Anomalous origin of left coronary artery from the right pulmonary artery in association with type III aortopulmonary window and interrupted aortic arch

Anomalous origin of the left coronary artery from the pulmonary artery, also known as Garland-Bland-White syndrome, usually occurs as an isolated condition. We report an infant with caudal regression sequence diagnosed with interrupted aortic arch type B and type III aortopulmonary window, who was found to have anomalous origin of the left coronary artery from the right pulmonary artery at surgical repair. Successful repair of the aortopulmonary window and interruption was performed with reimplantation of the left coronary artery into the ascending aorta. This report highlights the importance of closely assessing the coronary ostia in patients undergoing complex aortopulmonary window repair.     

Concomitant repair of aortopulmonary window and interrupted aortic arch.

Aortopulmonary window with type A interrupted aortic arch was diagnosed in 2 critically ill neonates. Echocardiographic diagnostic methods provided precise anatomic information, which allowed cardiac catheterization to be avoided before operation. Repair was undertaken through a midline sternotomy using hypothermic, low-flow cardiopulmonary bypass with subclavian turn-down in one patient and hypothermic circulatory arrest with direct aortoaortic anastomosis in the other. Both methods provided good exposure and allowed favorable anatomic repair.     

一期纠治主肺动脉窗或右肺动脉起源于升主动脉

目的总结主肺动脉窗或右肺动脉起源于升主动脉的外科纠治经验。方法17例病儿中单纯主肺动脉窗5例,右肺动脉起源于升主动脉4例,主肺动脉窗伴主动脉弓中断、法洛四联症、房室间隔缺损、二尖瓣反流和气管狭窄等8例。1例无手术指征,16例行解剖纠治,平均手术年龄1．7岁;平均体重8．5 kg。结果手术无死亡。2例延迟关胸;1例再进胸止血。随访无死亡,无主动脉瓣上狭窄及肺动脉狭窄。结论主肺动脉窗及右肺动脉起源于升主动脉易早期产生肺动脉高压,一经诊断需立即手术。手术方式首选与伴发畸形一期纠治。主肺动脉窗及右肺动脉起源于升主动脉尽管多有伴发畸形,但早期手术纠治可获得较佳中、长期疗效。     

One-stage repair of interrupted aortic arch and aortopulmonary window with an autologous arterial flap

Interrupted aortic arch associated with an aortopulmonary window is a rare congenital malformation that requires an early diagnosis and surgical treatment to avoid irreversible pulmonary vascular changes. We describe herein successful one-stage repair in a neonate without the use of pericardium or other prosthetic material. Use of a transaortic approach permitted both accurate planning of the appropriate size of pulmonary trunk flap and also facilitated easy repair of the aortopulmonary septal defect. The growth potential of both great arteries was optimized.     

Repair of interrupted aortic arch and aortopulmonary window in an infant.

The diagnosis and repair in infancy of interrupted aortic arch and aortopulmonary window is described. Using deep hypothermia and circulatory arrest, aortic continuity was established with a prosthetic graft, which was anastomosed to the aortic orifice of the aortopulmonary window. The pulmonary artery side of the aortopulmonary window was closed directly. Postoperative cardiac catheterization demonstrated a good reconstruction. Previous experience with this rare variety of interrupted aortic arch complex is reviewed.     

One-stage repair of absence of the aortopulmonary septum and interrupted aortic arch

Absence of the aortopulmonary septum, interrupted aortic arch, aortic origin of the right pulmonary artery, intact ventricular septum, and patent ductus arteriosus is a rarely reported association. A 3-year-old boy underwent successful one-stage repair of this constellation of anomalies. A Dacron baffle was used both to close the huge aortopulmonary window and to direct blood to the right pulmonary artery. Type A interrupted aortic arch was repaired by direct anastomosis. Postoperatively, pulmonary artery pressure was less than half systemic pressure.     

Anterior translocation of the right pulmonary artery for airway obstruction caused by pulmonary hypertension

Both the bronchi are often severely squeezed by the dilated pulmonary artery in infants with ventricular septal defect and pulmonary hypertension. To relieve the considerable residual airway obstruction, the right pulmonary artery is translocated anteriorly to the ascending aorta concomitantly with the intracardiac repair. This technique is a useful procedure for infants with intractable respiratory failure caused by a dilated pulmonary artery.     

Autograft aortic arch extension and sleeve resection for bronchial compression after interrupted aortic arch repair

Successful correction of bronchial compression and severe bronchomalacia complicating repair of interrupted aortic arch was achieved using transverse aortic arch extension with a pulmonary artery autograft and left bronchial sleeve resection. This procedure increased space within the aortic arch and eliminated bronchial narrowing with excellent results.     

Pulmonary autograft patch aortoplasty for reconstruction of an interrupted aortic arch associated with an aortopulmonary window

We present here an infant with an aortopulmonary window associated with an interrupted aortic arch. Single-stage repair with a pulmonary autograft patch for augmentation of the interrupted aortic arch repair was performed. Transpulmonary patch closure was used to repair the aortopulmonary window. A computed tomography scan at the 1-month follow-up demonstrated a reconstructed aortic arch without obstruction, kinking, or any sign of bronchial or branch pulmonary artery compression.     

Aortic arch reconstruction for interrupted aortic arch using an aberrant right subclavian artery

An aberrant right subclavian artery can be used in a variety of ways in complex aortic arch reconstructions, including reconstruction of an interrupted aortic arch. Here, we described the case of a 4-month-old female infant with a type B interrupted aortic arch, who underwent arch reconstruction using an aberrant right subclavian artery.     

Use of the aberrant right subclavian artery in complex aortic arch reconstruction

An aberrant right subclavian artery can be used in a variety of ways in complex aortic arch reconstructions. Four patients (3 with interrupted aortic arch and 1 with coarctation) in whom this technique was used are presented.