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Right arrow Congenital - acyanotic

Ann Thorac Surg 2005;79:38-46
© 2005 The Society of Thoracic Surgeons


Original article: Cardiovascular

Surgical Treatment of Pulmonary Artery Sling and Tracheal Stenosis

Andrew C. Fiore, MDa,*, John W. Brown, MDb, Thomas R. Weber, MDa, Mark W. Turrentine, MDb

a Divisions of Pediatric Surgery and Cardiothoracic Surgery, St. Louis University Medical Center, St. Louis, Missouri, USA
b Divisions of Pediatric Surgery and Cardiothoracic Surgery, Indiana University Medical Center, Indianapolis, Indiana, USA

Accepted for publication June 4, 2004.

* Address reprint requests to Dr Fiore, Cardinal Glennon Children's Hospital, 1465 S Grand Blvd, Glennon Hall A432, St. Louis, MO63104 (E-mail: fiorem2{at}slu.edu).

Presented at the Fiftieth Annual Meeting of the Southern Thoracic Surgical Association, Bonita Springs, FL, Nov 13–15, 2003.


    Abstract
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 DISCUSSION
 Acknowledgments
 References
 
BACKGROUND: Pulmonary artery sling is a rare vascular ring and is commonly associated with tracheal stenosis. Symptomatic newborns and infants with these complex lesions have a high mortality rate without surgical intervention. The ideal operation remains controversial, with debate focusing on the need for left pulmonary artery for reimplantation and the technique of tracheal reconstruction.

METHODS: From 1983 to 2003, 14 patients with pulmonary artery sling (mean age, 7 months; range, 6 days to 27 months) underwent repair of pulmonary artery sling alone (6 patients), tracheoplasty alone (1 patient), and pulmonary artery sling repair with tracheoplasty (7 patients). Preoperatively, 7 patients were intubated, 2 had VATER (vertebral, anal, tracheal, esophageal, and radial anomalies) syndrome, and 2 patients had agenesis of the right lung. The left pulmonary artery was reimplanted at the ductal insertion site in 13 patients. One patient had left pulmonary artery translocation. Tracheoplasty employing extracorporeal circulation consisted of autologous pericardial patch (6 patients) or slide tracheoplasty (2 patients). Six patients with pulmonary artery sling and mild tracheal stenosis required only left pulmonary artery reimplantation. Concomitant procedures included closure of atrial septal defect (4 patients), ventricular septal defect (4 patients), and shunt for Fallot's tetralogy (2 patients).

RESULTS: There were 2 hospital deaths (2 of 14;14%) from abdominal sepsis (1) and renal failure (1). Reoperations included diaphragm plication (2), tracheostomy (1), and bronchoscopy with laser resection of granulation tissue (5 patients: 2 slide, 3 pericardium). Follow-up was complete in all patients (mean, 42 months) with 1 late death from fungal sepsis. At follow-up, all left pulmonary artery anastomoses were patent by echocardiography, and no patient has required reoperation for trachea reconstruction.

CONCLUSIONS: These data demonstrate that tracheal repair is not always necessary in the presence of pulmonary artery sling; that agenesis of the right lung is not a contraindication to successful complete repair; and that simultaneous correction of intracardiac defects can be safely performed in selected patients. This study suggests that in newborns and infants, pericardial patch and slide tracheoplasty are effective methods for trachea reconstruction.


    Introduction
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 DISCUSSION
 Acknowledgments
 References
 
Pulmonary artery sling is a rare congenital condition in which the left pulmonary artery (LPA) originates extrapericardially from the posterior aspect of the right pulmonary artery. The LPA passes leftward around the right bronchus and between the lower trachea and esophagus, thereby indenting these structures and displacing the trachea to the left. The right main stem bronchus and esophagus are compressed anteriorly. The ligamentum arteriosum or ductus arteriosus takes origin from the main pulmonary artery, and passes anteriorly and superior to the left main stem bronchus to join the descending thoracic aorta to complete the ring (Fig 1).



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Fig 1. The anomalous left pulmonary artery (LPA) is seen arising from the right pulmonary artery (RPA) and coursing between the esophagus and the trachea. Associated trachea stenosis and the ductus arteriosus are illustrated. (MPA = main pulmonary artery.)

 
Glaevecke and Doehle [1] first recognized this condition in 1897 and Contro and associates [2] introduced the term "vascular sling" to distinguish this entity from a vascular ring. The term "ring-sling complex" was introduced by Berdon and associates [3] to emphasize the often coexisting tracheal anomaly. Complete tracheal rings are present in 50% to 65% of patients with pulmonary artery sling. The tracheal cartilages are circular and not U-shaped, giving rise to the term "stovepipe" trachea. The membranous trachea is absent, and the segment with complete rings is usually narrowed. The length of the tracheal stenosis is frequently extensive, making reconstruction difficult. Compression of the lower trachea leads to respiratory symptoms of wheezing and stridor. Bronchial compression (usually right) causes air trapping, pneumonia, and atelectasis. Failure to recognize these symptoms can lead to sudden death in neonates and infants [4].

The first successful repair of pulmonary artery sling was performed by Willis Potts and associates in 1953 [5]. They divided the left pulmonary artery near its origin, removed it from its position between the trachea and esophagus, and reanastomosed it to the main pulmonary artery anterior to the trachea. The current treatment of pulmonary artery sling has changed little since that time except that coexisting tracheal and cardiac lesions are also repaired when present.

The ideal treatment of long segment congenital tracheal stenosis (LSCTS) associated with complete tracheal rings remains controversial. Methods of tracheal reconstruction include resection with end-to-end anastomosis, slide tracheoplasty, or patch tracheoplasty using three different materials: fresh autologous pericardium, free rib cartilage graft, and free tracheal autograft patch.

The purpose of this report is to review our experience with the surgical treatment of pulmonary artery sling and associated tracheal stenosis.


    Patients and Methods
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 DISCUSSION
 Acknowledgments
 References
 
Between December 1985 and September 2002, 14 infants underwent operative repair of pulmonary artery sling. There were 4 boys and 10 girls. At the time of operation, their ages ranged from 6 days to 27 months (mean, 7 months), and patient weight ranged from 2.1 kg to 11.7 kg (mean, 6.0 kg.) Eight patients with LSCTS underwent pulmonary artery sling and tracheal surgery, whereas 6 patients with mild tracheal narrowing and absence of complete tracheal rings had repair of pulmonary artery sling only.

Presentation and Diagnosis
All patients with LSCTS presented with stridor, wheezing, or respiratory distress. Six of 8 patients were intubated preoperatively; only 1 patient without LSCTS and mild tracheal stenosis was ventilator dependent preoperatively.

The diagnosis of pulmonary artery sling was achieved with echocardiography in all patients. Earlier in this series, 2 patients underwent angiography to identify the course of the anomalous left pulmonary artery. Rigid bronchoscopy was performed preoperatively in all patients to identify complete tracheal rings (8 patients with LSCTS; 57%) and to define the degree and extent of tracheal stenosis.

Surgical Technique
The preincision preparation follows the guidelines established by Backer and associates [7]. Median sternotomy is performed. The thymus is completely resected, and pericardium is harvested and soaked in saline for tracheal repair. The aorta and pulmonary artery are separated, and the ductus (or ligamentum) is doubly ligated and divided. In the absence of additional cardiac anomalies, cardiopulmonary bypass is instituted using a single right atrial and aortic cannulation with the heart beating throughout the procedure at a systemic temperature of 32°C nasopharyngeal.

If additional intracardiac anomalies need to be addressed, the patient is converted to bicaval cannulation, the aorta is cross-clamped, and the heart arrested with blood cardioplegia after the pulmonary artery sling is repaired.

The left pulmonary artery is identified originating from the superior aspect of the right pulmonary artery and is dissected circumferentially to the left hilar branches. This usually requires entering the left pleural space, with care taken to avoid phrenic nerve injury. Using a partial occluding clamp, the left pulmonary artery is transected from its origin on the right pulmonary artery leaving a small cuff of left pulmonary artery to close with interrupted 7-0 PDS sutures (Ethicon, Somerville, NJ) in a manner to prevent right pulmonary artery stenosis. The left pulmonary artery is brought anterior to the trachea. The residual ductal stump on the main pulmonary artery is excised, and the left pulmonary artery is sewn to the main pulmonary artery using a partial occluding clamp. The length of the left pulmonary artery is shortened if necessary to prevent kinking, and the anastomosis performed with continuous or interrupted 7-0 PDS suture (Fig 2).



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Fig 2. The ductus arteriosus is divided and the anomalous left pulmonary artery (LPA) is detached from the right pulmonary artery (RPA) and reimplanted into the main pulmonary artery (MPA) using absorbable suture.

 
The trachea repair, if needed, is now performed with the cross clamp off and the patient rewarmed with the heart beating. The space between the aorta and superior vena cava is dissected to expose the trachea. The innominate artery and vein are circumferentially dissected and encircled with vessel loops for traction. The strap muscles and thyroid isthmus may require division to fully expose the area of tracheal stenosis. Care is taken to avoid damaging the lateral tracheal blood supply, especially along the distal half of the trachea at the level of the carina. The endotracheal tube is removed, and bronchoscopy performed to define the extent of tracheal stenosis. Needles can be placed through the trachea, using bronchoscopic visualization, to delineate the extent of stenosis externally.

If autologous pericardial patch tracheoplasty is to be performed, the trachea is incised in the anterior midline through the stenotic segment and through at least one normal tracheal ring superiorly and inferiorly. A rectangular piece of fresh pericardium is tailored to enlarge the tracheal lumen to 1.5 times the predicted normal diameter. The pericardium is sutured only to the tracheal edges with continuous 6-0 PDS suture avoiding the mucosal layer (Fig 3). Several partial thickness sutures are used to suspend the pericardium anteriorly to surrounding mediastinal structures to prevent patch collapse. The mediastinum is filled with saline, and the patient is ventilated to a peak airway pressure of 35 to 40 cm H20 to assess the patch for leaks. Bronchoscopy is then performed to assess the repair and confirm airway patency. The endotracheal tube is replaced, and the patient is weaned from cardiopulmonary bypass.



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Fig 3. Autologous pericardial patch tracheoplasty. The pericardial patch is secured to the trachea using continuous absorbable suture avoiding the tracheal mucosa.

 
Postoperatively, all patients having pericardial patch tracheoplasty are sedated and paralyzed to allow the anastomosis to adhere to surrounding structures. Patients are ventilated with positive end expiratory pressure of 8 cm H2O. Bronchoscopy is performed before extubation [8].

The technique of slide tracheoplasty follows the elegant description by Grillo [9] with the exception that cardiopulmonary bypass was utilized in all patients and interrupted 5-0 PDS suture was used for the anastomosis (Fig 4). These patients are paralyzed postoperatively and are extubated as soon as clinically indicated. This technique doubles the tracheal circumference, resulting in a quadruple cross-sectional area.



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Fig 4. Slide tracheoplasty. (A) The trachea is divided and the most stenotic segment is excised. (B) The proximal and distal limbs are opened and spatulated as illustrated. The arrows represent the technique of sliding the proximal and distal trachea segments over each other. (C) The spatulated end-to-end anastomosis is performed with interrupted absorbable suture taking bites to avoid the mucosa (see inset).

 
Postoperative Care
Postoperatively, patients were ventilated in the intensive care unit ranging from 1 to 97 days (mean, 26). The hospital stay ranged from 3 to 225 days (mean, 55). The mean follow-up in all hospital survivors was 42 months, with a range of 2 to 93 months.

Definitions
Early death is defined as death in the hospital or death within 30 days of discharge. All other deaths are considered late. Tracheal stenosis is defined as mild when bronchoscopy shows narrowing that is clinically insignificant. Moderate or severe stenosis results in clinical symptoms of wheezing, stridor, or ventilator dependence and requires surgical treatment.


    Results
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 DISCUSSION
 Acknowledgments
 References
 
The demographics, operative procedure and outcome of the entire series are summarized in Table 1.


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Table 1. Patient Characteristics
 
Pulmonary Artery Sling Repair
Pulmonary artery sling repair was performed using the reimplantation technique in 13 of 14 patients (93%). Of these 13 patients, the pulmonary artery was reimplanted into the main pulmonary artery in 11 using sternotomy, whereas 2 patients were repaired using a left thoracotomy. The remaining patient in this series had the left pulmonary artery translocated anterior to the trachea at the time of surgery for LSCTS. This patient had agenesis of the right lung and absence of the right main stem bronchus and right pulmonary artery.

Echocardiography was performed in the 12 hospital survivors to assess left pulmonary artery patency at a mean 42 months (range, 2 to 93). The left pulmonary artery was patent in all patients, but 3 patients had mild and 1 patient had moderate (mean gradient,14 mm Hg) left pulmonary artery stenosis.

Tracheal Stenosis Repair
Eight patients underwent repair of long segment congenital tracheal stenosis. Six patients had autologous pericardial patch tracheoplasty and 2 patients received slide tracheoplasty.

Among the 6 patients with pericardial patch tracheoplasty, 4 were intubated preoperatively and 4 had concomitant repair of associated intracardiac defects (tetralogy of Fallot, 1; ventricular septal defect, 2; atrial septal defect, 1). Three of these patients also underwent incision and pericardial patch extension into the right main stem bronchus. The 3 deaths in this series (2 early; 1 late), and the 1 patient who required tracheostomy, had pericardial patch tracheoplasty.

Two patients underwent slide tracheoplasty. Both patients were intubated preoperatively and had complete agenesis of the right lung and right main stem bronchus. One patient also had Down's syndrome and required concomitant repair of atrial septal defect and ventricular septal defect. Delayed sternal closure was necessary in both patients. Their postoperative course was complicated by tracheitis, edema, and the development of granulation tissue at the anastomosis. Both patients were treated with antibiotics, steroids, and laser resection of the granulation tissue. At the time of follow-up, neither patient has required tracheal reoperation, and their parents report that these patients have a normal breathing pattern.

A comparison of the hospital course using the two alternative methods of tracheal reconstruction is summarized in Table 2. Although the number of patients in each cohort is small and statistical comparison would not be meaningful, the data suggest that slide and pericardial patch tracheoplasty are similar with respect to length of hospitalization, time on the ventilator, and frequency of laser resection of granulation tissue.


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Table 2. Comparison of Slide With Pericardial Patch Tracheoplasty
 
Six patients with mild tracheal stenosis and absence of complete tracheal rings underwent repair of pulmonary artery sling only. At the time of follow-up, 2 of these patients are completely asymptomatic, while 3 patients have mild residual right main stem bronchial stenosis. The remaining patient has moderate right bronchial stenosis. He has been treated with bronchial dilatations, and at follow-up has a residual 10% to 20% stenosis; he remains mildly symptomatic.

Repair of Associated Defects
Seven patients underwent repair of associated cardiac and noncardiac defects in addition to division of the ligamentum (Table 1). The most common associated cardiac anomalies addressed were closure of atrial and ventricular septal defects which were performed concomitantly with the pulmonary artery sling and tracheal reconstruction. The 2 patients with tetralogy of Fallot underwent palliative shunts at the time of pulmonary artery sling and tracheal reconstruction. The Fallot operation was completed at 6 months and 2 years after pulmonary artery sling surgery.

Five of these 7 patients presented with genetic syndromes in addition to pulmonary artery sling and LSCTS (VATER [vertebral, anal, tracheal, esophageal, and radial anomalies] syndrome in 2 patients; Holt-Oram syndrome in 1 patient; asplenia syndrome, 1 patient; Down's syndrome, 1 patient). The patient with VATER syndrome required repair of duodenal and anal atresia, and the asplenic patient required a colostomy for anal atresia, and both were performed before pulmonary artery sling surgery. The patient with Holt-Oram syndrome had tetralogy of Fallot requiring a central shunt, and the patient with Down's syndrome presented with agenesis of the right lung and right pulmonary artery as well as atrial septal defect and ventricular septal defect. The latter patient underwent repair of pulmonary artery sling, slide tracheoplasty for LSCTS, and closure of atrial and ventricular septal defects.

Morbidity
Among the 12 hospital survivors, postoperative morbidity necessitated the following operations: plication of the left hemidiaphragm in 2 patients, insertion of a permanent pacemaker in 1 patient, resection of the right upper and right middle lobe for lobar emphysema in 1 patient, and reoperation for mediastinal bleeding in 1 patient. The patient who was reexplored for postoperative hemorrhage also had a superficial wound infection.

Tracheostomy was required in a 6-day-old, 2.5-kg patient with VATER syndrome. She had pulmonary artery sling, colostomy for imperforate anus, and a 2-mm distal tracheal lumen with a 1-mm opening into the right main stem bronchus. The pericardial patch was extended into the right main stem bronchus. The repair of pulmonary artery sling was uneventful, but she required multiple laser resections of obstructing granulation tissue at the carina and right main stem bronchus necessitating tracheostomy 23 months postoperatively. Currently, she is growing well at home with intermittent ventilatory support and a 4- to 5-mm distal tracheal lumen.

Mortality
There were 2 early deaths at 1 month and 2 months after repair of pulmonary artery sling and LSCTS (2 of 14; 14%). One of these patients, with VATER syndrome, underwent colostomy for imperforate anus and repair of duodenal atresia. His pulmonary artery sling repair, pericardial patch tracheoplasty, and closure of atrial and ventricular septal defects were performed uneventfully. Ten weeks postoperatively, duodenal dehiscence developed and he died of abdominal sepsis and multiorgan system failure. The other early death occurred in a 14-month-old boy who underwent pulmonary artery sling repair, ventricular septal defect closure, and pericardial patch tracheoplasty. The pericardial patch had to be extended into the left bronchus. He had postoperative bleeding requiring reexploration and low cardiac output; he died 1 month postoperatively of renal failure.

One late death occurred at 14 months, a 2-year-old girl with Holt-Oram syndrome, tetralogy of Fallot, pulmonary artery sling, and LSCTS. She was discharged uneventfully after repair of pulmonary artery sling, LSCTS, and insertion of a central shunt. Six months later she underwent complete repair of tetralogy of Fallot. Her postoperative course was complicated by complete heart block and left diaphragm paralysis. She underwent permanent pacemaker insertion and left diaphragm plication but died 6 weeks postoperatively of Pseudomonas and fungal sepsis.

In the entire series, our highest mortality rate (2 of 6 patients, 33%) and our greatest morbidity rate (2 of 6 patients, 33%) were in patients less than 1 year of age.


    Comment
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 DISCUSSION
 Acknowledgments
 References
 
The principal areas of controversy involving patients who present with pulmonary artery sling and congenital tracheal stenosis include the optimal methods to establish the diagnosis, the relative merits of pulmonary artery reimplantation versus translocation, the need for cardiopulmonary bypass, and the most advantageous technique to repair long segment congenital tracheal stenosis.

Virtually all patients who present with pulmonary artery sling have some compression of the right main stem bronchus and lower trachea causing abnormal breathing. Even in the absence of coexisting congenital tracheal stenosis, death from airway obstruction has been reported as early as 2 days after birth and is common before 6 months of age in untreated patients [10].

The procedure of choice to establish the diagnosis of pulmonary artery sling is echocardiography. This noninvasive procedure can define any coexisting intracardiac abnormalities and is safe to use in critically ill neonates with compromised airway. In our series, echocardiography was diagnostic of pulmonary artery sling in all patients and only 1 patient has required angiography since 1985. Magnetic resonance imaging and computed tomography with contrast can establish the diagnosis but are rarely necessary. Anterior pulsatile indentation of the esophagus outlined on barium swallow is virtually pathognomonic for pulmonary artery sling, but the examination is contraindicated in these critically ill neonates, especially if ventilator dependent. Once the diagnosis is made, patients should be offered operative intervention.

The optimal method to repair pulmonary artery sling is sternotomy with cardiopulmonary bypass. This technique permits ease of left pulmonary artery dissection and a safe noncompromised environment to reimplant the left pulmonary artery into the main pulmonary artery after adequate resection of all residual ductal tissue. It is safer and easier to dissect the main pulmonary artery and the rightwardly displaced left pulmonary artery with the lungs deflated. Mobilization of these structures reduces the potential for anastomotic tension and may enhance long-term LPA patency. Continuous or interrupted absorbable suture is recommended for this anastomosis. Pawade and associates [11] implanted the LPA into the main pulmonary artery using cardiopulmonary bypass in 18 patients. One patient died late. The left pulmonary artery was patent in all 14 patients studied postoperatively. Cardiopulmonary bypass also permits concomitant repair of coexisting congenital cardiac anomalies if indicated.

An alternative technique to repair pulmonary artery sling described by Jonas and associates [11] is translocation of the undivided LPA anterior to the trachea at the time of tracheal resection for congenital tracheal stenosis. This technique has the advantage that there is no pulmonary artery suture line, but has the disadvantage that it cannot be employed in those patients in whom the tracheal stenosis is repaired by pericardial patch tracheoplasty or in patients without clinically significant tracheal stenosis. Two criticisms have been raised against the translocation technique. Castanada and associates [13] reported use of this approach in 5 patients with pulmonary artery sling, in 2 of whom this procedure resulted in kinking of the proximal LPA necessitating reimplantation into the main pulmonary artery. In addition, Backer and coworkers [14] suggest that placement of the left pulmonary artery in front of the trachea could potentially result in anterior compression of the trachea or left main stem bronchus. We employed LPA translocation in 1 patient who also had slide tracheoplasty for LSCTS and agenesis of the entire right lung. The left pulmonary artery is patent by echocardiography and has not resulted in airway compression at 6 months' follow-up.

Complete tracheal rings and LSCTS are present in approximately 50% to 60% of patients with pulmonary artery sling. The majority of patients present with stridor and respiratory obstruction of variable degree. Critical obstruction can develop rapidly after acute respiratory infection with imposed inflammation, edema, and small amounts of secretions. Urgent placement on ventilatory support in this setting is the rule, especially in newborns and infants.

Spiral computed tomography can be useful to make the diagnosis of tracheal stenosis if the patient is sufficiently stable for transport. This modality is cost effective compared with magnetic resonance imaging or cardiac catheterization, does not require sedation as the procedure is performed in 10 to 15 seconds, and affords excellent detail of the trachea [15]. Spiral computed tomography was used to assess the postoperative tracheal reconstruction in 4 of our patients.

The key to accurate diagnosis of tracheal stenosis is bronchoscopy. This was the primary tool of diagnosis in all of our patients. The pediatric tracheal and cardiothoracic surgeon working together can precisely define the degree and length of tracheal stenosis after cardiopulmonary bypass is established and the entire trachea is exposed. This is a key component in the management of these patients as the extent of stenosis is not always apparent when viewing the trachea externally.

The optimal technique to repair congenital tracheal stenosis remains controversial, and a number of techniques have been utilized. The primary concerns include the growth potential of the reconstructed trachea, the incidence of early and late granulation tissue at the repair site, the fate of cartilaginous or pericardial surfaces, and the long-term functional outcome.

Autologous pericardial patch tracheoplasty has the advantage that minimal dissection is required to expose the anterior trachea, thus preserving the lateral blood supply. The pericardial patch is relatively simple to construct, and it can enlarge the entire trachea, including either main stem bronchus if necessary. There is evidence that the trachea grows with time, and the pericardium has the capacity to reepithelialize [16]. The major disadvantage of this technique is the potential for patch collapse, the need for prolonged periods of paralysis with ventilatory support, and the development of obstructing granulation tissue along the suture line. In our series, we suspended the pericardium to adjacent mediastinal structures and did not experience patch collapse. However, virtually all patients required long periods of ventilatory support and repeat endoscopic laser resection of granulation tissue. We observed excessive proliferation of granulation tissue when the pericardial patch extended beyond the carina into either main stem bronchus. The single tracheostomy employed in this series was in 1 such patient. When relief of main stem bronchial stenosis is indicated, a free cartilage graft maybe more advantageous than pericardial patch extension.

Backer and associates [17] reported on 28 patients with severe tracheal stenosis, who underwent pericardial patch tracheoplasty and required reoperation or reintervention. Eight patients had pulmonary artery sling and of these, 4 patients (50%) required reoperation compared with 2 of 20 patients (10%) without pulmonary artery sling. This report underscores the frequency of postoperative complications associated with pericardial patch tracheoplasty requiring reoperation, especially in the presence of pulmonary artery sling.

Slide tracheoplasty is a novel approach to the management of LSCTS. It was initially developed by Tsang and Goldstraw and has been modified by Grillo [18]. This technique has the potential advantage in that foreign material is avoided. As with pericardial patch tracheoplasty, however, granulation tissue and edema develop along the suture line, necessitating prolonged periods of ventilatory support. Extremely long tracheal stenoses or those that involve either main stem bronchus pose potential limitations to this technique. We employed this procedure in 2 patients. Both patients are at home growing well without significant breathing abnormality at 13 and 16 months postreconstruction, suggesting that the tracheal anastomosis is growing. When compared with pericardial patch tracheoplasty, the hospital and intubation days were similar as was the need to endoscopically resect granulation tissue (Table 2).

The 6 patients with mild tracheal stenosis and absence of complete tracheal rings who underwent only pulmonary artery sling surgery can be particularly challenging. The decision to repair the trachea is based on the presence of clinical symptoms indicating moderate to severe stenosis and the degree of tracheal narrowing at bronchoscopy when compared with normal internal tracheal diameters in infants and children [19]. Postrepair bronchoscopy frequently demonstrates varying degrees of right main stem bronchial stenosis. We advocate conservative management as reconstructive techniques in this location are suboptimal. Bronchial stenting, balloon dilatation, laser resection, free cartilage graft, and pericardial patch tracheoplasty have all been employed with variable success [20–22]. In our series, 1 symptomatic patient in this subgroup has required multiple dilations of the right main stem bronchus, whereas the remaining 5 patients have mild stenosis but remain asymptomatic without any intervention.

Patients who present with pulmonary artery sling, LSCTS and a genetic syndrome deserve a word of caution. They are a particularly high risk group presenting with coexisting cardiac and non cardiac abnormalities. Our highest postoperative morbidity and 2 of the 3 deaths in this series were in this patient subgroup. The pulmonary artery sling and LSCTS must be addressed, but conservative management of coexisting lesions should be contemplated. Based on our experience, concomitant closure of atrial and perimembranous ventricular septal defects can be performed with minimal added morbidity, but more complex anomalies (tetralogy of Fallot) should be palliated or observed while the patient recovers from the pulmonary artery sling and tracheal reconstruction.

The association of right lung agenesis with pulmonary artery sling and LSCTS has been reported by several investigators and was observed in 2 patients in our series [23, 24]. This high-risk subgroup can be safely managed with repair of pulmonary artery sling and LSCTS. Slide tracheoplasty was successfully employed in both patients.

Conclusions
The infant born with pulmonary artery sling and tracheal stenosis presents a significant challenge to the pediatric and cardiothoracic surgeon alike. These patients require close cooperation between these two surgical specialities as well as their medical counterparts. We believe that the diagnosis of pulmonary artery sling is optimally made with echocardiography, while bronchoscopy is the key to the assessment of tracheal stenosis. It is our practice to undertake repair of both lesions simultaneously using sternotomy and cardiopulmonary bypass. The anomalous left pulmonary artery can be repaired by reimplantation into the main pulmonary or translocated anterior to the trachea. Coexisting intracardiac pathology can be repaired or palliated in selected patients. In those patients with long segment congenital tracheal stenosis, pericardial patch tracheoplasty or slide tracheoplasty are acceptable methods of reconstruction. From this small clinical series, we cannot categorically advocate one method of tracheal reconstruction. We believe both techniques are equally effective, and the choice of tracheal reconstruction should be guided by the clinical experience of the operating surgeon.

It must be emphasized that the postoperative care of these patients requires close vigilance by pediatric intensivists, anesthesiologists, cardiothoracic surgeons, and pediatric general surgeons. The management of the reconstructed trachea requires close collaboration among these specialities to achieve the best long-term result [25].


    DISCUSSION
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 DISCUSSION
 Acknowledgments
 References
 
DR CONSTANTINE MAVROUDIS (Chicago, IL): Thank you, Andy, that was a very nice presentation on a difficult series of patients. I would like to compare your results a little bit with ours and ask you a few questions.

In this series of 14 patients with pulmonary artery sling, 57% required a simultaneous tracheal stenosis repair and 64% had concomitant repair of congenital heart disease. This certainly highlights the importance of assessing pulmonary artery sling patients for tracheal stenosis and congenital heart disease, just as you have done.

Since Potts reported the first pulmonary artery sling repair at our institution, Children's Memorial Hospital, in 1953, 1 year before the founding of this organization, 34 patients have undergone repair of pulmonary artery sling. In our series, 22, or 65%, required repair of a significant tracheal stenosis. This is about what you have shown. Interestingly, only 6 of 34, or 18%, had a concomitant cardiac procedure, one third of your incidence. Also interesting, 4 of these concomitant cardiac procedures at CMH occurred in our last 8 patients, for a more recent incidence of 50%. So we are heading in the same direction that you are.

In our series, there were no operative deaths, but there were 4 late deaths. One late death was a child with biliary atresia who died awaiting a liver transplant. The other 3 late deaths occurred 6 months after the tracheal stenosis repair.

Our current procedure of choice for repair of associated tracheal stenosis is the tracheal autograft technique. The principle of the tracheal autograft technique is to shorten the trachea and use the removed segment as an anterior patch. This has now been used in 14 of our pulmonary artery sling patients with 1 death. The pericardial patch was previously used in 8 patients with 2 deaths. One patient had a slide tracheoplasty and 3 others had tracheal resection, and from that group there was 1 death. We have attempted to follow up all these patients with nuclear medicine pulmonary perfusion scans and have found that in all patients who had this scan the left blood flow is approximately 36%. I would like to ask you a few questions.

In our series, the current diagnostic procedure of choice for making a diagnosis of pulmonary artery sling is echocardiogram, as you have noted, on the sick neonate with tracheal stenosis who can't easily be moved. We use multidetecting computed tomography angiography in the older stable patients. What do you believe is the diagnostic procedure of choice for pulmonary artery sling under these circumstances?

We have only used the pulmonary artery translocation technique in 2 patients, both of whom had agenesis of the right lung and in whom the tracheal stenosis required transection of the trachea. You have made some comments about this, but I wonder if you can tell us, if you see it again, would you in fact transect the pulmonary artery and do a reanastomosis?

The third question relates to the presentation at this meeting 2 years ago in San Antonio from the Wake Forest group. They reported the elective use of extracorporeal membrane oxygenation after complex tracheal surgery repair in neonates. Have you used this strategy in your patients, and do you believe that this is a viable elective postoperative strategy?

Congratulations on a very difficult set of patients, you presented it extremely well.

DR FIORE: Thank you, Gus. At this time I would like to recognize the important contributions made by Drs Backer, Mavroudis, and Holinger at Children's Memorial in the treatment of children with pulmonary artery sling and coexisting tracheal stenosis.

We believe, as I think you and Carl do, that echocardiography is the diagnostic treatment of choice for pulmonary artery sling. I suppose in a stable patient—and most of these patients are not very stable with varying degrees of airway obstruction—you could perform computed tomography angiography to image the anomalous pulmonary artery.

The optimal method to repair pulmonary artery sling in patients who require pericardial patch tracheoplasty, or in patients who require no tracheal surgery, is division and reimplantation as we described. In patients in whom the trachea is divided, as in the performance of slide tracheoplasty, then strong consideration should be given to the translocation technique. Although our experience is limited, we would probably try translocation as a first procedure and see how the course of the anomalous pulmonary artery appeared after lung inflation. If kinking occurred, then we would shorten the artery and perform reimplantation.

Extracorporeal membrane oxygenation was not required in any of our patients. However, it is a very important option to have postoperatively. If the tracheal and cardiac repair were performed perfectly, then extracorporeal membrane oxygenation can be a life-saving adjunct procedure for these critically ill neonates.[6, 12]


    Acknowledgments
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 DISCUSSION
 Acknowledgments
 References
 
The authors gratefully acknowledge Terri Wriley for her expert technical assistance with manuscript preparation. They also thank Palaniswamy Vijay, PhD, and Barbara Kountzman, RN, for assisting with data acquisition.


    References
 Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 DISCUSSION
 Acknowledgments
 References
 

  1. Laevecke H, Doehle H. Uber cine seltene angeborene Anomalie der Pulmonalarterie Munch Med Wochenschr 1897;44:950.1.
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  5. Potts WJ, Holinger PM, Rosenblum AH. Anomalous left pulmonary artery causing obstruction to right main bronchus JAMA 1954;155:1409-1411.
  6. Heinemann MK, Ziemer G, Sieverding L, Baden W, Kaulitz R, Luhmer I. Long-segment tracheal resection in infancy utilizing extracorporeal circulationIn: Imai Y, Momma K, editors. Proceedings of the 2nd World Congress of Pediatric Cardiology and Cardiac Surgery. New York: Futura Publishing; 1998. pp. 711-713.
  7. Backer CI, Mavroudis C, Dunham ME, et al. Pulmonary artery sling: results with median sternotomy, cardiopulmonary bypass, and reimplantation Ann Thorac Surg 1999;67:1738-1745.[Abstract/Free Full Text]
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  10. Backer CL, Ilbawi MN, Idriss FS, DeLeon SY. Vascular anomalies causing tracheo- esophageal compressionReview of experience in children. J Thorac Cardiovasc Surg 1989;97:725-731.[Abstract]
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