Surgical treatment of interrupted aortic arch in infants under three months of age

From March, 1982, through March, 1988, 8 infants less than 3 months of age underwent surgical treatment of interrupted aortic arch. Five patients had IAA type A and 3 patients had type B. Seven patients with associated VSD underwent staged operations and 1 infant with aortopulmonary window underwent primary repair off cardiopulmonary bypass. Aortic arch reconstruction was by subclavian aortic anastomosis (6) or large tube graft (8 mm) interposition (2) combined with pulmonary artery banding (5). All seven patients with associated VSD survived the first-stage operation. One patient subsequently underwent pulmonary artery banding, two underwent patch aortoplasty and four patients underwent intracardiac repair and removal of a pulmonary artery band, with six survivors (86%). The operative death occurred in an infant in whom modified Damus-Kaye-Stansel operation was carried out for severe subaortic stenosis, which rerouting all left ventricular blood through VSD and the main pulmonary artery into the ascending aorta and reconstructing right ventricular-distal pulmonary artery connection by a valved external conduit. All six surviving patients are doing well at present (mean follow up of 3 years) without any significant pressure gradient between the ascending and thoracic aorta. Based on these data we conclude: (1) Aortic arch reconstruction and pulmonary artery banding can be reliably performed even in critically ill infants with IAA and isolated VSD. (2) The primary repair will provide better chance of survival in infants with IAA associated with significant LVOTO, truncus arteriosus or aortopulmonary window that do not readily lend themselves to pulmonary artery banding.     

Surgical treatment of coarctation of the aorta and interrupted aortic arch

From December, 1984, through April, 1987, ten infants with coarctation of the aorta and six with interrupted aortic arch underwent staged repair of aorta and other cardiac lesions. Simultaneous pulmonary artery banding was performed in six of 8 patients with ventricular septal defect (VSD) and in all of seven patients with complex cardiac lesions. With first operation, there were no operative deaths and two late deaths. Eight of 14 survivors underwent total correction of associated lesions at three to 17 months after initial operation. VSD was closed in five patients with one operative death. One patient required pulmonary artery debanding alone because of decrease of VSD size. The Damus-Kaye-Stansel operation was performed successfully in one patient with Taussig-Bing anomaly and the Jatene operation was done in one with transposition of the great arteries. Based on these results, we prefer staged repair with pulmonary artery banding for coarctation or interruption of the aorta associated with complex cardiac lesions.     

Total anatomic correction of interrupted aortic arch complex. Experience in 4 infants

In two cases of interrupted aortic arch (IAA) of type A, one associated with a ventricular septal defect (VSD) and one with an aortopulmonary window, and two of type B, both associated with a VSD, total anatomic repair was performed at respective ages of 6 months and 24, 8 and 3 days. All four operations were performed through a median sternotomy, using profound hypothermia and circulatory arrest. The repair included resection of the patent ductus arteriosus, direct end-to-side anastomosis of the descending to the ascending aorta and closure of the VSD or, in one case, of the aortopulmonary window. The two oldest infants (with type A IAA) survived. Reexamination two years postoperatively demonstrated good width of the aortic anastomosis with no gradient. In the child who had had an aortopulmonary window there was a proximal tight stenosis of the right pulmonary artery, which was corrected at reoperation. Total anatomic correction of IAA through an anterior approach is technically feasible and the aortic anastomosis seems to grow satisfactorily. The management of very sick neonates with IAA remains a great challenge.     

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.     

Primary repair for aortic arch obstruction associated with ventricular septal defect

Background

The aim of the present study was to evaluate the current outcome and reoperation rate after applying a one-stage correction strategy for interrupted aortic arch (IAA) with ventricular septal defect (VSD) and also for aortic coarctation and hypoplastic aortic arch (CoA-HyAA) with VSD beginning September 1999.

Methods

Twenty-four consecutive patients with IAA (n = 12) or CoA-HyAA (n = 12) with VSD underwent early one-stage correction. Patients' mean age was 12 days (range, 2 to 188); mean weight was 3.6 kg (range, 2.1 to 7.3), 6 patients were less than 2.5 kg. Three IAA were type A, 5 type B1, 3 type B2, and 1 type C. Associated anomalies included a large VSD in all, left ventricular outlet tract obstruction in 5, transposition of the great arteries, aortopulmonary window, persistent truncus arteriosus, and double-outlet right ventricle in 1 patient. Selective brain perfusion through innominate artery and selective coronary perfusion through aortic root during aortic arch reconstruction was used in all patients. Mean follow-up was 2.2 ± 0.9 years.

Results

There was no early, no late mortality, and no postoperative neurologic complications. Mean crossclamp duration was 72 ± 32 minutes, lowest temperature 22.8 ± 4°C and selective brain and coronary perfusion duration was 34 ± 13 minutes. Eighteen patients required delayed sternal closure at 1.7 days postoperatively. New perioperative management reduced the overall morbidity. Four patients after IAA plus VSD repair developed aortic arch restenosis and were successfully treated by balloon dilatation. One patient with d-TGA underwent right ventricular outflow tract reconstruction of right ventricular outlet tract obstruction 7 months after the initial repair. Pressure gradients across the anastomosis at most recent follow up were less than 10 mm Hg. All patients are asymptomatic and are developing normally.

Conclusions

One-stage complete correction is feasible in newborns with aortic arch obstruction with VSD. Complex cardiac anatomy presents no additional risk for the procedure. The early one-stage correction yields excellent surgical results and good functional outcome.     

Repair of coarctation with persistent fifth arterial arch and atresia of the fourth aortic arch

Coarctation of the aorta with persistent fifth arterial arch and atresia of the fourth aortic arch between the left common carotid and left subclavian arteries was treated surgically in a two-month-old boy with transposition of the great arteries and double-outlet right ventricle. The aortic arch was repaired using side-to-side anastomosis of the left common carotid and left subclavian arteries, patch repair of the coarctated segment at the origin of the left subclavian artery, and ligation of the patent ductus arteriosus. Pulmonary arterial banding and balloon atrioseptostomy were performed for associated anomalies.     

Pulmonary root translocation for repair of Taussig-Bing anomaly with interrupted arch

Anterior translocation of the pulmonary root was used as a new approach to the staged repair of Taussig-Bing anomaly with an interrupted aortic arch. It was performed to construct the right ventricle outflow tract with intraventricular baffling of the left ventricle to the aorta as the second stage operation after repair of the interrupted arch and pulmonary artery banding. This technique allows minimization of pulmonary regurgitation and has the major theoretical advantage for growth potential, which could diminish the need for reoperation.     

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.     

Closure of ventricular septal defect of interrupted aortic arch with left ventricular outflow obstruction]

Leftward shift of the infundibular septum (IS) in interrupted aortic arch (IAA) with ventricular septal defect (VSD) often develops significant left ventricular outflow obstruction (LVOTO). Seven-day-old boy with 2.6 kg body weight underwent the two-stage operation for this anomaly. The aortic arch was interrupted between the left common carotid and the left subclavian artery. At the first stage, a 5 mm GORE-TEX graft was used to connect the interrupted arch, and pulmonary artery banding was performed. In closure of VSD at the second stage, IS was penetrated by stitches for the VSD patch to left ventricular outflow tract. IS with leftward shift could be pulled toward right ventricular side with patch fixation and LVOTO was prevented by this method.     

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.     

Mirror image Berry syndrome: a case report of complex aortopulmonary malformation in viscero-atrial situs inversus

We report a very rare case of an infant with viscero-atrial situs inversus presenting mirror image Berry syndrome (aortopulmonary window, interrupted aortic arch, anomalous origin of the left pulmonary artery, and intact ventricular septum). Successful surgical treatment was accomplished 56 days following the initial rescue bilateral pulmonary artery banding.     

A case of tracheo-bronchial stenosis after extended end-to-end aortic arch anastomosis for interrupted aortic arch treated with suspension of the ascending artery and pulmonary artery

A 9-day-old boy had pulmonary artery banding and extended end-to-end aortic arch anastomosis for ventricular septal defect (VSD) and type A interrupted aortic arch. Severe dyspnea gradually developed. At 3 months of age, intracardiac repair of VSD was performed. Weaning from the ventilator was difficult. Endoscopic examination and chest CT revealed stenosis of the right and left main bronchi and compression of tracheal bifurcation and the right and left main bronchi by the ascending aorta and pulmonary artery. Suspension of the ascending aorta and pulmonary artery was performed 15 days after VSD closure. Nine days after this procedure, the patient was weaned from respirator. Postoperative course was uneventful. Bronchial stenosis may be caused from extended end-to-end aortic arch anastomosis.     

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。     

Growth of the hypoplastic ascending aorta after radical palliation

Surgical treatment of hypoplasia of the ascending aorta is a difficult problem. An approach using proximal pulmonary artery to distal aorta bypass with distal pulmonary artery banding was employed in 19 patients with an ascending aorta less than 6 mm in diameter but a patent aortic outflow. There were 11 male and 8 female neonates and infants, and weight ranged from 2 to 4.5 kg (median weight, 3.5 kg). Four patients had transposition of the great arteries, 12 had subaortic stenosis, and 5 had interrupted aortic arch. There was one operative death. Cerebral cyanosis developed in 3 of the 4 patients with transposition of the great arteries and necessitated additional operations within the first year postoperatively. Therefore, palliation with a pulmonary artery to descending aorta conduit and banding was abandoned in such patients. All 14 surviving patients with normally related great arteries had successful palliation and growth of left heart structures. Subsequent procedures included a Fontan/Damus procedure in 1 patient, patch aortoplasty and pulmonary arterioplasty in 5 patients, arch reconstruction with aortic valvulotomy and pulmonary arterioplasty in 2, double arch reconstruction in 2, and simple patch aortoplasty with plans for subsequent pulmonary arterioplasty and ventricular septal defect closure in 1. Results demonstrate that in patients with a hypoplastic ascending aorta, this radical method of palliation can result in growth of right and left heart structures and thus provides the possibility of biventricular repair.     

Neonatal repair of right interrupted aortic arch with cerebro-myocardial perfusion technique

Right interrupted aortic arch and descending aorta is exceedingly rare and most likely cause respiratory presentation, since patent ductus arteriosus (PDA) courses over the right mainstem bronchus. We report a case of successful neonatal biventricular repair of a right interrupted aortic arch (type B), with an aberrant right subclavian artery ventricular septal defect (VSD) in a 2.7?kg term neonate with DiGeorge syndrome. Patient presented in severe respiratory distress and acidosis at one day old. Two-dimensional (2D) echocardiography revealed aortic arch interruption beyond the common carotid arteries with large perimembranous outlet VSD. Aortic annulus diameter was 4.8?mm and there was no left ventricle (LV) outflow tract obstruction. Three-dimensional (3D) CT-scan confirmed these findings and identified a right-sided ductal arch that continued over the right mainstem bronchus into a right-sided descending aorta and aberrant right subclavian artery. Brachiocephalic perfusion and ductal perfusion was employed for cooling during cardiopulmonary bypass. Under deep hypothermia (27 °C rectal temperature), selective cerebro-myocardial perfusion was used for successful aortic arch repair without sacrificing the aberrant right subclavian artery. A direct tension-free anastomosis was attained. Her postoperative course was uneventful and her respiratory symptoms disappeared postoperatively. Early surgical correction is mandatory for these patients with unique anatomy and presentation.     

Transposition of the great arteries with aortic arch obstruction. Anatomical review and report of surgical management

Transposition of the great arteries (S,D,D) is a common congenital cardiovascular malformation that is occasionally associated with ventricular septal defect and left ventricular outflow tract obstruction. Recently right ventricular outflow tract obstruction associated with an aortic arch anomaly has been recognized as an infrequent but important variant of transposition of the great arteries, and this constellation presents a unique surgical challenge. Five infants with this constellation whose systemic circulation was dependent on flow through the ductus arteriosus have undergone definitive surgical treatment with four survivors. An anatomical review of 129 specimens with transposition of the great arteries revealed that 17% had right ventricular outflow tract obstruction and 7% had associated aortic arch obstruction as well. All specimens with aortic arch obstruction and ventricular septal defect except one had a malalignment type ventricular septal defect. Although several treatment options may be considered, recent experience with arterial switch repair in the neonate with transposition of the great arteries (S,D,D) prompted repair in these patients by arterial switch, ventricular septal defect closure, repair of aortic arch obstruction, and augmentation of the right ventricular outflow tract.     

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.     

Staged repair of type A interrupted aortic arch using the Blalock-Park method in infancy: development of the reconstructed aortic arch

Three infants with type A interrupted aortic arch, VSD and PDA underwent staged operation. Aortic arch reconstruction with Blalock-Park's method, PDA division, and PA banding were performed as the first operation. Subsequently all patients were underwent the second operation, consisting of VSD closure and PA debanding, and are doing well now. Pressure gradient at the anastomosis site, the diameters of the anastomosis site and the left subclavian artery, and the diameter ratios of the anastomosis site and the left subclavian artery to the ascending aorta were evaluated after the first and second operation. Two patients with good size of the left subclavian artery at the first operation showed excellent growth of the reconstructed aortic arch and trivial pressure gradient at the anastomosis site. The other patient, whose preoperative left subclavian artery was small (2.5 mm in diameter) resulted in residual stenosis at the anastomosis site. The development of the left subclavian artery was satisfactory in all patients. These data suggest that the aortic arch reconstructed with Blalock-Park's method can grow if the diameter of the anastomosis site has adequate size at the first operation.     

Early palliation of univentricular hearts with subaortic stenosis and ventriculoarterial discordance. The arterial switch option.

The optimal Fontan-type operation greatly depends on appropriate initial palliation. Several surgical techniques have been used in infancy to palliate patients with univentricular hearts, ventriculoarterial discordance, and subaortic stenosis. The two most common are pulmonary artery banding and Damus-Norwood procedures. Palliative arterial switch operation is another surgical option that was used in this early series of seven infants. The principle of this operation is to "switch" the subaortic obstruction into a subpulmonary obstruction; the coronary artery relocation on the large pulmonary trunk creates a harmonious aortic root and the connection of the rudimentary ventricular chamber to the pulmonary artery trunk creates a natural protection of the pulmonary vascular bed through the restrictive bulboventricular foramen. Seven infants with univentricular hearts, ventriculoarterial discordance, and subaortic stenosis underwent a palliative arterial switch operation. All infants had an associated aortic arch obstruction of various degrees, including one with interrupted aortic arch, five with coarctation with severe arch hypoplasia, and one with isolated arch hypoplasia. There were three with double-inlet left ventricle, three with tricuspid atresia, and one with transposition of the great arteries with ventricular septal defect and severe right ventricular hypoplasia. The subaortic obstruction was patent at birth in five patients who underwent a palliative switch operation in the first 2 months of life, and rapidly occurred following a previous neonatal pulmonary artery banding associated with arch repair in two patients who underwent a switch operation at 5 and 8 months of age, respectively. The operation includes aortic arch repair without prosthetic material, an atrial septectomy, and the arterial switch. An associated pulmonary shunt was required in five patients and a pulmonary artery banding in one. There was one early death in a patient with [S,L,L] anatomy and congenital atrioventricular block, leading to an early mortality of 14% (95% confidence limits: 1% to 28%). There was one late death. Four survivors are waiting for a Fontan-type procedure, and one survivor had satisfactory right ventricular growth. Early palliative arterial switch operation offers several advantages: reconstruction of a harmonious aortic root, natural protection of the pulmonary bed through the restrictive bulboventricular foramen, prevention of deleterious myocardial hypertrophy, and arch reconstruction without the introduction of a foreign material. This aggressive technique may provide a satisfactory palliation in infants with univentricular hearts and ventriculoarterial discordance, when the bulboventricular foramen/aortic anulus ratio is less than 0.8 or when the subaortic stenosis is severe enough to be associated with an arch obstruction.(ABSTRACT TRUNCATED AT 400 WORDS)     

Repair of type B interruption of the aortic arch. Results and follow-up

Surgical repair of type B interruption of the aortic arch and ventricular septal defect (VSD) in the newborn period is difficult and the optimal approach is controversial. We report here our early and late results using a two-staged approach in the repair of these lesions in seven consecutive neonates whose weights ranged from 2.9 to 3.8 kg and who were all less than 21 days of age. Each underwent aortic arch reconstruction off cardiopulmonary bypass with placement of a large (6 to 10 mm) polytetrafluoroethylene (PTFE) graft and pulmonary artery banding for interruption of the aortic arch (six) or extreme hypoplasia (one) of the arch. All seven (100%) survived the first-stage operation. No significant (greater than 10 mm Hg) conduit gradient was found in the seven patients studied 3 to 18 months postoperatively. One death occurred in a patient at 1 year of age with severe subaortic stenosis. Six patients subsequently underwent closure of VSD and removal of a pulmonary artery band, with five survivors (83%). The operative death occurred in an infant in whom pulmonary hypertension developed from an inadequate pulmonary artery band. One late death occurred at home when a tracheostomy tube, required because of severe tracheomalacia, became plugged. The long-term survival rate for completion of both stages is 57%. We conclude: (1) Aortic arch reconstruction and pulmonary artery banding can be reliably performed even in critically ill infants, and the 8 or 10 mm grafts should be adequate for several years. (2) Significant subaortic stenosis occurred in only one patient. (3) Pulmonary artery banding was the greatest source of difficulty, and distortion of the pulmonary arteries, inadequate banding, and compression of the trachea were all seen. Therefore, the staged repair will provide good results with this complex anomaly, and most of the problems associated with this approach may be eliminated by early second-stage repair.