先天性血管环的外科治疗

目的 讨论先天性血管环的诊断和手术治疗及影响预后的因素.方法 回顾性分析2010年1月至2013年12月收治的42例先天性血管环患儿(占同期手术患儿的1.04％)的病例资料.其中男性26例,女性16例;手术年龄24d至6岁,平均10.7个月.包括肺动脉吊带26例,双主动脉弓10例,右位主动脉弓合并迷走左锁骨下动脉3例,肺动脉吊带合并右位主动脉弓并迷走左锁骨下动脉2例,肺动脉吊带合并左位主动脉弓并迷走右锁骨下动脉1例.38例患儿合并其他的心血管畸形.36例患儿行螺旋CT检查气道重建,23例患儿行纤维支气管镜检查,除2例气道未见异常外其余患儿均存在不同部位不同程度的气管狭窄或软化.所有患儿均行手术矫治先天性血管环,根据情况同期行心内畸形矫治手术;1例同期行气管狭窄切除并Slide成形术,1例在术后行气管内支架植入术.结果 3例患儿院内死亡(7.1％),死亡原因均为术后反复发生气管内肉芽肿形成;其余39例均顺利出院.存活患儿总体术后呼吸机辅助时间14 h(22 h)[M(QR)],心脏ICU停留时间5d(8 d),住院时间19 d(9 d).肺动脉吊带患儿呼吸机辅助时间、心脏ICU术后停留时间均长于双主动脉弓患儿[23 h(123 h)比9 h(9 h),7 d(13 d)比4 d(2 d)],但差异无统计学意义(P＞0.05);住院时间明显长于双主动脉弓患儿[23 d(9 d)比16 d(6 d)],差异有统计学意义(x2=10.157,P=0.006).结论 先天性血管环早期手术治疗是安全、有效的,近期效果良好.合并的气管软化和狭窄的程度和范围是影响患儿预后的关键.     

小儿血管环外科治疗的单中心临床分析北大核心CSCD

目的探讨儿童血管环的最佳解剖分型、手术时机、手术方法和治疗效果。方法回顾性分析阜外医院小儿心脏外科中心2014~2019年各类血管环手术治疗58例患儿的临床资料,其中男32例(55.2%)、女26例(44.8%),中位年龄16.5(2~73)个月。对术前症状、影像学检查、血管环分类、手术方法、术后恢复等进行总结分析。结果全组患儿双主动脉弓畸形20例(34.5%),右位主动脉弓伴左侧动脉导管或韧带22例(37.9%),左位主动脉弓伴迷走右锁骨下动脉15例(25.9%),回旋主动脉伴颈位主动脉弓1例(1.7%)。中位呼吸机辅助时间6.0(0~648)h,中位住院时间14.5(7~104)d。全组共1例合并主动脉缩窄患儿围术期因重症肺部感染死亡,其余患儿出院后无症状生存,无再次手术,中位随访时间7.0(1~62)个月。结论对于原发病无法解释的呼吸困难及吞咽困难,或存在右位主动脉弓的患儿,需要术前完善CT或磁共振成像等影像学检查,以明确血管环诊断。手术治疗血管环安全可靠,能有效缓解症状,手术成功率高,再次手术干预率低,随访效果满意。     

婴幼儿血管环外科诊治

目的 总结婴幼儿先天性血管环外科诊治的临床经验.方法 2009年9月至2010年12月诊断并手术治疗14例血管环患儿,其中男9例,女5例;年龄2月～6岁;体重4.5～15.0 kg.所有病例术前均行X线胸片、超声心动图及增强多排螺旋CT检查确诊,行食管造影及纤维支气管镜检查各5例.病理类型包括双主动脉弓1例、肺动脉吊带7例、右位主动脉弓伴左位动脉导管/韧带6例,7例患儿合并心内畸形.全组患儿存在不同范围(12％～62％)和程度(45％～74％)的气管狭窄,5例术前存在局限性食管狭窄.12例患儿在体外循环下手术同期矫治心内畸形,2例在非体外循环下手术.结果 术后辅助呼吸7～ 308 h,中位数21 h;ICU停留16 ～314 h,中位数79.5 h.1例肺动脉吊带患儿住院死亡(7.1％).13例出院.失访1例,12例随访1～ 15个月,患儿消化道症状消失,活动耐力改善.呼吸道症状不同程度缓解,5例(41.7％,5/12例)仍然存在呼吸道症状.结论 临床上对有长期呼吸道和(或)消化道症状的患儿需考虑血管环的可能,增强CT是确定血管环诊断的最佳方法.血管环应尽早手术治疗,合并的长段气管狭窄需手术治疗,近-中期效果良好.     

自体肺动脉组织一期矫治主动脉弓病变合并心内畸形

目的 探讨自体肺动脉组织一期矫治主动脉弓病变合并心内畸形的手术方法和治疗效果.方法 1993年4月至2009年11月,共25例主动脉弓病变合并心内畸形的患儿接受了一期矫治手术.男性17例,女性8例;年龄26 d～6.5岁,平均11个月;体质量3.0～14.5 kg,平均12.4 kg.其中6例患儿为主动脉弓缩窄,6例为主动脉弓中断,13例为主动脉弓发育不良.手术技术包括切开肺动脉前壁,切除动脉导管,应用部分自体肺动脉壁重建主动脉弓,重建肺动脉,并同时矫治合并心内畸形.结果 24例患儿术后恢复顺利,1例患儿死于围手术期肺动脉高压危象.随访心脏超声及心脏CT检查证实主动脉弓重建及心内畸形矫治满意.术后未发生神经系统及其他并发症.随访过程中,患儿生长发育正常,主动脉弓无再狭窄.结论 自体肺动脉具有生长潜力且柔韧性好,是一种理想的重建主动脉弓的材料.一期矫治手术治疗先天性主动脉弓病变合并心内畸形可以得到满意的结果.     

先天性血管环产前超声诊断价值和意义

目的探讨产前超声诊断及鉴别诊断先天性血管环中完全性血管环的临床价值和意义。方法将经超声产前检查结合产后影像学检查或者引产后病理确诊的完全性血管环胎儿12例为研究对象,分析其产前超声的主要表现。结果本组中1例双主动脉弓构成完全性血管环,9例右主动脉弓、左位动脉导管、左锁骨下动脉构成完全性血管环,2例右位主动脉弓、左位动脉导管、迷走左无名动脉构成完全性血管环。结论临床产前诊断先天性完全性血管环最重要的切面为三血管气管,产前超声检查能较准确的诊断以及鉴别诊断常见的先天性完全性血管环,临床应用价值肯定。     

婴幼儿完全性血管环的早期外科治疗

目的 评价完全性血管环的早期外科治疗效果.方法 1999年3月至2006年6月手术治疗完全性血管环病儿16例,其中男7例,女9例.3月龄～3.2岁,平均(2.2±1.7)岁.术前均行超声心动图(ECHO)和心导管及心血管造影检查.诊断包括双主动脉弓6例;右位主动脉弓伴左侧韧带和左锁骨下动脉起源于降主动脉10例.术前麻醉诱导后和术毕行支气管镜检查比较.双主动脉弓通过切开次弓后部进入降主动脉的位置,将环离断;右位主动脉弓伴左侧韧带者,双重结扎离断动脉韧带或者离断后缝合两残端,迷走左锁骨下动脉起源于降主动脉处会有Kommerell憩室予切除,切下左锁骨下动脉转移至左颈总动脉作端侧吻合.结果 除1例病儿因术后2个月不能脱离呼吸机放弃治疗外,余病儿均生存.术后经支气管镜检查,受压迫的支气管腔均明显改善,7例病儿出现呼吸道并发症,术后监护时间延长,术后呼吸机辅助28～129 h,5例病儿由于肺血管阻力高,吸一氧化氮(NO)治疗1周后好转.随访3个月～2年,左锁骨下动脉移植至左颈总动脉者术后彩色超声检查显示血流通畅,左侧桡动脉搏动良好.结论 术前或术中支气管镜检查有助于确定狭窄的部位和程度,进一步明确气管软化或支气管软化的部位.早期完全性血管环手术治疗效果良好,尤其是迷走左锁骨下动脉在近降主动脉处横断后转移至左颈总动脉病例,远期随访满意.     

先天性血管环五例外科治疗

先天性血管环指由于主动脉弓及分支、肺动脉的起源或发育等异常,食管和/或气管外血管环形成,压迫或不压迫食管和/或气管,是一种罕见的心血管疾病.包括迷走右锁骨下动脉、肺动脉吊带、主动脉双弓畸形等,治疗以外科手术为主.我科自1963年2月至2011年12月手术治疗5例先天性血管环患者,报道如下.     

一期手术矫治主动脉弓中断及伴发畸形

目的总结主动脉弓中断(IAA)及伴发畸形的手术治疗经验。方法自2000年1月至2005年12月我科共收治48例IAA及伴发畸形患者(平均年龄1.1岁、平均体重7.0kg),合并畸形有室间隔缺损23例,完全性大动脉错位2例,主-肺动脉窗伴右肺动脉起源于主动脉3例,第5主动脉弓残存狭窄2例,右心室双出口2例,永存动脉干2例,迷走右锁骨下动脉伴降主动脉发育不良1例。48例中35例接受手术纠治,其中34例IAA及伴发畸形一期解剖纠治,1例伴右心室双出口患者行主动脉弓吻合及肺动脉环缩术纠治。结果手术死亡4例。术后26例随访3个月~4年,死亡2例。术后发生并发症7例,Ⅲ°房室传导阻滞和膈神经麻痹各1例,轻度吻合口梗阻5例,其余患者无残余解剖问题,心功能良好。结论在新生儿期施行IAA一期矫治手术成功率高,围术期合适的干预是治疗成功的关键;由于伴发畸形复杂,远期生存率不容乐观。     

主动脉弓中断的外科治疗

目的 总结主动脉弓中断及合并心脏畸形的外科治疗经验.方法 1997年1月至2008年1月,36例主动脉弓中断患者进行了外科手术治疗,其中男性22例,女性14例.儿童患者36例,年龄2个月～7岁,平均年龄2.8岁.成人患者1例,年龄31岁.33例合并心内畸形,其中31例正中开胸同时矫治主动脉弓中断和心内畸形;1例左侧切口矫治主动脉弓中断,正中开胸修补心内畸形;1例采用姑息手术.3例无心内畸形的患者2例采用左后外侧切口,1例采用正中胸部加上腹部切口.术式包括16例管道连接,9例直接吻合,9例直接吻合并补片成形,1例应用左锁骨下动脉翻转.在31例正中切口一期手术中,17例应用选择性脑灌注加下半身停循环,8例采用深低温低流量灌注,6例采用全身停循环.结果 住院死亡5例,3例死于肺部感染,1例死于肺动脉高压危象,1例死于术后低心排血量.术后早期有其他重要并发症7例.31例存活患者随访3个月～5年,无远期死亡,无需要再次手术的病例.结论 合并心内畸形的主动脉弓中断患者可采取选择性脑灌注加下半身停循环或深低温全身低流量下正中一期手术同时矫治.     

胸内大血管畸形所致食管狭窄症的外科治疗：附4例报告…

诊治右位主动脉弓伴左动脉韧带，迷走右锁骨下动脉所致吞咽困难各２例，右位主动脉弓属食管后型，迷走右锁骨下动脉自主动脉弓与降主动脉的分界处发出，经食管后向右斜行达右上肢压迫食管，２例行手术治疗，２例因症状轻微，未行手术，手术者近期吞咽困难完全缓解，结合文献对本症的发生及诊断治疗进行讨论。     

Echocardiography in the preoperative evaluation of vascular rings.

Vascular rings may produce tracheal and/or esophageal compression in infants and children. Traditionally recognized fluoroscopically, the exact anatomy of the ring and appropriate surgical correction are determined intraoperatively. The role of preoperative echocardiography was examined. Twenty patients with symptomatic vascular rings were evaluated preoperatively with echocardiography at this institution. Their ages ranged from 10 days to 11 years (mean, 17 months). There were 9 boys and 11 girls. Most (17/20) presented with respiratory symptoms in the first year of life, although in 3 patients dysphagia was the primary complaint (at birth, 4 months, 9 years). All underwent initial evaluation with a barium esophagogram prior to the echocardiogram. Surgical correction was subsequently performed and the exact anatomy identified. All barium esophagograms were interpreted prospectively as demonstrating a "vascular ring." Although often suspected fluoroscopically, the actual type of ring was correctly identified by echocardiogram in all cases including determination of the dominant arch and associated anomalies. The types of vascular rings included double aortic arch (10), right aortic arch with left ligamentum arteriosum and/or aberrant left subclavian artery (6); aberrant right subclavian artery (2), and pulmonary artery sling (2). Barium esophagogram remains the best screening test for children in whom a vascular ring is suspected. However, echocardiography is a useful noninvasive complementary examination to confirm the diagnosis, clarify anatomy, and exclude other major intracardiac pathology prior to surgical correction.     

Congenital vascular rings: surgical management of 111 cases

Between October 1974 and May 1987, 111 congenital vascular rings were submitted to surgical correction. There were 83 infants (age: 5 days-12 months, median: 7 months; weight: 1.9-8.5 kg, median: 7.1 kg), and 28 children (age: 1-13 years, median: 3.5 years; weight: 7.5-48 kg, median: 26.5 kg). Patients were divided into five groups: (1) double patent aortic arch (44 cases), (2) double aortic arch with atresia in different parts of the left arch (36 cases), (3) right aortic arch with left ligamentum arteriosum (21 cases), (4) left aortic arch with aberrant right subclavian artery and truncus caroticus (8 cases), and (5) pulmonary artery sling (2 cases). We had no intraoperative mortality but in the postoperative period, 2 neonates died of severe bacterial infections of the respiratory tract.     

Vascular ring and pulmonary loop: experience with the surgical treatment]

Operation was carried out on 143 children (aged from 5 days to 16 years) for tracheobronchial obstruction caused by vascular rings. A double arch of the aorta was found in 50 patients, double arch of the aorta with atresia of the left arch--in 50 patients, right arch of the aorta with ligamentum arteriosum--in 30, left arch of the aorta with an aberrant right subclavian artery--in 10, and a pulmonary loop in 3 patients. Stridor was the main clinical manifestation of the vascular ring. The diagnosis was established by means of two-projection chest radiography and esophagography with a barium meal, bronchoscopy, and angiography. The surgical approach was gained through a posterolateral (left or right) thoracotomy. No deaths occurred during the operation. Two infants died in the postoperative period from a severe bacterial infection of the respiratory tract. On the basis of a rich clinical experience the authors came to the conclusion that the presence of a right aortic arch (except for cases with situs inversus) is always pathognomic because it either causes the appearance of a vascular ring without any intracardiac anomaly, or it may be combined with a congenital heart disease in which a vascular ring is usually not encountered (tetralogy of Fallot, truncus arteriosus communis, etc.). The authors never encountered atresia of a segment of the right aortic arch. The last-named was either patent or absent.     

Operation for Aortic Arch Anomalies

Forty-two patients with aortic arch anomalies resulting in tracheoesophageal compression were treated during the period 1948 through 1978. These anomalies are important causes of upper respiratory and esophageal obstruction in babies and small children and can be corrected safely with excellent relief of symptoms. Nineteen patients (45%) had a right aortic arch with a ligamentum arteriosum, 17 patients (40%) had double aortic arches, and 6 patients (15%) had aberrant right subclavian arteries. Other associated congenital malformations and mental retardation were seen in 15 patients (36%). Diagnosis was accurately made in 38 patients (90%) by barium esophagogram. Basic surgical principles include exposure through a left thoracotomy, complete identification of the aortic arch anatomy, and division of the constricting ring. Surgical treatment resulted in 2 deaths (5%), and 1 patient died late. Early postoperative respiratory complications were common. All survivors were relieved of their symptoms late (m, 94 months) postoperatively.     

Resection of Kommerell's diverticulum and left subclavian artery transfer for recurrent symptoms after vascular ring division.

OBJECTIVES: A Kommerell's diverticulum in patients with a right aortic arch may become aneurysmal and be an independent cause of tracheoesophageal compression, even after ligation and division of a left ligamentum. We review the indications for and results of Kommerell's diverticulum resection and left subclavian artery transfer in children with a right aortic arch who previously underwent vascular ring (ligamentum) division. METHODS: From 1998 through 2001, eight children have been referred with recurrent respiratory symptoms (n=8) and/or recurrent dysphagia (n=4) after vascular ring division. Each child had a right aortic arch with a left ligamentum and had undergone division of the ligamentum elsewhere. All had a Kommerell's diverticulum that was not addressed at the initial operation. All patients had a repeat left thoracotomy with resection of the diverticulum. Five patients had division and reimplantation of the left subclavian artery into the left carotid artery to relieve the sling-like effect of the retroesophageal left subclavian artery on the right aortic arch. One other patient had primary Kommerell's diverticulum resection and transfer of the left subclavian artery to the left carotid artery. RESULTS: The mean age at the initial operation was 1.7+/-0.9 years, and the mean age at reoperation was 8.0+/-3.7 years. In all patients postoperative bronchoscopy confirmed relief of the tracheal compression. There were no complications related to the subclavian artery transfer. Two patients developed postoperative chylothorax, one requiring thoracic duct ligation. The median hospital stay was 5 days. All patients had dramatic resolution of their preoperative symptoms. CONCLUSIONS: Kommerell's diverticulum is an important anatomic structure that can cause recurrent symptoms in patients with a right aortic arch after ligamentum division. In selected patients, reoperation with resection of the Kommerell's diverticulum and transfer of a retroesophageal left subclavian artery results in relief of symptoms. This technique has become our procedure of choice as a primary operation for children with a right aortic arch and a significant Kommerell's diverticulum.     

Vascular rings and slings. Diagnosis and surgical treatment of 49 patients

Forty-nine infants with symptomatic vascular rings and slings, ranging in age from 20 days to 12 months, required surgical intervention between 1973 and 1984. The following anomalies were present in our patients: double aortic arch with left descending aorta (14), double aortic arch with right descending aorta (6), anomalous innominate artery (13), right aortic arch with aberrant left subclavian artery (4), left aortic arch with aberrant right subclavian artery (10), aberrant left pulmonary artery (pulmonary sling) (2). All the babies had symptoms related to compression of the trachea and/or esophagus. Four patients required temporary tracheostomy in the early postoperative period; 1 patient, affected by a pulmonary sling, required tracheal resection and anastomosis, for severe tracheomalacia. There was one hospital death in a patient with severe tracheal compression from an anomalous innominate artery and brain damage as a result of metabolic problems. Forty-eight patients survived and follow-up ranged from 3 months to 11 years. For each type of vascular anomaly encountered, and based on personal experience, we have outlined a diagnostic scheme allowing an accurate morphological definition and a subsequent surgical procedure.     

Dysphagia associated with an aortic arch anomaly in adults

Aortic arch anomalies typically become evident in early childhood with clinical manifestations such as cyanosis and feeding problems. Although uncommon and therefore not often considered, this developmental defect can cause dysphagia in the adult. The double aortic arch is the most common vascular ring in infants. Less well-known vascular rings in the adult are a right aortic arch with constricting left ligamentum arteriosum and an aberrant right subclavian artery. Dysphagia is common secondary to the constriction of the esophagus. Two patients recently presented to the Metropolitan Nashville General Hospital with prolonged history of dysphagia. Studies included barium swallow and aortogram, which showed right aortic arch in both patients with constricting left ligamentum arteriosum. The treatment for these symptomatic anomalies was division and excision of the ligamentum arteriosum and freeing of the esophagus. A right aortic arch on the chest roentgenogram in a patient with dysphagia should suggest a vascular ring as the etiology. If this is suspected, diagnosis is easily made and surgical treatment is effective. The relevant embryology will be discussed.     

Surgical treatment of vascular ring including right cervical aortic arch

Cervical aortic arch is a developmental entity consisting of persistence of the right or left third branchial arch and regression of the fourth branchial arches. In most cases, the aorta is redundant and crosses behind the esophagus to the opposite side. In the presence of an aberrant subclavian artery contralateral to the side of the aortic arch and a ligamentum arteriosum, a vascular ring is formed around the trachea and esophagus. Two young patients with right-sided cervical aortic arch, aberrant left subclavian artery, and ligamentum arteriosum presented with dysphagia and exertional dyspnea. In one patient, through a left thoracotomy, the ligamentum arteriosum was divided, and the trachea and esophagus were dissected thoroughly above and below the level of the ring. In addition, the aberrant left subclavian artery was divided at its origin from a large diverticulum and implanted into the left common carotid artery; the aortic diverticulum was resected. In the other patient, who had associated 22q11 chromosomal deletion, in addition to left-sided compression of the trachea and esophagus, there was additional marked compression of the right anterolateral trachea by the redundant ascending aorta. Through a median sternotomy, the ligamentum arteriosum was divided, and the trachea and esophagus were widely mobilized; an additional aortopexy of the ascending aorta to the right of the sternum resulted in the absence of tracheal compression. The cases of the two reported patients illustrate the clinical variability of vascular ring, including a right cervical aortic arch and the consequently versatile surgical approach that is needed to successfully address this combination of vascular anomalies.     

Tracheobronchial compression of vascular origin. Review of experience in infants and children

BACKGROUND: Tracheobronchial compression of vascular origin is an uncommon but important cause of respiratory distress in infants and children. We reviewed our surgical experience with 31 children, presenting vascular tracheobronchial compression. METHODS: Thirty-one children, with a median age of seven months, were operated on for airway and/or oesophageal compression secondary to vascular anomalies. Diagnostic findings, mainly established by bronchoscopy and angiography, revealed several pathologic disorders such as right aortic arch with aortic diverticulum in 10, double aortic arch in five, innominate artery compression in six, arteria lusoria in four, and pulmonary artery sling in one patient. Five children developed tracheobronchial compression after previous repair of a complex congenital heart disease. Clinical symptoms on admission were dominantly stridor, recurrent airway infection, dyspnea, respiratory distress and upper digestive complaints such as dysphagia and gastroesophageal reflux. RESULTS: Surgical relief was successful in 30 patients. Only one child with palliated tetralogy of Fallot and pulmonary artery sling died because of intractable extensive tracheobronchial stenosis. Late follow-up over three to 90 months showed a symptom-free evolution in most patients, including one reoperation for recurrent compression and one late death. CONCLUSIONS: Tracheobronchial compression by vascular structures in childhood is uncommon and may be masked by nonspecific respiratory symptoms, resulting in an often delayed diagnosis. Once imaging studies have clearly delineated the causal pathologic vascular structures, surgical correction is often straightforward and effective, in spite of the common presence of tracheobronchial malacia.     

Conversion of lusoric artery into right subclavian artery in one-stage neonatal repair of aortic arch anomalies and intracardiac defects.

In 2 patients, a lusoric artery was compressing the mediastinal structures during a one-stage repair of type B aortic arch interruption and ventricular septal defect. In order to prevent the long-term complications of sacrifice of the subclavian artery, the lusoric artery was not simply divided but converted into a regular right subclavian artery by re-implantation of the abberant vessel at the origin of the right carotid artery during the one-stage repair. At follow up, both patients have normal right radial arterial pulsations. In one patient, angiography was repeated and confirmed a well functioning right subclavian artery. Conversion of a lusoric artery into a right subclavian artery during one-stage repair of aortic arch anomalies and intracardiac defects is not only feasible but also indicated to preserve subclavian artery function and to prevent compression of the mediastinal structures by a vascular ring.