Diagnosis and Treatment of Congenital Heart Diseases with Right Aortic Arch_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

GUO Jian , LI Xiaofeng , LIU Hui , YUAN Feng , BAI Song , ZHAO Yudong , DING Nan , ZHENG Jia , LI Qilin.

Department of Cardiac Surgery，Children’s Heart Center，Beijing Children’s Hospital，Beijing 100045，P. R. China;

Corresponding author：

Keywords：

Congenital heart disease; 　Vascular ring; 　Right aortic arch

DOI：

10.7507/1007-4848.20130200

Video：

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Objective To investigate diagnostic and treatment strategies of patients with congenital heart diseases and right aortic arch. Methods Clinical data of 27 children who underwent surgical correction for congenital heartdiseases and right aortic arch in Children’s Heart Center of Beijing Children’s Hospital from January 1，2012 to April 1，2013 were retrospectively analyzed. There were 20 male and 7 female patients with their age of 10.96±12.08 months and body weight of 7.70±3.13 kg. All the patients had right aortic arch，including 14 children with tetralogy of Fallot，9 childrenwith ventricular septal defect，1 child with patent ductus arteriosus，1 child with pulmonary artery sling and 2 children with isolated double aortic arch. Thirteen children were found to have a vascular ring including aberrant left subclavian artery and double aortic arch. All the patients received surgical correction for their intracardiac anomalies and concomitant transposition of the left subclavian artery for aberrant left subclavian artery or division of the nondominal arch for double aortic arch. Results Three children died perioperatively including 1 child who was unable to be weaned from cardiopulmonary bypass，1 child without vascular ring who died 7 days postoperatively，and 1 child with double aortic arch who died of acuterespiratory distress syndrome. There was another child who gave up further postoperative treatment. Twenty-three children were followed up for 3-17 months after discharge. Echocardiography showed satisfactory correction of their intracardiac anomalies. All the patients alive recovered well with good pulses in arteries of both arms. Conclusion Careful strategiesare needed for surgical correction of patients with congenital heart diseases and concomitant right aortic arch. Comprehensivepreoperative evaluation including contrast-enhanced CT and magnetic resonance imaging is helpful for clinical decision making in diagnosis and treatment. Clinical outcomes of 1-stage surgical correction of vascular ring and other concomitant intracardiac anomalies are satisfactory with good short-term results.

Citation： GUO Jian,LI Xiaofeng,LIU Hui,YUAN Feng,BAI Song,ZHAO Yudong,DING Nan,ZHENG Jia,LI Qilin.. Diagnosis and Treatment of Congenital Heart Diseases with Right Aortic Arch. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2013, 20(6): 628-631. doi: 10.7507/1007-4848.20130200 Copy

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5.	陈金兰，杨一峰，刘锋，等. 先天性心脏病合并气道异常的围术期处理 . 中国胸心血管外科临床杂志， 2010， 17 （6）：509-511.
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7.	Berdon WE， Baker DH， Wung JT， et al. Complete cartilage-ring tracheal stenosis associated with anomalous left pulmonary artery：the ring-sling complex . Radiology， 1984， 152 （1）：57-64.
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10.	Knight L， Edwards JE. Right aortic arch. Types and associated cardiac anomalies. Circulation， 1974， 50 （5）：1047-1051.
11.	Kypson AP， Anderson CA， Rodriguez E， et al. Double aortic archin an adult undergoing coronary bypass surgery：a therapeutic dilemma ?Eur J Cardiothorac Surg， 2008， 34 （4）：920-921.
12.	Naoum JJ， Parenti JL， LeMaire SA， et al. Endovascular repair of a right-sided descending thoracic aortic aneurysm with a right-sided aortic arch and aberrant left subclavian artery . Ann Thorac Surg， 2008， 85 （3）：1074-1076.
13.	Kleine P， Balci M， Moritz A. Primary complete repair of partial double aortic arch and Kommerell diverticulum. Ann Thorac Surg， 2011， 91 （2）：627-629.
14.	Shinkawa T， Greenberg SB， Jaquiss RD， et al. Primary translocationof aberrant left subclavian artery for children with symptomatic vascularring . Ann Thorac Surg， 2012， 93 （4）：1262-1265.
15.	Kouchoukos NT， Masetti P. Aberrant subclavian artery and Kommerell aneurysm Surgical treatment with a standard approach. Thorac Cardiovasc Surg， 2007， 133 （4）：888-892.
16.	郭健，李晓峰，刘晖，等. 迷走左锁骨下动脉的外科治疗 . 中华小儿外科杂志， 2012， 33 （9）：712-713.
17.	Yang C， Shu C， Li M， et al. Aberrant subclavian artery pathologies and Kommerell's diverticulum：a review and analysis of published endovascular/hybrid treatment options . J Endovasc Ther， 2012， 19 （3）：373-382.
18.	Backer CL， Mavroudis C， Rigsby CK， et al. Trends in vascular ring surgery. J Thorac Cardiovasc Surg， 2005， 129 （6）：1339-1347.
19.	Backer CL， Hillman N， Mavroudis C， et al. Resection of Kommerell’sdiverticulum and left subclavian artery transfer for recurrent symptomsafter vascular ring division. Eur J Cardiothorac Surg ， 2002， 22 （1）：64-69.
20.	Kir M， Saylam GS， Karadas U， et al. Vascular rings：presentation， imaging strategies， treatment， and outcome. Pediatric Cardiology， 2012， 33 （4）：607-617.

1. 　王谦，钟玉敏，孙爱敏，等. 血管环伴气管狭窄的多层螺旋CT诊断 . 上海交通大学学报：医学版， 2011， 31 （9）：1312-1315.
2. 　周丹，孙善权，崔虎军，等. 双主动脉弓合并法洛四联症同期矫治一例 . 中国胸心血管外科临床杂志， 2009， 16 （1）：18.
3. 　徐志伟，张海波，王顺明，等. 婴幼儿先天性气管狭窄伴复杂先心病的Ⅰ期纠治 . 中华小儿外科杂志， 2003， 24 （1）：15-17.
4. 　王树水，曾国洪，庄建，等. 婴儿及新生儿血管环并呼吸道压迫的诊断与治疗 . 实用儿科临床杂志， 2010， 25 （1）：14-17.
5. 　陈金兰，杨一峰，刘锋，等. 先天性心脏病合并气道异常的围术期处理 . 中国胸心血管外科临床杂志， 2010， 17 （6）：509-511.
6. 　Oshima Y， Yamaguchi M， Yoshimura N， et al. Management of pulm onary artery sling associated with tracheal stenosis. Ann Thorac Surg， 2008， 86 （4）：1334-1338.
7. 　Berdon WE， Baker DH， Wung JT， et al. Complete cartilage-ring tracheal stenosis associated with anomalous left pulmonary artery：the ring-sling complex . Radiology， 1984， 152 （1）：57-64.
8. 　Suh YJ， Kim GB， Kwon BS， et al. Clinical course of vascular rings and risk factors associated with mortality. Korean Circ J， 2012， 42 （4）：252-258.
9. 　Hastreiter A R ， D'Cruz I A ， Cantez T， et al. Right-sided aorta.Ⅰ. Occurrence of right aortic arch in various types of congenital heart disease.Ⅱ. Right aortic arch， right descending aorta， and associated anomalies. Br Heart J， 1966， 28 （6）：722-739.
10. 　Knight L， Edwards JE. Right aortic arch. Types and associated cardiac anomalies. Circulation， 1974， 50 （5）：1047-1051.
11. 　Kypson AP， Anderson CA， Rodriguez E， et al. Double aortic archin an adult undergoing coronary bypass surgery：a therapeutic dilemma ?Eur J Cardiothorac Surg， 2008， 34 （4）：920-921.
12. 　Naoum JJ， Parenti JL， LeMaire SA， et al. Endovascular repair of a right-sided descending thoracic aortic aneurysm with a right-sided aortic arch and aberrant left subclavian artery . Ann Thorac Surg， 2008， 85 （3）：1074-1076.
13. 　Kleine P， Balci M， Moritz A. Primary complete repair of partial double aortic arch and Kommerell diverticulum. Ann Thorac Surg， 2011， 91 （2）：627-629.
14. 　Shinkawa T， Greenberg SB， Jaquiss RD， et al. Primary translocationof aberrant left subclavian artery for children with symptomatic vascularring . Ann Thorac Surg， 2012， 93 （4）：1262-1265.
15. 　Kouchoukos NT， Masetti P. Aberrant subclavian artery and Kommerell aneurysm Surgical treatment with a standard approach. Thorac Cardiovasc Surg， 2007， 133 （4）：888-892.
16. 　郭健，李晓峰，刘晖，等. 迷走左锁骨下动脉的外科治疗 . 中华小儿外科杂志， 2012， 33 （9）：712-713.
17. 　Yang C， Shu C， Li M， et al. Aberrant subclavian artery pathologies and Kommerell's diverticulum：a review and analysis of published endovascular/hybrid treatment options . J Endovasc Ther， 2012， 19 （3）：373-382.
18. 　Backer CL， Mavroudis C， Rigsby CK， et al. Trends in vascular ring surgery. J Thorac Cardiovasc Surg， 2005， 129 （6）：1339-1347.
19. 　Backer CL， Hillman N， Mavroudis C， et al. Resection of Kommerell’sdiverticulum and left subclavian artery transfer for recurrent symptomsafter vascular ring division. Eur J Cardiothorac Surg ， 2002， 22 （1）：64-69.
20. 　Kir M， Saylam GS， Karadas U， et al. Vascular rings：presentation， imaging strategies， treatment， and outcome. Pediatric Cardiology， 2012， 33 （4）：607-617.

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Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Diagnosis and Treatment of Congenital Heart Diseases with Right Aortic Arch

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