Surgical repair for pulmonary artery sling on a 46-day-old infant

Surgical repair on a 46-day-old girl with pulmonary artery sling is reported. She was suffered from dypnea and admitted to a hospital on 12 days after her birth. On chest roentgenogram atelectasis of right lung was found. She had been on respirator since 21 days after her birth. On bronchogram and pulmonary arteriogram, the trachea and right bronchus were compressed and shifted with the anomalous origin of left pulmonary artery which originated from the right pulmonary artery and passed between the trachea and esophagus. These results confirmed the diagnosis of pulmonary artery sling. Hence, she was referred to our hospital for surgical treatment. She underwent surgical repair on 46-day-old. In operation, we chose a mid-sternal splitting incision, and excised 5 mm of ductus arteriosus. Under extracorporeal circulation, the left pulmonary artery was amptated from the right pulmonary artery and pulled back to left side between the trachea and the esophagus. The left pulmonary artery was anastomosed to the main pulmonary artery at the anterior to the left bronchus. She weaned from respirator, and was extubated on the 3rd day after procedure. She recovered uneventfully in post-operative course. On the 24th day after operation she discharged from hospital. On pulmonary perfusion scanning and pulmonary arteriography performed one year after operation, the left pulmonary artery was patient with slightly decreased perfusion in the left lung. Surgical repair for pulmonary artery sling was recognized as high mortality because of frequently associated tracheobronchial anomalies. In the Japanese literature, only 4 patients survived surgically and lived in late stage.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pulmonary artery sling. Results of surgical repair in infancy.

Pulmonary artery sling is a rare congenital vascular anomaly in which the left pulmonary artery originates from the right pulmonary artery and encircles the right main-stem bronchus and distal trachea before entering the hilum of the left lung. This causes compression of the trachea and right main-stem bronchus, and most infants with this anomaly have severe respiratory distress within the first year of life. Between 1953 and 1990 12 infants (nine male, three female) underwent surgical repair of pulmonary artery sling. Ages ranged from 8 days to 9 months (mean age 5 months). Bronchoscopic examination was performed in all patients. Complete tracheal rings were the most common associated lesion (five patients). Nine patients had pulmonary angiography. Most recently, computed tomography and magnetic resonance imaging have been used to diagnose pulmonary artery sling and associated complete tracheal rings when present. Surgical repair consisted of transection of the left pulmonary artery at its origin and implantation into the main pulmonary artery anterior to the trachea via right thoracotomy (one), left thoracotomy (six), or median sternotomy (five). Three patients had simultaneous pericardial patch tracheoplasty for complete tracheal rings. There were no operative deaths. Two late deaths occurred, at 7 months and 2.5 years postoperatively. Of 10 long-term survivors nine have had postoperative studies to determine the patency of the left pulmonary artery. Seven anastomoses were patent (78%). Pulmonary artery sling can be repaired in infancy with low operative mortality and excellent long-term patency of the left pulmonary artery by dividing the left pulmonary artery and implanting it into the main pulmonary artery anterior to the trachea. Simultaneous pericardial patch tracheoplasty should be performed if complete tracheal rings are associated. We recommend repair at the time of diagnosis with median sternotomy and extracorporeal circulation.     

Pulmonary artery sling: reimplantation versus antetracheal translocation.

BACKGROUND: We compared two repair techniques for pulmonary artery sling. The first comprised detachment of the aberrant left pulmonary artery from the right pulmonary artery and its implantation into the main pulmonary artery, and the second, translocation of the left pulmonary artery anterior to the trachea (without implanting it into the main pulmonary artery), resection of tracheal stenosis, and end-to-end reconstruction of the trachea. METHODS: Five symptomatic infants (3 boys and 2 girls; median age 5 months; range, 3 weeks to 11 months) with pulmonary artery sling were operated on through a median sternotomy with aid of cardiopulmonary bypass. In 3 patients, the left pulmonary artery was transected from the right pulmonary artery and implanted into the main pulmonary artery. In addition, the anterior trachea was augmented with a pericardial patch (n = 2). In the remaining 2 patients, associated tracheal stenosis was resected, the left pulmonary artery was translocated anterior to the trachea, and the trachea was reconstructed. RESULTS: All 5 infants survived the operation. The 3 patients in whom the left pulmonary artery was implanted into the main pulmonary artery had an uncomplicated postoperative course. All 3 patients, at a follow-up of 10 months to 7.9 years, were free of symptoms; the left pulmonary artery was documented to be widely patent. The remaining 2 patients in whom the left pulmonary artery was translocated anterior to the trachea could not be extubated. In both patients the distal trachea was compressed anteriorly by the left pulmonary artery. One of these patients died at 1 week postoperatively secondary to tracheal dehiscence. In the other patient, the left pulmonary artery was implanted into the main pulmonary artery with good result; at a follow-up of 3.9 years, mild residual stridor has persisted. CONCLUSIONS: In pulmonary artery sling, implantation of the aberrant left pulmonary artery into the main pulmonary artery, if necessary combined with anterior tracheoplasty, reliably eliminates tracheal and esophageal compression and maintains antegrade flow into the left pulmonary artery. Translocation of the left pulmonary artery anterior to the trachea without implanting it into the main pulmonary artery is not favored because that might result in anterior compression of the trachea. In addition, we are concerned about growth of the circumferential tracheal anastomosis in neonates and infants.     

Primary repair of pulmonary artery sling with double outlet right ventricle and distal tracheal stenosis

The fate of patients with pulmonary artery sling depends on associated tracheal lesions. Distal tracheal stenosis involving the carina frequently results in lethal obstruction due to secretion or inflammatory edema. Further-more, associated complex cardiac anomalies with excessive pulmonary flow make the situation more complicated. We present a case of successful simultaneous one-stage repair of pulmonary artery sling, double outlet right ventricle and distal tracheal obstruction. Pulmonary artery sling was relieved by relocation of the left pulmonary artery (left pulmonary artery to main pulmonary artery) and a tracheoplasty (resection and end-to-end anastomosis). We advocate early aggressive simultaneous repair of pulmonary artery sling with tracheal stenosis and concomitant repair of intracardiac anomalies whenever possible.     

Young child with recurrent stridor

Stridor is a serious clinical sign that warrants immediate attention. We report a young child who presented with recurrent stridor. The diagnosis was confirmed by bronchoscopy, and computed tomography of the thorax showed that tracheal compression at the lower half portion with an anomalous left pulmonary artery arising from the right main pulmonary artery formed a ‘sling’ around the trachea (i.e. left pulmonary artery sling). She had two severe episodes of noisy respiration. The acute respiratory symptoms appeared because of oedema of the airway associated with infection and was aggravated by bronchial secretions. She underwent successful surgical correction of both lesions. Early diagnosis and expert management of airway problems can reduce morbidity and mortality of this potentially lethal clinical scenario.     

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.     

Pulmonary artery sling: results with median sternotomy, cardiopulmonary bypass, and reimplantation

Background. The classic surgical approach to pulmonary artery (PA) sling has been through a left thoracotomy with division of the left PA and reimplantation into the main PA anterior to the trachea. Another approach is anterior left PA translocation with distal tracheal resection. Since 1985, we have repaired PA sling with a median sternotomy approach, cardiopulmonary bypass, and division and reimplantation of the left PA into the main PA with simultaneous repair of associated tracheal stenosis. The purpose of this review is to determine the outcome of that strategy.

Methods. From 1985 to 1998, 16 infants had surgical treatment of PA sling, 14 had left PA division and reimplantation into the MPA, 2 patients had repair using the translocation technique. Mean age at repair was 6.9 months, median age was 4 months. All infants, except 1 with an absent right lung, were operated on at the time of diagnosis. All had rigid bronchoscopy, which revealed associated complete tracheal rings in 12 patients. Seven patients had tracheal repair with pericardial tracheoplasty, 4 had repair using a tracheal autograft technique, and 2 had a distal tracheal resection (one for tracheomalacia). Of the 2 patients having the translocation technique, 1 had a severely hypoplastic right lung and the other had complete absence of the right lung.

Results. There has been no operative mortality. Hospital stay ranged from 5 to 188 days (mean 36 ± 42 days). There was 1 late death 7 months postoperatively from respiratory complications of pericardial tracheoplasty. All left pulmonary arteries are patent and blood flow to the left lung by nuclear scan (n = 10) ranges from 24% to 46% (mean 35% ± 9%).

Conclusion. The strategy of median sternotomy, cardiopulmonary bypass, and left PA division and reimplantation into the main PA with simultaneous tracheal repair has resulted in a low operative mortality and excellent patency of the left pulmonary artery. Results with repair of the commonly associated complete tracheal rings has recently improved with the use of the free tracheal autograft technique.     

Pulmonary artery sling.

Eighteen patients who underwent surgical treatment of pulmonary artery sling at this institution since 1969 were reviewed. Four of them had associated tracheal stenoses produced by complete cartilaginous rings (stovepipe trachea). All patients underwent operation. The median age at operation was 180 days (range, 27 days to 54 months). In the 14 patients with isolated pulmonary sling, the operation consisted of division of left pulmonary artery and reimplantation into the main pulmonary artery anterior to the trachea. In the 4 patients with stovepipe trachea, the stenotic tracheal segment was resected on cardiopulmonary bypass and the left pulmonary artery was brought anterior to the trachea before the latter was reanastomosed to the main pulmonary artery. There were no early deaths. One patient died late. The pulmonary artery anastomosis was patent in all 14 patients investigated postoperatively. Three patients have residual tracheobronchial problems. One patient is mentally retarded and is institutionalized. All other patients are symptom-free and growing normally and have normal chest radiographs.     

Pulmonary artery sling with tracheal stenosis —Primary repair in infancy—

Between 1984 and 1996 five infants underwent surgical repair of pulmonary artery sling associated with severe congenital tracheal stenosis. All infants had symptoms of severe respiratory distress and three of them required ventilator support preoperatively. ages ranged from 2 to 11 months (mean age 6 months). Complete tracheal rings were present in all patients as an associated lesion and right upper lobe tracheal bronchus in 3 patients. The length of tracheal stenosis ranged from 18 to 45 mm (median 40 mm). Three had associated intracardiac anomalies (Scimitar sydrome (1), VSD (1), double-outlet right ventricle with VSD (1), double-outlet right ventricle with pulmonary hypertension (1)). Surgical intervention was carried out through a right thoraco tomy (1) or median sternotomy (4). Cardiopulmonary bypass (CPB) was used in 3 patients and extracorporeal membrane oxgenator (ECMO). in 1. All infants had reimplantation of the left pulmonary artery into the main pulmonary artery left anterior to the trachea. Four patients underwent simultaneous tracheoplasty using costal cartilage grafts and one had complete resection of obstructed trachea between the right upper lobe tracheal bronchus and carina. The length of resected trachea was about 30% of the entire length of the trachea. Three infants underwent simultaneous intracardiac repair. There was no hospital death. All were weaned from ventilatory support and extubated on 1 to 16 months (mean 4, 5 months) postoperatively. AS an additional procedure, aortopexy, removal of granulation tissue or balloon dilatation of the trachea were carried out in one patient each following tracheoplasty using cartilage grafts. There was one late death at 1 year postoperatively. Three of 4 survivors are doing well with no stridor. We adono stridor. We adovocate 1) early aggressive primary repair of pulmonary artrey sling with tracheal stenosis, 2) concomitant repair of tracheal lesion and intracardiac anomalies whenever possible, 3) application of CPB or ECMO to avoid cumbersome intubation technique, and 4) utmost effort to perform tracheal resection and end-to-end anastomosis.     

Pulmonary Artery Sling

A case of pulmonary artery sling in a 1-year-old boy, treated by resection of the left pulmonary artery (LPA) at its origin from the right pulmonary artery (RPA) and reanastomosis of the LPA to the main pulmonary artery (MPA), is reported. In this patient the LPA-MPA anastomosis was demonstrated to be patent in a follow-up pulmonary angiogram six months after the operation. Pulmonary artery sling should be kept in mind when assessing respiratory distress syndrome in children.     

Total arch replacement through a mediansternotomy for an aortic arch aneurysm with an aberrant right subclavian artery

The patient was a 77-year-old man. In 2004, he developed thrombosed aortic dissection extending from the distal aortic arch to the renal artery bifurcation. He was discharged after his condition improved with conservative treatment. He was followed up as an outpatient because there was an aneurysm, which measured 50 mm in diameter, at the aortic arch. Thereafter the aneurysm gradually enlarged. In May 2009, computed tomography (CT) showed that the aneurysm had increased to 10 cm in diameter and the patient began to have back pain. Thus, surgery was planned. CT revealed that the right subclavian artery originated distal to the left subclavian artery and coursed posterior to the esophagus and trachea. Surgery was performed using median sternotomy. Arch replacement and right subclavian artery reconstruction were performed under hypothermic circulatory arrest with selective cerebral perfusion. The right subclavian artery was controlled at the right border of the trachea, and cerebral perfusion was performed at this site. An end-to-side anastomosis to the reconstructed right common carotid artery was performed by an anterior tracheal approach. The patient had no cerebral complications and his postoperative course was uneventful.     

Early and late results of surgical correction of pulmonary artery sling.

Since 1976, we have operated on 4 children with pulmonary vascular sling. They were 5, 12, 19, and 54 months old. All patients were seen initially with severe stridor. Diagnosis was made by barium swallow in all 4. Each child was operated on through a left thoracotomy; the left pulmonary artery was dissected deep between the trachea and esophagus. Systemic heparinization and microsurgical techniques were used. All patients improved after operation. Radioisotope pulmonary ventilation/perfusion scans were performed 9, 9, 14, and 21 months after operation. Decreased ventilation was noted in the right upper lobe of one scan; the other three ventilation scans were normal. Perfusion scans showed good patency of both pulmonary arteries in all 4 patients. It is concluded that pulmonary vascular slings should be treated surgically as soon as diagnosed. Delayed operation can lead to severe tracheomalacia, as demonstrated in our 54-month-old patient.     

One stage operation for aneurysm of the diverticulum of the ductus arteriosus and coronary artery bypass grafting

Aneurysm of the diverticulum of the ductus arteriosus in the adult is rare. One stage operation for aneurysm of the diverticulum of the ductus arteriosis and coronary artery bypass grafting (CABG) is reported. A 61-year-old man was admitted for diagnosis of thoracic aneurysm on chest X-ray and CT. Chest CT scan showed an aneurysm above the left main pulmonary artery. An aortography showed the left vertebral artery originated directly from the aortic arch and a saccular aneurysm arising from the aortic isthmus and lesser curvature of the aortic arch. Coronary arteriography showed 75% stenosis at the right coronary artery (seg. #1) and 75% stenosis at the left anterior descending artery. Operation was performed through a median sternotomy. The aneurysm of 6 to 3 cm was located between the aortic isthmus and left pulmonary artery. Ascending aorta and right atrium were used to institute cardiopulmonary bypass (CPB). CABG (LITA to #7, SVG to #4 PD) was performed. Arterial cannulation was then switched to the left femoral artery. The proximal aorta was cross-clamped between the left vertebral artery and the left subclavian artery under the partial CPB, and the distal aorta was occluded with a occulusive balloon catheter via the right femoral artery. The selective left axillar artery cannulation was performed to perfuse LITA. The aneurysm was resected and closed with a patch. His post-operative course was uneventful.     

Left pulmonary artery sling with right lung aplasia

We report 2 cases of right pulmonary aplasia with left pulmonary artery sling responsible for severe respiratory symptoms. Repositioning of the left pulmonary artery without tracheal surgery was successful in both patients. The postoperative course was simple, and the outcome was favorable at last follow-up (after 2 years and 3 months, respectively). Computed tomography provided an accurate diagnostic evaluation that helped to choose the best surgical technique.     

Pulmonary artery configuration after palliative operations for hypoplastic left heart syndrome

Pulmonary artery architecture and symmetry after palliative operations for hypoplastic left heart syndrome may affect subsequent suitability for a modified Fontan operation. Two-dimensional echocardiography was used to measure pulmonary artery diameter and assess symmetry after two types of systemic-pulmonary artery shunts: modified right Blalock-Taussig shunt (14 patients) and central shunt (from underside of aortic arch gusset to pulmonary artery confluence) (14 patients). Age, weight, preoperative diameter of right and left pulmonary arteries (proximal, middle, and distal segments), and mean interval between preoperative and postoperative echocardiographic studies (20.2 +/- 4.4 days in the Blalock shunt group; 19.1 +/- 6.8 days in the central shunt group) were similar. Early postoperatively, patients with a Blalock shunt showed a significant decrease in the diameter of all pulmonary artery segments except the distal right pulmonary artery. The diameters tapered from distal right to distal left pulmonary artery in this group. Patients with the central shunt had a significant decrease in the diameter of all pulmonary artery segments. There were no significant differences when cross comparisons were made of the various pulmonary arterial segments in patients after a central shunt. Similar findings persisted in 19 patients from both groups who had a late postoperative echocardiogram (mean interval between studies = 271 days in the group of 10 patients with Blalock shunt and 167 days in the group of nine patients with a central shunt). In conclusion, the central shunt preserves pulmonary artery symmetry, which may be important in candidates for the Fontan operation in infancy.     

Experimental en bloc double-lung transplantation

A technique of en bloc double-lung transplantation through a median sternotomy has been developed. Hypothermic circulatory arrest was used after surface cooling in 7 puppies (mean weight, 3.1 kg; range, 1.5 to 4.1 kg). A double lung block, consisting of the two lungs, distal trachea, the main pulmonary artery, and a large cuff of left atrium, was removed from weight-matched donors. After the arrest of the circulation by inflow occlusion, bilateral pneumonectomies were performed in the recipient. The donor double-lung block was then implanted with anastomoses at the distal trachea, the posterior left atrium, and the main pulmonary artery. All animals were successfully rewarmed and had excellent hemodynamic function and gas exchange for periods of up to 24 hours. If primate experiments with long-term survival confirm these initial results, this procedure may have a role in the treatment of patients with end-stage respiratory disease but acceptable cardiac function.     

Anomalous origin of the left coronary artery from the pulmonary artery. Surgical repair of an unusual form

The authors present a case of a 24-year-old woman with anomalous left coronary artery originating from the pulmonary artery. Surgical correction was performed by the direct transposition of the anomalous left coronary artery from the pulmonary artery to the ascending aorta, without interposed graft. Pre- and postoperative evaluations, including angiograms and cycloergometer stress test are presented. The advantages of this technique are discussed, emphasizing the creation of a two-coronary system without the use of prosthetic, arterial or venous grafts. In the present case the left coronary artery originated from the right posterior sinus of the pulmonary artery. The patient is doing well 23 months after operation.     

Catheter-induced pulmonary artery haemorrhage resulting from a pneumothorax

We present a case of pulmonary artery perforation in a patient who developed a pneumothorax after cardiac surgery. In the process of inserting a chest tube the patient became tachypnoeic, and developed haemoptysis. The trachea was intubated, and right bronchial intubation was performed with persistent bleeding. The pulmonary artery catheter was gently withdrawn and the balloon inflated, with cessation of bleeding. The patient was taken to the operating room, a bronchial blocker was placed in the right lower lobe bronchi, and the pulmonary artery catheter was removed. The bronchial blocker was removed the following day with no bleeding. The aetiology of perforation was secondary to the pneumothorax, which caused a shift of the mediastinum to the right, elevated pulmonary artery pressures, and the distal migration of the catheter through the pulmonary artery. It is recommended that treatment include tracheal intubation, inflation of the pulmonary artery catheter balloon, and the placement of a right lower lobe bronchial blocker.     

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.     

Long segment congenital tracheal stenosis in twins successfully treated by slide tracheoplasty

Long segment congenital tracheal stenosis (LSCTS), associated with complete tracheal rings, is a rare condition, difficult to manage and historically associated with high mortality rate. We report two pairs of identical twins all affected by LSCTS successfully treated by sliding tracheoplasty. All had severe respiratory distress. Three infants had left pulmonary artery (LPA) sling and one intra-cardiac malformation. Slide tracheoplasty was done under cardiopulmonary bypass, and cardiovascular malformations were corrected at the same time. One child needed plication of paralyzed right hemi-diaphragm and another distal tracheal Palmaz stent insertion due severe tracheobronchomalacia. All children are doing well during 6 months follow-up. Slide tracheoplasty seems to produce the same good early results in twins as for singletons with LSCTS.