Right aortic arch with left descending aorta (circumflex aorta) Roentgenographic diagnosis

Right aortic arch with retroesophageal segment is a rare anomaly with distinctive roentgenographic features. We present 2 patients, who on the barium oesophogram had an additional finding which has not previously been described. The typical features include an indentation on the right upper barium filled oesophagus (in the antero-posterior view), an oblique indentation descending from right to left (caused by the retroesophageal segment) and the left descending aorta. The oblique indentation has not previously been described. The embryology and incidence of the lesion are briefly discussed.     

Complete thrombotic obliteration of the ascending aorta and the aortic arch as a cause of acute heart failure in a newborn

Thrombosis of the great vessels, and especially of the aorta, is rare in neonates. We report a case with thrombosis of the ascending aorta, aortic arch, brachiocephalic trunk and subclavian artery. Clinically, severe heart failure occurred on the 1st day of life and the diagnosis was confirmed by echocardiography and cardiac catheterization, including angiocardiography. Left ventricular function was found to be extremely depressed. An infusion with prostaglandin E₁ was initiated in order to improve the systemic circulation by dilating the arterial duct. The infant died of neurological complications prior to surgery.Research fellow from Medical Academy, Sofia, Bulgaria, supported by A. v. Humboldt Stiftung     

Interrupted aortic arch: Natural history and operative results

Summary The chances of survival for patients with interruption of the aortic arch depend mainly on early recognition and medical treatment before symptoms of cardiogenic shock appear, and specifically on early operative repair after the exact diagnosis has been established. Accurate angiography with visualization of the interrupted aortic arch and delineation of associated cardiac malformations is of prime importance for the surgical management and prognosis. Among our 36 patients with interrupted aortic arch, 27 were operated on. The operative mortality in 22 patients with associated ventricular septal defect (VSD) and persistent ductus arteriosus (PDA) was reduced from 43% (in seven patients seen up until 1979) to 7% (in 15 patients seen since 1980). One patient with interrupted aortic arch type C and another infant with associated truncus arteriosus were successfully corrected on day 9 and day 17 of life, respectively. In the patients with associated VSD and PDA as well as in the one patient with associated truncus arteriosus, the primary correction—direct anastomosis of the interrupted segments without prosthesis and VSD closure and in the case with truncus, the additional positioning of a valve-bearing allograft conduit from the right ventricle to the pulmonary artery—has been more successful than a two-step approach with initial pulmonary artery banding.     

Single arterial trunk arising from the aortic arch associated with coarctation of the aorta

A 6-day-old baby with a single arterial trunk arising from the aortic arch is reported. The baby had coarctation of aorta with patent ductus arteriosus and ostium primum atrial septal defect. A review of the literature in English reveals no other case reported of an infant with this association of anomlies.     

Anomalous origin of right pulmonary artery from ascending aorta with right ventricular endocardial fibroelastosis

Summary A case of aortic origin of the right pulmonary artery with right ventricular endocardial fibroelastosis is reported. Its diagnostic features, surgical aspects, and postmortem findings are discussed. This is a rare combination. The relevant literature is reviewed.     

小儿主动脉弓发育不良的手术及灌注技术进展

目前,小儿主动脉弓发育不良的定义存在争议,主要的评价方法有:①近弓、远弓和峡部分别＜升主动脉的60％、50％和40％、或者横弓直径＜升主动脉远端的50％、或者横弓直径＜降主动脉直径的50％;②经验法则:横弓直径(mm)＜体重(kg)+1;③Z值:不同节段的大小低于正常平均值的两个标准差以下,即Z值＜-2.小儿主动脉弓发育不良病变谱广,不同患儿解剖和生理特点差异大,且常合并各类心内畸形,小儿主动脉弓发育不良术后近远期并发症发病率、再干预率、病死率的控制仍不满意.小儿主动脉弓发育不良的治疗也存在争议,存在多种手术和灌注方法.目前较多使用的手术方法有:扩大端端吻合术,左锁骨下动脉瓣翻转术,端侧吻合术,补片扩大术等.使用较多的灌注方法有:深低温停循环技术和选择性脑灌注技术等.因此选择合理有效的手术和灌注方法,能提高主动脉弓发育不良患儿近远期的治疗效果.根据不同主动脉弓发育不良患儿的情况进行个体化治疗是现在小儿主动脉弓发育不良外科治疗的趋势.现对小儿主动脉弓发育不良的手术及灌注技术进展做一综述.     

主动脉弓病变43例术后早期结果分析

目的 探讨主动脉弓病变手术术后早期临床结果,以提高手术成功率、降低病死率和缩短ICU治疗时间.方法 回顾性分析2012年1月至9月在阜外心血管病医院连续完成的43例主动脉弓病变手术患儿术后早期临床资料.男26例,女17例;年龄29 d ～ 10岁,中位年龄6个月;体质量3.2 ～38.0 kg,中位体质量6.0 kg.9例为单纯主动脉弓病变或仅合并单一心内畸形,34例为合并2种或2种以上复杂心内畸形.分别按年龄及ICU滞留时间进行分组研究.结果 术后呼吸机辅助中位数时间为23 h,ICU治疗中位数时间为3d;死亡3例.≤6个月小婴儿术后呼吸机辅助和ICU治疗时间明显长于＞6个月婴幼儿.单因素分析结果认为年龄小,体质量低,合并复杂畸形,阻断时间、转机时间及呼吸机辅助时间长是术后患儿滞留ICU的危险因素.15例患儿发生术后并发症,其中肺部感染12例,渗漏综合征5例,气管狭窄2例,低心排综合征2例.低心排综合征经体外膜肺氧合辅助和心肺复苏术抢救成功.结论 主动脉弓病变手术效果确切,术后病情复杂.年龄≤6个月的危重主动脉弓病变合并复杂心内畸形的患儿亟待手术,但手术有风险,术后ICU滞留及并发症发生率高.相对而言,年龄＞6个月的主动脉弓病变患儿手术及术后更加安全.     

Right aortic arch with aberrant left innominate artery

A 9-year-old girl with type D right aortic arch anomaly, has been presented. The diagnosis was based upon the presence of signs and symptoms of a vascular ring and the early visualization of a retroesophageal aberrant left innominate artery, by means of countercurrent right brachial angiography. Divison of the ligamentum arteriosum at operation relieved the symptoms.     

Aortic atresia with aortopulmonary window and interruption of the aortic arch

Summary The cross-sectional echocardiographic and postmortem appearances of the heat from a patient with the rare association of aortic valve atresia, aortopulmonary window, and interrupted aortic arch are described. Differentiation of this anomaly from truncus arteriosus with interrupted aortic arch is important.     

Percutaneous balloon aortoplasty for restenosis after extended aortic arch anastomosis for type B interrupted aortic arch

A 4-month-old boy underwent a percutaneous balloon aortoplasty for restenosis after extended aortic arch anastomosis for type B interrupted aortic arch (IAA). Balloon aortoplasty resulted in a decrease in the peak systolic pressure gradient across the obstructive segment from 84 mmHg to 19 mmHg and in an increase in diameter from 2.5 mm to 4.3 mm. No complications related to the procedure were observed. We assume that balloon aortoplasty can be effective and safe for relieving postoperative aortic obstruction associated with IAA.     

Two-dimensional echocardiographic visualization of the aortic arch by right parasternal scanning in neonates and infants

Summary Echocardiography has previously been of limited use in visualizing the aorta in neonates and infants. Using a new technique—right parasternal scanning—the aortic arch and brachiocephalic vessels were studied in 50 children under 18 months of age. Complete visualization was accomplished in 47 patients including those 1) under 1,000 grams, 2) with indwelling endotracheal tubes, 3) who had D- or L-transposition, or 4) with right or double aortic arch. Inadequate studies were related to bilateral pneumothorax (2 neonates) and lack of cooperation (1 infant). Close correlation was obtained between images of the aorta by echocardiography and by angiography. Two-dimensional echocardiography can now visualize the aortic arch in small infants and children. In conjunction with another new technique—simultaneous Doppler flow-detection—coarctation of the aorta can be completely evaluated noninvasively in children of all ages including neonates. Presented, in part, at the World Congress of Pediatric Cardiology, June 2–6, 1980, London, England     

Magnetic resonance angiography of an ipsilateral double aortic arch due to persistent left fourth and fifth aortic arches

Persistence of a fifth aortic arch with an ipsilateral fourth arch manifested as a double-lumen aortic arch (DLAA) is a rare congenital anomaly. We present one of the first cases diagnosed by magnetic resonance angiography (MRA) in a 7-year-old girl referred for treatment of subaortic membrane and aortic regurgitation.     

Interrupted aortic arch with a right descending aorta and right ductus arteriosus,causing severe right bronchial compression

Summary We report here a patient with interrupted aortic arch and a right descending aorta, in whom the surgical management was complicated by the development of right bronchus compression and unilateral emphysema, due to the presence of the right ductus arteriosus.     

Right aortic arch with coarctation in Chinese children

Background Because of the rarity of right aortic arch coarctation there are few reports of large groups of patients. Objective To characterize the frequency and type of right aortic arch coarctation in a large group of pediatric patients. Materials and methods From June 1997 through May 2007, 11,276 consecutive children with congenital heart disease underwent multidetector CT (MDCT), MRI or angiocardiography examination. All children with a right aortic arch or coarctation were reviewed. Results Right aortic arch coarctation was found in 11 children representing 0.1% of the total group of 11,276 children, 1.7% of 658 children with native coarctations and 2.3% of 473 children with a right aortic arch. Among the 11 patients, 6 had long-segment narrowing and 7 had an aberrant left subclavian artery. Conclusion MDCT, MRI and angiocardiography are reliable imaging techniques for the diagnosis of right aortic arch and coarctation. Our findings showed that the pattern of right aortic arch coarctation was different from that of left aortic arch coarctation, suggesting that they are different etiological entities. The pivotal role possibly played by flow dynamics in the development of right aortic arch coarctation is discussed.     

Biphasic flow velocity pattern in the descending aorta in double aortic arch

We report a case of double aortic arch in which the biphasic flow velocity pattern in the descending aorta changed to a monophasic flow velocity pattern after corrective surgery. The hypoplasia of left aortic arch might prolong the acceleration time of the flow velocity in the left aortic arch.     

A persistent fifth aortic arch in man: A double-lumen aortic arch (presentation of a new case and review of the literature)

Summary Persistent fifth aortic arch manifested as a double-lumen aortic arch (DLAA) is a rare anomaly. We present such a case with transposition of the great arteries, ventricular septal defect (VSD), and pulmonary atresia. Review of the eight previously published cases indicates relatively frequent association of DLAA with preductal coarctation of the aorta. We attempt to explain the coexistence of this lesion with DLAA. We also discuss evidence supporting derivation of the inferior channel of the DLAA from the embryologic fifth aortic arch.     

Abnormal development of fourth aortic arch derivatives in the pathogenesis of tetralogy of Fallot

Summary Aortic arch (AoA) anomalies were studied in 233 patients with tetralogy of Fallot (TOF) of whom some had coexisting pulmonary atresia (PA). There was a 23% incidence of a right AoA in patients without PA, 21% in those with both PA and persistent ductus arteriosus (PDA), and 50% in those with PA and major aortopulmonary collateral arteries (MAPCAs). There was a 5% incidence of an aberrant subclavian artery in patients without PA and a 16% incidence in those with PA and MAPCAs. In this cohort an elongated ascending aorta was observed both with and without high aortic arch. These aortic arch anomalies were frequently associated with PA and MAPCAs.     

Aortic atresia with interruption of the aortic arch and an aortopulmonary fistulous tract: Case report

We present a case of interruption of the aortic arch (IAA) in association with aortic atresia. A pulmonary artery to ascending aorta fistulous channel at the level of the sinuses of Valsalva of the pulmonary artery was present and supplied the ascending aorta and coronary arteries. The communication had a significant length, so embryologically it is not an aortopulmonary window. There have been only three reported cases in the literature of IAA with aortic atresia; in none was a pulmonary artery to ascending aorta fistulous tract present.