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.     

Coarctation of the aorta with right aortic arch: surgical technique and new classification.

BACKGROUND: The combination of right aortic arch and coarctation of the aorta has seldom been reported. This rare abnormality occurs as an isolated lesion or in association with other congenital defects, such as mixed gonadal dysgenesis and Turner's syndrome. METHODS: The medical records of 2 patients who underwent operation for right aortic arch and aortic coarctation in our institution were reviewed together with case reports in the literature. Various surgical options have been reported: synthetic grafts, subclavian flap, and end-to-end anastomosis. End-to-end anastomosis is our method of choice for coarctation of the aorta and for right aortic arch with coarctation, even though an excessive amount of dissection is needed. RESULTS: Both patients from our institution are doing well, with no sign of recoarctation in either patient. Our experience and recent advances in the understanding of the anatomy of this lesion led us to develop an alternative simplified classification for right aortic arch, which is presented here. CONCLUSIONS: Right aortic arch and coarctation of the aorta is a rare morphologic combination. On the basis of our experience with repair of coarctation of the aorta and our review of the literature, we think that end-to-end anastomosis is the operative technique of choice with the best long-term results. Our simplified classification is easy to understand when dealing with right aortic arch.     

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.     

Palliative repair of aortic atresia associated with tricuspid atresia and transposition of the great arteries.

Successful palliative repair of aortic atresia and hypoplastic aortic arch associated with tricuspid atresia in a neonate is described. The repair consisted of reconstruction of the hypoplastic aortic arch with an equine pericardial patch, division of the patient ductus arteriosus, connection of the pulmonary artery to the aorta, implantation of the proximal part of the ascending aorta into the main pulmonary artery, and anastomosis of a polytetrafluoroethylene graft 5 mm in diameter between the right ventricular outflow tract and the central pulmonary artery, which was transferred anteriorly to the main pulmonary artery.     

Protection of visceral organs with an aortic occlusion balloon catheter and by femoral perfusion in aortic arch replacement

A 68-year-old man developed hemoptysis. Prominent left 1st arch was pointed out by chest X-p, and enhanced computed tomography (CT) revealed 60-mm distal arch aneurysm. Protrusion of the aneurysm strongly suggested aorto-pulmonary fistula. The operation was performed via median sternotomy. Cardiopulmonary bypass was established with the ascending aorta cannulation. During systemic cool down, an aortic occlusion balloon (Equalizer) was inserted in the right femoral artery and its position was monitored by transesophageal echography. Femoral perfusion cannula was inserted in the right femoral artery distal to the site of the Equalizer insertion. When the rectal temperature reached 28 degrees C, Equalizer was inflated and femoral perfusion was started. After the aortic arch was incised, balloon-tipped cannula were inserted into the aortic arch vessels. At this point, separate antegrade cerebral perfusion and lower body perfusion were established. The aortic arch was replaced with woven Dacron tube graft. It took 60 minutes to accomplish distal anastomosis, and urination was 80 ml during this period. Postoperative course was uneventful without renal dysfunction. This method of visceral organ protection seemed to be useful for the patients with impaired renal or liver function and for the patients whose distal anastomosis is difficult.     

Hypothermic circulatory arrest through a left thoracotomy in a 12-year-old child with aortic coarctation

Surgical correction of adult complex aortic coarctation using hypothermic circulatory arrest often requires central cannulation to secure cerebral perfusion. It is not easy to place the cannula in the ascending aorta, however, especially in children undergoing surgery through a left thoracotomy. In a 12-year-old male with hypoplastic distal aortic arch, we placed an arterial cannula in the ascending aorta using the Seldinger puncture technique through the stenotic segment of the distal aortic arch. Replacement of the stenotic segment with a 20 mm-size Dacron graft was then routine. The ascending aorta was exposed only for the proximal anastomosis. The left subclavian artery was also reconstructed. This central cannulation technique is simple and is useful in repairing complex aortic coarctation.     

Palliative reconstructive surgery for hypoplastic left heart syndrome

From August, 1985, through August, 1987, 104 consecutive, nonselected neonates underwent palliation of hypoplastic left heart syndrome. The technique included pulmonary artery homograft augmentation of the diminutive ascending aorta and aortic arch, atrial septectomy, transection of the main pulmonary artery with patch closure of the distal main pulmonary artery, anastomosis of the proximal main pulmonary artery to the augmented ascending aorta, and a 4-mm, modified, right Blalock-Taussig (N = 21) or central (N = 83) shunt. There were 30 early and 11 late deaths. Early mortality was most commonly associated with hypoventilation. Complications included development of aortic arch obstruction (N = 11) and progressive hypoxemia (N = 11). Alterations in surgical techniques and perioperative management should permit continued improvement in early and long-term survival.     

Vascular rings and slings. Diagnosis and surgical treatment of 49 patients

Forty-nine infants with symptomatic vascular rings and slings, ranging in age from 20 days to 12 months, required surgical intervention between 1973 and 1984. The following anomalies were present in our patients: double aortic arch with left descending aorta (14), double aortic arch with right descending aorta (6), anomalous innominate artery (13), right aortic arch with aberrant left subclavian artery (4), left aortic arch with aberrant right subclavian artery (10), aberrant left pulmonary artery (pulmonary sling) (2). All the babies had symptoms related to compression of the trachea and/or esophagus. Four patients required temporary tracheostomy in the early postoperative period; 1 patient, affected by a pulmonary sling, required tracheal resection and anastomosis, for severe tracheomalacia. There was one hospital death in a patient with severe tracheal compression from an anomalous innominate artery and brain damage as a result of metabolic problems. Forty-eight patients survived and follow-up ranged from 3 months to 11 years. For each type of vascular anomaly encountered, and based on personal experience, we have outlined a diagnostic scheme allowing an accurate morphological definition and a subsequent surgical procedure.     

Ascending aortic origin of a branch pulmonary artery--surgical management and long-term outcome.

OBJECTIVE: Ascending aortic origin of a branch pulmonary artery (AOPA, hemitruncus arteriosus) is a rare congenital malformation. While there have been isolated case reports, larger series, relating to long-term outcomes following surgery are few. This article analyses the surgical results of a series of nine patients, over a period of 29 years. METHODS: Between 1974 and 2003, nine patients [neonates, 6; infants, 3; male, 5; female, 4] were operated on for AOPA. Median age at presentation was 14 days (range birth to 231 days). Six [corrected] patients (group 1) had associated simple lesions like patent ductus arteriosus or right aortic arch. Three patients (group 2) had complex lesions with right ventricular outflow tract obstruction. One patient (group 2) had DiGeorge syndrome. All patients except group 2 presented with congestive cardiac failure and, in addition one had pre-operative coronary ischemia. Diagnosis was established by angiocardiography in two patients and by echocardiography in seven [corrected] The median age at operation was 28 days (range 7-365). Follow-up period ranged from 7 months to 20.5 years (median 9 years). RESULTS: All nine patients had an anomalous right pulmonary artery (RPA) arising from the proximal ascending aorta, while the left branch was of right ventricular origin. All had evidence of pulmonary hypertension or elevated right ventricular pressure pre-operatively. There was no operative mortality. Of eight patients who had direct anastomosis of the RPA to the main pulmonary artery, one required patch enlargement and another required stenting of an anastomotic stenosis. One patient had a RV-RPA conduit, which required replacement 8, 13, and 14 years later. At follow-up, all patients were alive. All patients in group 1 had normal haemodynamic function and were in NYHA class I. In group 2, all were in NYHA class II with evidence of right ventricular hypertrophy. Four patients had post-operative ventilation-perfusion scans which showed satisfactory perfusion to both lungs. CONCLUSIONS: Early surgery is indicated in this lesion and is compatible with good long-term outlook. Surgical repair should not be deferred for corrective procedures of associated cardiac anomalies.     

Successful surgical repair of truncus arteriosus with interrupted aortic arch in infancy by an anterior approach

A 98-day-old infant was successfully operated on for truncus arteriosus (type I) with interrupted aortic arch (type B) using a one-stage anterior approach. The interrupted aortic arch was reconstructed by direct anastomosis between the ascending and descending aorta. The ventricular septal defect was closed, and a 12-mm porcine valved conduit was placed on the right side of the ascending aorta to establish continuity between the right ventricle and pulmonary artery. Surgical procedures for the repair of this malformation are the focus of discussion.     

Surgery for thoracic aortic aneurysms involving the distal arch through a left thoracotomy

Surgical outcome for thoracic aortic aneurysms involving the distal arch via a left thoracotomy using retrograde cerebral perfusion combined with profound hypothermic circulatory arrest was reviewed. Twelve patients with a atherosclerotic aortic aneurysm between 1994 and 1997 were involved. A proximal aortic anastomosis was made by means of an open aortic technique. For the first four patients, oxygenated arterial blood from cardiopulmonary bypass was perfused retrogradely through a venous cannula positioned into the right atrium. In the last eight cases, venous blood provided by a low-flow perfusion of the lower half body via the femoral artery, which was still oxygen-saturated, was circulated passively in the brain in a retrograde fashion with the descending aorta clamped. Prosthetic replacement was done between the distal arch and the proximal descending aorta in 6 patients and from the distal arch to the entire descending thoracic aorta in 6 patients. The median duration of hypothermic circulatory arrest and continuous retrograde cerebral perfusion was 36 minutes and 33 minutes respectively. The overall outcome was satisfactory without early mortality--all patients survived, although an octogenarian died of respiratory failure 1 year postoperatively. Another octogenarian with a ruptured aneurysm developed delay of meaningful consciousness, and other two patients with a severely atherosclerotic aneurysm suffered permanent neurological dysfunction (stroke) presumably due to an embolic episode. The safe and simple combination of profound hypothermic circulatory arrest, retrograde cerebral perfusion, and open aortic anastomosis protects the brain adequately and produces satisfactory results in surgery for aortic aneurysms involving the distal arch through a left thoracotomy.     

Repair of acute ascending aorta-arch dissection with continuous body perfusion: a case report

An approach for the replacement of the distal ascending aorta-proximal arch and acute dissection is described. During the operation, the patient's entire body was continuously perfused, the aortic arch was excluded from the arterial circulation, and the aorta was not clamped at any time. To achieve continuous body perfusion, we independently cannulated the right axillary and the left femoral arteries. The right atrium was cannulated for systemic venous return, and the right radial artery was used for arterial blood pressure monitoring. The myocardium was protected with retrograde cardioplegia, and the body was protected with moderate hypothermia. Vascular clamps were placed to the proximal innominate, left carotid, and left subclavian arteries without discontinuing perfusion of the right axillary artery. A temporary clamp was applied to the femoral line, the aorta was transected, and a large Foley catheter was inserted through the true aortic lumen. The Foley bulb was positioned in the proximal descending thoracic aorta and distended with saline until the aortic blood return ceased. The femoral line clamp was removed from the cannula, and the entire body was perfused during the completion of the distal aortic anastomosis. At the completion of the anastomosis, the Foley bulb was slightly deflated. Once the inserted graft was filled with blood, a large vascular clamp was applied to the graft, and the previously placed clamps were removed from the arch branches. The femoral line was removed, and the body was perfused and rewarmed via the axillary cannulation. Following completion of the proximal graft-aortic anastomosis, the heart was reperfused, and all cannulas were removed in the usual fashion. Rapid recovery characterized the patient's initial postoperative course; however, multiple organ failure secondary to pump-induced inflammatory response followed. Aggressive medical management resulted in complete patient recovery. No neurologic deficits were observed, and the patient regained full cognitive function. This report describes a simple approach to facilitate repair of the aortic arch and minimize postoperative organ failure.     

Stent-graft repair of an aortic rupture caused by invasive hemangiopericytoma

A 2.6 kg female with the diagnosis of type "B" interruption of the "right" aortic arch and mitral atresia underwent stage I palliation with translocation and anastomosis of the distal right carotid artery to the descending aorta. The distal main pulmonary artery was anastomosed to the under surface of the neoaortic arch. A modified Blalock-Taussig shunt was constructed between the left side innominate artery and the left pulmonary artery. The child awaits stage II palliation.     

Two different techniques of retrograde cerebral perfusion for thoracic aortic surgery through a left thoracotomy

The authors used profound hypothermic circulatory arrest and continuous retrograde cerebral perfusion for aortic surgery that involved the distal arch through a left thoracotomy. For the first seven patients, oxygenated blood from cardiopulmonary bypass was perfused retrogradely through a venous cannula positioned into the right atrium. In the last 11 cases, venous blood, provided by a perfusion from the lower body, was circulated passively in the brain with the descending aorta clamped. The period of profound hypothermic circulatory arrest was 34.6 +/- 11.1 min, and continuous retrograde cerebral perfusion was 31.3 +/- 11.1 min. Seventeen patients survived, but there was one early death. Two patients with a severely atherosclerotic aneurysm developed permanent neurological dysfunction. The combination of profound hypothermic circulatory arrest, continuous retrograde cerebral perfusion and open aortic anastomosis through a left thoracotomy protects the brain adequately, and facilitates evacuation of debris and air in the aortic arch. It produces satisfactory results for aortic surgery that involves the distal arch.     

Early repair of coarctation of the aorta.

BACKGROUND: Repair of coarctation of the aorta with hypoplasia and elongation of the proximal aortic arch is a technically demanding procedure with a substantial rate of recurrent stenosis at the coarctation repair site. In addition, a high incidence of hypertension has been reported in patients who underwent repair beyond infancy. PATIENTS AND METHODS: Between January 1991 and June 1997, 52 patients (34 neonates and 18 infants with a median age of 37 days; range 2 days to 8 months) with a mean peak systolic upper to lower extremity resting gradient of 33.5 +/- 18.9 mmHg underwent repair of aortic coarctation. The echocardiographically measured median diameter of the aortic arch immediately distal to the innominate artery was 5.4 mm (range 4.0 to 8.1 mm). Eight patients (15%) were considered hypertensive. In 41 patients, through a left thoracotomy, an end-to-side anastomosis was constructed between the descending aorta and the undersurface of the proximal aortic arch. In 12 of these patients (who all had a hypoplastic and elongated aortic arch) this procedure was preceded by the construction of an extended side-to-side left carotid-subclavian arterioplasty. The remaining 11 patients, all with hypoplasia of the aortic arch, had concomitant complete repair of intracardiac anomalies through a median sternotomy. In 8 of these patients, in addition to anastomosis of the descending aorta to the undersurface of the proximal aortic arch, the ascending aorta and aortic arch were augmented with a pulmonary homograft patch. RESULTS: One neonate with associated Shone's syndrome died (2%) on the first postoperative day. There was no late mortality. Early postoperative complications included recurrent laryngeal nerve injury in 1 patient and prolonged chest tube drainage in 4 patients. At a median follow-up of 55 months (range 15 to 92 months), only 3 patients (5. 7%) developed a recurrent stenosis at the coarctation repair site. The remaining 48 patients are free of recurrent stenosis by echocardiography and clinical examination. None of the patients had systemic hypertension. CONCLUSIONS: 1. Coarctation repair consisting of resection of all ductal tissue with end-to-side anastomosis of the descending aorta to the undersurface of the (proximal) aortic arch, if necessary combined with a side-to side left carotid-subclavian arterioplasty, may lead to excellent results. 2. This technique can be applied with low mortality and morbidity via a left thoracotomy if the proximal aortic arch is at least 5 mm in diameter. 3. In view of the low mortality, the low incidence of restenosis at the coarctation repair site, and the absence of substantial morbidity including the development of hypertension, we advocate repair of aortic coarctation at neonatal age or in early infancy to avoid the detrimental sequelae of delayed repair of coarctation, in particular hypertension.     

The Damus-Stansel-Kaye procedure: anatomical determinants and modifications

Seven of 119 patients undergoing anatomical correction for transposition of the great arteries and Taussig-Bing anomalies without pulmonary stenosis had the Damus-Stansel-Kaye procedure and the rest, the arterial switch. The age of the patients having the Damus-Stansel-Kaye procedure ranged from 0.5 year to 5 years (mean age, 2.2 +/- 1 years). Four patients had transposition, 2 had Taussig-Bing anomaly, and 1 had corrected transposition. Indications for the Damus-Stansel-Kaye procedure were side-by-side great arteries associated with difficult coronary anatomy (5 patients), single coronary system (1 patient), and subaortic stenosis (1). A graft between the ascending and descending aorta for interrupted aortic arch made mobilization and posterior displacement of the ascending aorta for the arterial switch difficult. Subaortic stenosis (1 patient), subpulmonary ventricular septal defect (2 patients), and restrictive ventricular septal defect (4) precluded the Rastelli procedure. In 6 patients, the main pulmonary artery was transected at the band, a proximal main pulmonary artery to aorta anastomosis was complemented with a synthetic patch, and a right ventricle to distal main pulmonary artery valved conduit was inserted. Four patients had closure of the aortic outflow. Two patients had postoperative bleeding and 2, heart block. The only patient who did not have transection of the main pulmonary artery, an omission that led to an obstructed conduit at the distal anastomosis, died late. Two patients subsequently needed aortic outflow closure for critical aortic insufficiency. The Damus-Stansel-Kaye procedure has a definite role and can be safely performed in patients with transposition of the great arteries and Taussig-Bing anomalies.(ABSTRACT TRUNCATED AT 250 WORDS)     

The use of pulmonary autograft patch for type A aortic interruption and Swiss-cheese ventricular septal defect (VSD)

The surgical management of the aortic arch pathologies is controversial. Primary anastomosis and patch aortoplasties combined with end-to-end anastomosis have some complications like recurrence and aneurysm formation. Surgical repair of apical muscular (Swiss-cheese) defects is also still under debate. A 6-year-old patient with diagnosis of type A aortic arch interruption and Swiss-cheese ventricular septal defect (VSD) underwent successful intracardiac repair and aortic arch reconstruction. Aortic arch reconstruction was done by end-to-side anastomosis of distal aortic archus and thoracic aorta without cardiopulmonary bypass. The anterior side of the anastomosis was augmented by using pulmonary autograft patch and this patch was extended to the inferior surface of the archus aorta. Swiss-cheese VSD was repaired with a single patch using septal obliteration technique via transatrial approach. Pulmonary autograft patch aortoplasty and end-to-side anastomosis may be an alternative surgical management for surgical repair and it may be done without the need for cardiopulmonary bypass. In these patients associated multiple apical muscular VSDs can be repaired with a single patch, septal obliteration technique.     

A combination of reoperation for pseudoaneurysm following the Cabrol procedure and total aortic arch replacement in a patient with Marfan syndrome —A case with an aberrant right subclavian artery—

A 44-year-old male with Marfan’s syndrome had undergone an initial operation for DeBakey type I acute aortic dissection with annulo-aortic ectasia. He had undergone replacement of the ascending aorta and aortic valve with a composite graft and reconstruction of the coronary artery by the Cabrol procedure. At 5 years after the initial surgery he experienced chest pain and was subsequently examined. Computed tomography revealed a pseudoaneurysm in the ascending aorta and the residual aortic dissection. The maximum diameter of the pseudoaneurysm was 85 mm and the maximum diameter of the aortic arch was 55 mm. The aortic arch was associated with an aberrant right subclavian artery. Angiography revealed that the pseudoaneurysm was caused by leakage at the coronary ostium-graft anastomoses. We repaired the anastomoses and performed total aortic arch replacement with reconstruction of four arch branches. The postoperative course was uneventful without any complications. We report this case because there have been few reports regarding arch replacement in cases with an aberrant right subclavian artery.     

Truncus arteriosus with interrupted aortic arch: successful correction in a neonate

Truncus arteriosus (type II) with interrupted aortic arch (type B) was successfully repaired at 11 days of age using anterior translocation of the pulmonary arteries, resection of the ductus arteriosus, and direct anastomosis between the descending aorta and truncus. This technique permitted wide reconstruction of the aortic arch and minimized the number of suture lines. It also positioned the right ventricle-pulmonary artery conduit anteriorly, which may simplify its subsequent replacement.     

Neonatal aortic arch reconstruction avoiding circulatory arrest and direct arch vessel cannulation.

BACKGROUND: Aortic arch reconstruction in neonates routinely requires deep hypothermic circulatory arrest. We reviewed our experience with techniques of continuous low-flow cerebral perfusion (LFCP) avoiding direct arch vessel cannulation. METHODS: Eighteen patients, with a median age of 11 days (range 1 to 85 days) and a mean weight of 3.2 +/- 0.8 kg, underwent aortic arch reconstruction with LFCP. Seven had biventricular repairs with arch reconstruction, 9 underwent the Norwood operation and 2 had isolated arch repairs. In 1 Norwood and 7 biventricular repair patients, LFCP was maintained by advancing the cannula from the distal ascending aorta into the innominate artery. In 8 of 9 Norwood patients, LFCP was maintained by directing the arterial cannula into the pulmonary artery confluence and perfusing the innominate artery through the right modified Blalock-Taussig shunt fully constructed before cannulation for cardiopulmonary bypass. In 2 patients requiring isolated arch reconstruction, the ascending aorta was cannulated and the cross-clamp was applied just distal to the innominate artery. RESULTS: LFCP was maintained at 0.6 +/- 0.2 L x min(-1) x m(-2) for 41.0 +/- 13.9 minutes at 18.5 degrees C +/- 1.1 degrees C. In 10 of the 18 patients, blood pressure during LFCP was 15 +/- 8 mm Hg remote from the innominate artery (left radial, umbilical or femoral arteries). In 8 of the 18 patients, right radial pressure during LFCP was 24 +/- 10 mm Hg. The mean mixed-venous saturation was 79.8% +/- 10% during LFCP. Two patients had preoperative seizures, whereas none had seizures postoperatively. One patient died. CONCLUSIONS: Neonatal aortic arch reconstruction is possible without circulatory arrest or direct arch vessel cannulation. These techniques maintained adequate mixed-venous oxygen saturations with no associated adverse neurologic outcomes.