1)的CPB管理方法.心肌保护采用康斯特器官保护液(HTK液).记录两组患者的一般资料及术中、术后指标.结果 两组均无与CPB相关的神经系统并发症.两组患儿年龄、体重、病种、术前肝肾功能及术前左心室射血分数值比较差异均无统计学意义(P>0.05).CPB时间MHCA组较DHCA组明显缩短[(120.00±22.60)min比(137.40±22.88)min,P=0.019];术后24 h胸腔引流量及正性肌力药物评分MHCA组较DHCA组明显减少,分别为[(49.84±20.66)ml比(78.20±52.31)ml,P=0.03;(9.72±2.47)分比(12.24±3.07)分,P=0.004].结论 在婴儿主动脉弓重建手术中,采用DHCA或MHCA结合SACP的技术均能均减少术后神经系统并发症发生,不增加术后其他并发症.MHCA可减少CPB时间、术后胸腔引流量及术后血管活性药物的使用量.     

Ductal Arteriosus Aneurysm,Right Aortic Arch,and Isolated Left Subclavian Artery in a Neonate

Ductal arteriosus aneurysm (DAA) is a well‐recognized condition, especially in infancy, and is usually asymptomatic. We report the first case of a newborn who presented with significant inspiratory stridor and, using multiple imaging investigations, was subsequently diagnosed with the rare constellation of a congenital DAA, a right aortic arch and an isolated left subclavian artery with normal intra‐cardiac anatomy. The patient underwent surgical resection of the DAA with significant improvement in symptoms.     

Severe Tricuspid Stenosis during Percutaneous Occlusion of Perimembranous Ventricular Septal Defect with the New Amplatzer Device

Background. The new Amplatzer device has been used with success for occlusion of the perimembranous ventricular septal defects and the index of complications is very low. Results. The reported case corresponds to a very rare severe acute tricuspid stenosis, caused by adherence of the prosthesis to the anterior tricuspid leaflet. Conclusion. Device release was achieved by manipulation on the tricuspid apparatus.