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.     

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.     

Aortopulmonary window

Aortopulmonary window is a rare defect caused by failure of fusion of the two opposing conotruncal ridges that are responsible for separating the truncus arteriosus into the aorta and pulmonary artery. Aortopulmonary window may occur as an isolated lesion or it can be associated with other cardiac abnormalities in one third to one half of cases. The most common associated lesions are arch abnormalities, specifically interrupted aortic arch and coarctation of the aorta. Antenatal diagnosis is rare. In the current era, early mortality following repair of simple aortopulmonary window approaches zero and depends on the presence of associated lesions, especially interrupted aortic arch. Long-term outcome should be excellent. Early morbidity includes pulmonary artery stenosis and residual aortopulmonary septal defects. Long-term follow-up is indicated to look for recurrent lesions such as the development of branch pulmonary artery stenosis and arch obstruction.     

Repair of interrupted aortic arch and associated malformations in infancy: indications for complete or partial repair

There is uncertainty regarding the best method of repair of interrupted aortic arch. The question is whether to perform primary definitive repair of this anomaly plus the associated defects versus arch repair only and palliation of the intracardiac defects, usually by pulmonary artery banding. Since 1976, 16 infants with interrupted aortic arch have been treated surgically. They were seen at 5.2 +/- 3 days of age and weighed 3.2 +/- 0.7 kg. The interruption occurred between the left carotid and left subclavian arteries (type B) in 9 and between the left subclavian artery and the descending aorta in 7 (type A). Isolated ventricular septal defect (VSD) was the only associated anomaly in 7 and aortopulmonary window, in 4. Two patients had truncus arteriosus type 1. Three had transposition of the great arteries: 1 with VSD and 2 with single ventricle. Prior to 1980, our policy was to palliate all patients. Between 1976 and 1980, 4 infants underwent left thoracotomy with arch repair plus pulmonary artery banding (3, VSD; 7, transposition of the great vessels and single ventricle) with only 1 (25%) survivor. Because of this high mortality, 8 patients with interrupted aortic arch and VSD or aortopulmonary window, seen since 1980, received complete repair with median sternotomy, end-to-end arch anastomosis, and closure of the VSD or aortopulmonary window utilizing profound hypothermia and circulatory arrest. All 8 survived.(ABSTRACT TRUNCATED AT 250 WORDS)     

Surgical management of aortopulmonary window.

OBJECTIVE: Aortopulmonary window is a rare congenital malformation involving a window-like communication between the ascending aorta and the pulmonary artery. Here, we present our experience regarding the surgical repair of an aortopulmonary window, and also assess the long-term outcome. METHODS: Thirteen children with an aortopulmonary window associated with various congenital lesions underwent a repair of the defect. The age at operation ranged from 3 days to 1 year (median age, 19 days). The patient's weight ranged from 2.1 to 7.0 kg (mean weight, 3.6 kg). The associated lesions included an interrupted aortic arch (5 patients), a ventricular septal defect (2), an atrial septal defect (1), mitral valve regurgitation (1), and tricuspid atresia [Ic] with mitral valve regurgitation (1). The aortopulmonary window was repaired with a cardiopulmonary bypass in 11 patients, and 2 patients were ligated without a cardiopulmonary bypass. RESULTS: One patient associated with tricuspid atresia died (mortality rate of 7.7%). There has been no late death during a mean follow-up of 7 years and 3 months. CONCLUSIONS: The surgical results for an aortopulmonary window are encouraging, even if such patients are associated with major cardiac anomalies and an interrupted aortic arch. Most have shown a good long-term outcome.     

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.     

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.     

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。     

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.     

Anterior translocation of the right pulmonary artery for relief of airway compression in the repair of distal aortopulmonary window and interrupted aortic arch

Airway compression by dilated right pulmonary artery (RPA) in infants with congenital heart disease can cause severe respiratory insufficiency and increase postoperative morbidity. Anterior aortopexy can be a good solution. However, in a case in which aortic arch repair is required, anterior aortopexy might not be effective or can be dangerous. Anterior translocation of the RPA can be a good option to avoid postoperative airway obstruction. We adopted this technique in a one-stage complete repair of distal aortopulmonary window, aortic origin of the RPA, interrupted aortic arch, and ventricular septal defect in a neonate with compression of both main bronchi. After 37 months of follow-up, no problem was encountered with her airway or RPA. We describe the technique used and the results obtained.     

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.     

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。     

Surgical management of aortopulmonary window and associated lesions

BACKGROUND: Aortopulmonary window is a rare congenital heart defect commonly associated with other cardiac anomalies. Although single institutional experience is low, several surgical techniques have been reported. The purpose of this study is to describe our approach to the management of aortopulmonary window and its associated lesions. METHODS: Between February 1996 and November 2002, 13 patients underwent repair of aortopulmonary window. The age range went from 4 days to 5.5 months (mean 42 +/- 52 days), with 9 patients younger than 1 month old. The weight range was from 1.9 to 6.7 kg (mean 3.5 +/- 1.2 kg). Concomitant cardiac anomalies were present in 11 patients. The major additional anomalies were interruption of aortic arch in 4 patients and tracheal stenosis in 1 patient. Initial diagnoses were made using two-dimensional echocardiography only. RESULTS: There was one postoperative death. In general, patients with aortopulmonary window and additional major defects had a prolonged intensive care unit and hospital stay when compared with the other patients. Follow-up time ranged from 2 months to 6.8 years (mean of 2.5 +/- 2.2 years). There were no reoperations and no late deaths. Transcatheter balloon dilatation of the repaired aortic arch was required in 1 patient and of the right pulmonary artery in another. All other patients had good flow to both pulmonary arteries. No residual shunts were detected at the aortopulmonary window site, and pulmonary pressures were normal. CONCLUSIONS: Aortopulmonary window may be effectively diagnosed with echocardiography. Early surgical treatment (neonatal period, if possible) is safe and associated with the best long-term results, even in the presence of other cardiac anomalies. Complete separation and reconstruction of both aorta and pulmonary arteries under direct vision may prevent recurrence and distortion of adjacent structures.     

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.     

Complete Extirpation of a Bronchogenic Cyst Causing Recurrent Laryngeal Nerve Palsy by Thoracoscopy: Report of a Case

We excised a bronchogenic cyst causing recurrent laryngeal nerve palsy using thoracoscopic surgery. A 28-year-old woman presented after the sudden onset of hoarseness, and laryngoscopic examination showed left vocal cord palsy. Computed tomography and magnetic resonance imaging showed a cystic mass, 4 cm in diameter, in the aortopulmonary window. Thoracoscopic examination revealed that the mass was adhered to the recurrent laryngeal nerve below the aortic arch. We extirpated the cyst via thoracoscopy without any injury to the nerves or major blood vessels. This case illustrates the benefits of thoracoscopic surgery for providing good visualization of the perineural structures and as a safe surgical treatment for a cystic mass in the aortopulmonary window.     

Berry syndrome—a rare congenital cardiac anomaly

Berry syndrome is a rare congenital cardiac anomaly, characterized by distal aortopulmonary window, hypoplasia or interruption of the aortic arch, intact ventricular septum, and aortic origin of the right pulmonary artery and patent ductus arteriosus. Anatomic depiction of each component is important for the diagnosis. Single-stage surgical repair is challenging but feasible with good survival outcomes. The available literature on this anomaly is limited. Hence, this paper aims at reviewing the literature on Berry syndrome.     

Correction of Absence of the Aortopulmonary Septum by Creation of Concentric Great Vessels

An 18-year-old patient is reported who had simultaneous occurrence of aortopulmonary septal absence, complete interruption of the aortic arch, and patent ductus arteriosus. This is the only known patient who has had total anatomical correction of this combination of defects. Correction was accomplished by staged procedures; the first stage consisted of obliteration of the patent ductus and establishment of aortic continuity; this was followed one year later by the second stage, which included a unique method for repair of absence of the aortopulmonary septum. The surgical desirability and embryological implications of criteria for differentiating truncus arteriosus from aortopulmonary septal defect are presented. Follow-up physiological evaluations and the patient's death from the effects of increasing pulmonary vascular disease (despite early symptomatic improvement) approximately two years after completion of the repair underscore the importance of early diagnosis and operative intervention.     

Operations for subaortic stenosis in univentricular hearts

Optimal prevention and treatment of subaortic stenosis (SAS) in the univentricular heart with subaortic outlet chamber and high pulmonary blood flow remains controversial, especially when complicated by aortic arch obstruction. Herein we analyze our surgical results. Group 1 consisted of 11 infants (mean age, 10 days) with univentricular heart and SAS. Ten required repair of interrupted aortic arch (n = 7) or coarctation with hypoplastic arch (n = 7). Four patients had relief of SAS by either Damus-Kaye-Stansel connection (n = 2) or aortopulmonary window (n = 2), with three operative deaths and one late death. Six had one-stage arterial switch and atrial septectomy with arch repair (5/6) with one operative death and one late death. Two survivors have progressed to bidirectional cavopulmonary shunt, a third has had a Fontan operation, and a fourth awaits Fontan. In group 2, 11 children required operation for acquired SAS after pulmonary artery banding. Nine have progressed to Fontan operation with either staged (n = 3) or concurrent (n = 6) relief of SAS by Damus-Kaye-Stansel connection or subaortic resection. Fontan mortality was 11% (70% confidence limits, 2% to 32%). Group 3 consisted of 3 patients without pulmonary artery banding who had SAS diagnosed at Fontan evaluation. All 3 survived Fontan operation and relief of SAS by Damus-Kaye-Stansel connection or subaortic resection. Group 4 consisted of 1 patient with previous pulmonary artery banding (no SAS) who underwent Fontan operation but required Damus-Kaye-Stansel connection 30 months later for SAS.(ABSTRACT TRUNCATED AT 250 WORDS)     

Biventricular repair for aortic atresia or hypoplasia and ventricular septal defect.

OBJECTIVE: Aortic valve atresia or hypoplasia can present with a ventricular septal defect and a normal mitral valve and left ventricle. These patients may be suitable for biventricular repair, although the optimal initial management strategy remains unknown. METHODS: From January 1991 through March 1999, 20 patients with aortic atresia or hypoplasia and ventricular septal defect underwent operation with the intent to achieve biventricular repair. Aortic atresia was present in 7 patients, and aortic valve hypoplasia was present in 13 patients. Among those patients with aortic hypoplasia, Z-scores of the aortic valve anulus ranged from -8.8 to -2.7. Associated anomalies included interrupted aortic arch (n = 12 patients), coarctation (n = 6 patients), aortopulmonary window (n = 1 patient), and heterotaxia (n = 1 patient). Nine patients were staged with an initial Norwood procedure followed by biventricular repair in 8 patients. One patient awaits biventricular repair after a Norwood procedure. The conditions of 11 patients were corrected with a single procedure. RESULTS: Among the 9 patients who underwent staged repair, there were no deaths after the Norwood procedure and 1 death after biventricular repair. For the 11 patients who underwent a primary biventricular repair, there was 1 early death and 2 late deaths from noncardiac causes. Follow-up ranged from 1 to 85 months (mean, 28 months). Actuarial survival for the entire group was 78% +/- 10% at 5 years and was not significantly different between staged repair (89%) and primary biventricular repair (73%). CONCLUSIONS: Both primary and staged biventricular repair for patients with aortic atresia or hypoplasia and ventricular septal defect may be performed with good late survival. Refinements in technique of conduit insertion and arch reconstruction have resulted in primary biventricular repair becoming our preferred approach.     

Current outcomes of one-stage surgical correction for Berry syndrome

Objectives

Berry syndrome is a combination of distal aortopulmonary window (APW), aortic origin of the right pulmonary artery (RPA), intact ventricular septum, and interrupted aortic arch. We present here our current experience of primary repair of this syndrome with the goal of optimizing treatment for this rare condition.