Successful complete repair of interrupted aortic arch and associated with DiGeorge syndrome in neonate]

We report a rare case of interrupted aortic arch and a right aortic arch associated with DiGeorge syndrome, in neonate. Through a median sternotomy bypass was established placing an arterial perfusion cannula both in the ascending aorta, and in the main pulmonary artery. The right and left pulmonary arteries were temporarily occluded, while this pulmonary cannula perfused the lower part of the body. The arch reconstruction was performed during profound hypothermic total circulatory arrest. The right descending aorta had an adequate length and direct anastomosis was carried out without any tension. The VSD was repaired through a right atrial approach. The patient had hypocalcemia and thymic abnormalities which was consistent with the DiGeorge syndrome. He was treated with calcium gluconate and alfacalcidol, but no serious infection due to immunodeficiency was seen after operation. Post operative catheterization revealed no pressure gradient at the site anastomosis of the aortic arch and satisfactory results.     

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.     

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.     

Primary repair of interrupted aortic arch and severe aortic stenosis in neonates

Two infants, aged 36 days old (Case 1) and 18 days old (Case 2) with interrupted aortic arch types B and A, respectively, and with severe aortic stenosis, were successfully operated on by use of pulsatile cardiopulmonary bypass. The great arteries were normally related in Case 1 and were transposed in Case 2. Repair involved the following procedure: ligation of the patent ductus arteriosus, restoration of aortic continuity with an 8 mm polytetrafluoroethylene graft, placement of an internal patch to tunnel all left ventricular blood from the left ventricle through the ventricular septal defect into the pulmonary artery in Case 1 and patch closure of the ventricular septal defect in Case 2, transection of the main pulmonary artery, anastomosis between the proximal pulmonary artery and the ascending aorta, and interposition of a valved conduit between the right ventricle and the distal pulmonary artery. The operative field could be approached easily through a median sternotomy. Postoperative cardiac catheterization revealed satisfactory anatomical and hemodynamic results in both cases.     

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.     

Simplified method for total correction of interrupted aortic arch with ventricular septal defect in infancy.

Type A interrupted aortic arch with a ventricular septal defect (VSD) in a 3-month-old infant was successfully corrected with the aid of profound hypothermia, limited cardiopulmonary bypass, and total circulatory arrest. The aortic arch was reconstructed by side-to-side anastomosis of the ascending aorta and the main pulmonary artery and then creation of a tube from the anastomotic orifice to the patent ductus arteriosus (PDA) by using a superabundant flap of the anterior wall of the main pulmonary artery. The pulmonary arteriotomy and VSD then were closed. The operative field can be approached easily through a median sternotomy with minimum dissection. Cardiac catheterization and angiocardiography 2 months later demonstrated a satisfactory reconstruction of both the aortic arch and the pulmonary artery.     

Repair of persistent truncus arteriosus with interrupted aortic arch.

OBJECTIVE: The aim of our study was to analyse experience with repair of truncus arteriosus with interrupted aortic arch. METHODS: Between 1993 and 2004, eight consecutive patients underwent repair of truncus arteriosus with interrupted aortic arch. The median age was 6.5 days (range 1-85 days) and median weight was 3.2 kg (range 2.6-4.8 kg). Five patients had type A and 3 patients had type B aortic arch interruption. The repair was performed in deep hypothermia with circulatory arrest in 4 patients and isolated selective low-flow perfusion of the head and the heart in the last 4 patients. The repair consisted in aortic arch reconstruction by direct anastomosis between descending and ascending aorta, closure of ventricular septal defect and reconstruction of the right ventricular to pulmonary artery continuity using a valved conduit. RESULTS: One (12.5%) patient died from sepsis and hepato-renal failure 18 days after surgery. Seven (87.5%) patients were followed up for 2.0-11.7 years (median 2.6 years). No patient died after the discharge from hospital. In 4 patients 1-3 reinterventions were required 0.6-10.0 years after repair. Reoperations were performed for conduit obstruction in 2 patients, aortic regurgitation in 2 patients, right pulmonary artery stenosis in 2 patients and airway obstruction in 1 patient. In 2 patients concommitant aortic valve and conduit replacement was required. Balloon angioplasty for aortic arch obstruction was necessary in 1 patient, and for bilateral pulmonary branch stenosis in 1 patient. Five (28.6%) surviving patients are in NYHA class I and 2 (71.4%) patients are in NYHA class II. CONCLUSIONS: Primary repair of persistent truncus arteriosus with interrupted aortic arch can be done with low mortality and good mid-term results. Aortic arch reconstruction in isolated low-flow perfusion of the head and the heart influences favourably the postoperative recovery. The main postoperative problems are associated with conduit obstruction and aortic insufficiency.     

Arterial switch operation with ventricular septal defect repair and aortic arch reconstruction

The arterial switch operation for transposition of the great arteries or double outlet right ventricle with ventricular septal defect (VSD) and aortic arch obstruction is a challenging procedure. One-stage neonatal repair is preferred; however, palliation may be indicated in the newborn who presents with Swiss cheese septum or the patient with extracardiac complications such as necrotizing enterocolitis or subarachnoid hemorrhage. The aortic arch repair is performed with continuous cerebral perfusion and includes patch enlargement of the transverse aortic arch and ascending aorta to control for the important diameter mismatch between the aorta and the pulmonary root. The VSD is no longer closed through the pulmonary valve. Instead, the VSD is approached through the aortic valve after harvesting of the coronary buttons and/or through the tricuspid valve and, if necessary, through an infundibulotomy. The coronary artery transfer is often complex because of the presence of unusual coronary artery patterns and requires precise technique. Subaortic obstruction is often present and requires correction. The right ventricle can be mildly hypoplastic but is not a contraindication to repair unless there is important tricuspid stenosis.     

Right aortic arch and isolated left innominate artery from a left sided patent ductus arteriosus: a very rare aortic arch anomaly

Malformation of the aortic arch system has been described in details by Stewart et al. in 1964. Innominate artery originating via the ductus arteriosus from the pulmonary artery is a very rare type of congenital aortic arch anomaly that has been seldomly reported. We report the case of an aortic arch anomaly revealed by a pulmonary hypertension because of left to right shunt. Surgical procedure was performed through a median sternotomy, without cardiopulmonary bypass. After section. of the ductus arteriosus, the left innominate artery was extensively dissected and mobilized to be implanted on the left side of the ascending aorta under lateral clamping.     

Coarctation and hypoplasia of the aortic arch: will the arch grow?

Hypoplasia of the transverse aortic arch of various degrees of severity is commonly seen in infants who have coarctation of the aorta. It is more often present when the coarctation is associated with intracardiac lesions that diminish or limit forward flow in the ascending aorta and promote right to left flow through an arterial duct. The increased frequency of surgical treatment of infants with complex coarctation, which is in part related to the ability to stabilize their condition with prostaglandin E1, has posed the question of the potential for growth and development of the originally hypoplastic aortic arch after conventional repair of aortic coarctation. Review of our experience with transverse aortic arch hypoplasia, found in 33 (32%) of 102 infants undergoing coarctation repair by subclavian flap aortoplasty or classic resection and end-to-end anastomosis, revealed excellent growth of the transverse arch after repair in all patients available for linear follow-up. The currently proposed extended arch repair should be reserved for the small group of infants with transverse aortic arch to ascending aorta diameter ratios (arch indices) of less than 0.25.     

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)     

Reparative operations for interrupted aortic arch with ventricular septal defect

From January, 1975, through September, 1982, 24 infants underwent primary or staged repair of interrupted aortic arch (IAA) with ventricular septal defect (VSD). Seven patients had IAA type A and 17 patients had type B. Eleven of the patients, median age 5 days, underwent staged operations and 13 infants, median age 6 days, underwent primary repair. Palliation was by tube graft interposition (six), subclavian-aortic anastomosis (three), left carotid-aortic anastomosis (one), or end-to-side aortic anastomosis (one) combined with pulmonary artery banding (eight) or early VSD closure. With palliation, there were three (27%) early deaths among the eleven patients and one (13%) late death among the eight remaining. Delayed repair at 5 days to 14 months (median 7 months) in seven patients incurred three (43%) early and no late deaths. Primary repair in 13 patients consisted of VSD closure combined with graft interposition (12) or end-to-side aortic anastomosis (one), with three (23%) early and no late deaths. Nine of 14 survivors had hemodynamic evaluation by catheterization 1 to 3 years following repair. None had a significant residual VSD or pressure gradients between the ascending and thoracic aorta. Six had subaortic stenosis, two mild (gradient less than 20 mm Hg) and four severe (gradient greater than 50 mm Hg), necessitating operation. Results of operations in neonates with IAA continue to improve. Essential in management is an awareness that subaortic stenosis and hypocalcemia may be accompaniments of this anomaly. Based on these data, we prefer primary repair for IAA with VSD.     

Total aortic arch replacement for distal aortic arch aneurysm with adhered left lung: report of a case

A 76-year-old man with a history of pleuritis and a cerebral infarct underwent a total arch replacement for an aneurysm of the distal aortic arch. Computed tomography revealed a true aneurysm having a maximum diameter of 70 mm as well as pleural thickening and calcification. Total arch replacement with selective cerebral perfusion was performed through median sternotomy and left thoracotomy. The myocardial ischemic time was shortened by reperfusion from the right axillary artery after the anastomosis of the proximal ascending aorta and the brachiocephalic artery. After the other branches of the arch were anastomosed, a distal anastomosis through the left thoracotomy was then performed using the pull-through method, enabling minimal decortication of the adhered lung. Our surgical procedure for distal arch aneurysm with adhered lung involves the addition of a left thoracotomy, but the reduction in the myocardial ischemic time and lung injury are of benefit.     

Surgical management of patients with interrupted aortic arch and severe subaortic stenosis

Ten patients underwent palliative surgery for interrupted aortic arch and severe subaortic obstruction due to posterior displacement of the conal septum. Their ages ranged between 4 and 28 days (mean, 11.0 +/- 7.7 days) and their weights, between 2.1 and 4.2 kg (mean, 2.85 +/- 0.6 kg). Preoperative echocardiography and cardiac catheterization were performed on all patients. The ratios of the left ventricular outflow tract diameters and the ascending aortic diameters to the descending aortic diameters were 0.56 +/- 0.03 and 0.56 +/- 0.06, respectively, compared with 0.81 +/- 0.12 and 0.95 +/- 0.17, respectively, in 20 patients with interrupted aortic arch but without obstruction (p less than 0.001). Four of the 10 patients underwent pulmonary artery banding and insertion of a bypass graft between the ascending and the descending aorta. All 4 died of low cardiac output soon after operation (100% operative mortality). The remaining 6 patients underwent banding and insertion of a graft between the main pulmonary artery proximal to the band, and the descending aorta. All of these patients survived, and all except 1 are doing well 3 months to 4 years postoperatively. The use of a pulmonary artery-descending aorta conduit and of distal pulmonary artery banding provides good palliation for patients with interrupted aortic arch and major subaortic stenosis.     

A case of atherosclerotic aneurysm of the right subclavian artery with the right axillary arterial stenosis and enlargement of the ascending aorta

A 54-year-old man presented with neck pain and hoarseness. Angiography showed a rare right subclavian artery aneurysm, enlargement of the ascending aortic aneurysm, and axillary artery stenosis. These aneurysms would normally be treated with end-to-end anastomosis, but due to the complexity of the lesion in this case, we performed more extensive surgery. This consisted of successful reconstruction of the subclavian artery and replacement of the ascending aorta and aortic arch.     

自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并弓发育不良

目的 探讨缩窄段切除加自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并主动脉弓发育不良的方法和效果.方法 2007年5月至2009年12月,14例主动脉缩窄合并主动脉弓发育不良病婴行主动脉缩窄段切除加自体肺动脉补片主动脉弓成形手术,其中男9例,女5例;年龄23天至17个月,中位值4.33月龄;平均体重(6.14±2.36)kg.所有病婴均诊断为合并室间隔缺损的主动脉缩窄,同时存在主动脉弓发育不良.手术在深低温体外循环下完成,其中8例采用选择性脑灌注技术,6例停循环.主动脉成形采用新鲜的自体肺动脉补片.同期修补合并的室间隔缺损.结果 围术期死于循环衰竭1例.13例生存,其中1例合并低心排血量综合征,经相应治疗恢复.术后超声心动图检查主动脉弓无残余梗阻.随访4个月至3年.超声心动图示主动脉弓压力阶差均<16 mm Hg(1 mm Hg=0.133 kPa),随访期间主动脉弓降部血流速度与出院时无明显变化.CT扫描显示主动脉弓几何构形正常;术后半年原左主支气管受压者症状明显改善或完全消失.无主动脉夹层动脉瘤发生.结论 缩窄段切除加自体肺动脉补片主动脉弓成形是治疗婴儿主动脉缩窄合并主动脉弓发育不良理想的手术方法.

Abstract：

Objective To discuss the operative techniques and results of coarctation resection plus aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant.Methods Between May 2007 and Dec 2009,14 cases including 9 males and 5 females with caorctation of the aorta and hypoplastic aortic arch underwent coarctation resection plus aortoplasty with pulmonary autograft patch in our hospital.The age ranged from 23 days to 17 months,with a median of 4.33 months.The mean body weight was (6.14 ±2.36) kg.All patients were diagnosed as aortic coarctation combined with VSD and hypoplastic aortic arch.The surgery was performed under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion in 8 cases and circulation arrest in 6 cases.Fresh pulmonary autograft patch harvested from the main pulmonary artery was used for aortoplasty.The associated VSD was repaired in the same stage.Results All patients survived except one died from circulatory failure during the perioperative period.Low cardiac output syndrome occurred in another case who was cured afterwards by correspondent treatments.No residual obstruction was detected by echocar-diography after the operation.Follow-up was carried out in 13 cases from 4 months to 3 years.Echocardiographic examination showed that the pressure gradient across the aortic arch was less than 16 mm Hg in all cases.The blood velocity at the descending aortic arch was not significantly changed during the follow-up period as compared with that of the immediate after operation.Computed tomography showed that the morphology of aortic arch was normal.The left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before operation,and no aortic aneurysm were detected in these patients.Conclusion Conclusion Coarctation resection plus aortoplasty with pulmonary autograft patch is the optimal surgical method for treating coarctation of the aorta combined with hypoplastic aortic arch in infant.     

Right aortic arch, right ligamentum, absent left pulmonary artery: a rare vascular ring.

A 5-month-old infant presented with respiratory failure secondary to severe right bronchial compression. Diagnostic imaging revealed a right aortic arch and absent left pulmonary artery. Surgical relief was obtained via median sternotomy by dividing a right ligamentum and pexing the enlarged right pulmonary artery to the ascending aorta.     

Single-stage repair of aortic coarctation with ventricular septal defect using isolated cerebral and myocardial perfusion.

OBJECTIVE: To avoid hypothermic circulatory arrest, we have repaired aortic coarctation with ventricular septal defect (VSD) in a one-stage procedure using an isolated cerebral and myocardial perfusion technique, and retrospectively compared this novel approach to the conventional two-stage approach. METHODS: Between October 1991 and February 1999, 24 infants, aged 4-137 days (median, 27 days) and weighing 1.7-4.3 kg (median, 3.0 kg), underwent the repair of aortic coarctation with VSD either in one (group I, n=11) or two stages (group II, n=13). In Group I, an arterial cannula for cardiopulmonary bypass was inserted into the ascending aorta in six patients with coarctation only, or into a polytetrafluoroethylene (PTFE) graft which was anastomosed to the innominate artery in the remaining five who had hypoplastic arches. A cross-clamp was placed between the innominate and left carotid arteries. The bypass flow was reduced to 30-50% of full flow at 28 degrees C, thereby maintaining a radial artery pressure of 30-45 mmHg. At this point, the aortic coarctation was repaired by an end-to-end arch anastomosis, while maintaining brain perfusion and with the heart still beating. In five patients with hypoplastic aortic arches, the innominate artery proximal to the graft was then secured down and the arch anastomosis was extended to the distal ascending aorta, while providing isolated cerebral perfusion and cardioplegic arrest. After arch reconstruction was performed, the clamp was moved onto the ascending aorta, and the VSD was closed with systemic perfusion. In contrast, for group II patients, coarctation repairs were performed through a posterolateral approach, and existing VSDs were closed as secondary procedures. RESULTS: The mean isolated cerebral and myocardial perfusion time for group I was 13 min (range, 7-20 min). The myocardial ischemic time did not differ between groups I and II (43+/-4 vs. 42+/-5 min, not significant). There were no hospital mortalities or neurological complications in either group, but one late death in each group. CONCLUSION: Single-stage repair of aortic coarctation with VSD does not increase myocardial ischemic time compared to the traditional two-stage approach. The isolated cerebral and myocardial perfusion technique may offer substantial brain and myocardial protection during aortic arch reconstruction.     

One-stage repair with separated cardiopulmonary bypass for coarctation of the aorta with left aortic arch and right thoracic descending aorta

We treated a 7-day-old neonate with aortic coarctation, left aortic arch, right-side descending aorta, aberrant right subclavian artery, and outlet ventricular septal defect. Surgical one-stage repair consisting of coarctation repair by extended direct anastomosis, patch closure of the ventricular septal defect, and translocation of the right subclavian artery was performed under a separated (upper and lower body) perfusion with ascending and thoracic descending aortic cannulation. The postoperative course was complicated with massive chylous effusion, which was successfully treated by thoracic duct ligation.     

Combined heart transplantation and resection of dissecting aneurysm of ascending aorta and aortic arch: a case report.

A 21-year-old male patient had suffered from palpitation and exertional dyspnea since October, 1997. He was admitted to our hospital, and a series of examinations were performed. Chest computed tomography (CT) revealed marked dilatation of the ascending aorta (about 7.5 cm at the proximal portion) and aortic annulus, an intimal flap in the ascending aorta and aortic arch was also noted. Cardiac catheterization revealed the pulmonary capillary wedge pressure was 33 mmHg, pulmonary artery pressure was 47/38 mmHg with a mean of 35.4. The cardiac index was 1.01 l/min/m2. Poor left ventricular contractility was shown by a left ventricular ejection fraction (LVEF) of 13.8% and a right ventricular ejection fraction (RVEF) of 5.13% by a radionuclide angiogram (RNA) study. Under the diagnosis of dilated cardiomyopathy and dissecting aortic aneurysm of the ascending aorta and aortic arch, he was put on a waiting list for heart transplantation. On November 11, 1997 he received heart transplantation. Resection of the dissecting aneurysm of the ascending aorta and the aortic arch and replacement with a 26 mm Vascutek graft were performed first under deep hypothermia and retrograde cerebral perfusion. Then while he was rewarming up, heart implantation was performed. He was discharged 30 days after surgery and has been doing well since then. As far as we know, no literature regarding combined heart transplantation and resection of a dissecting aneurysm of the ascending aorta and aortic arch has been reported.