Compression of the central airways by a dilated aorta in infants and children with congenital heart disease.

BACKGROUND: Children with congenital heart disease often experience respiratory symptoms in the preoperative and perioperative periods, which can complicate their management. An uncommon but important cause of respiratory insufficiency in such children is external airway compression. METHODS: We operated on 5 patients (median age, 6 months) with significant respiratory distress attributable to compression of the central airways by a dilated ascending aorta before or after repair of concomitant cardiovascular defects. Four of these patients had right aortic arch and 3 had pulmonary atresia with a ventricular septal defect and major aortopulmonary collaterals. In all patients, aortopexy was performed at the time of operation for the cardiovascular defects (n = 3) or after symptoms developed in the postoperative period (n = 2). The 3 patients in whom airway compression produced symptoms preoperatively also underwent reduction ascending aortoplasty. RESULTS: Symptoms resolved immediately after operation in 3 patients, whereas symptoms persisted in the other 2 patients and tracheostomy was required. At follow-up of 20 months to 5 years, all patients are alive and well, with mild or moderate respiratory symptoms in the 2 patients who required tracheostomy, both of whom were decannulated within 13 months. CONCLUSIONS: External airway compression can cause significant morbidity in patients with congenital heart defects other than vascular rings. In patients with respiratory symptoms in the context of a lesion that involves increased aortic outflow during intrauterine life and consequently, an enlarged ascending aorta, such as tetralogy of Fallot with pulmonary atresia, airway compression should be considered as a cause, especially if a right aortic arch is present or the patient also has pulmonary atresia with a ventricular septal defect and collaterals. Attempts to address this problem surgically may provide substantial relief, but increasing duration of airway compression is likely to lead to tracheal or bronchial malacia and persistent symptoms even after the compression is relieved.     

Aortopexy for persistent tracheal obstruction after double aortic arch repair

Persistent respiratory symptoms often occur after double aortic arch (DAA) repair but rarely require a second operation. We report 4 children with severe respiratory problems (failure to extubate, 2; severe respiratory distress, 2) caused by severe tracheomalacia and tracheal compression after DAA repair, treated by anterior aortopexy. Aortopexy proved effective and safe in improving symptoms and provides a simple treatment option for children with severe malacia or tracheal compression after DAA repair.     

Deviated trachea in hypoplasia and aplasia of the right lung: airway obstruction and its release by aortopexy

Three cases of male infants with hypoplasia and aplasia of the right lung and dextrocardia are reported. The infants developed increasing obstructive respiratory distress in the first 4 months of life. In all three cases, there was an opaque right hemithorax with overinflation of the left lung, a posterior deviation of the trachea with pulsatile stenosis, and a posteriorly located aortic arch. Hypoplasia (two cases) or aplasia (one case) of the right pulmonary artery and an absence or a remnant of the right main bronchus were also observed. Associated malformations were esophageal atresia with an abnormal high pouch of the proximal esophagus in case 1, and hypertelorismus syndrome in case 2. Because of severe respiratory distress in all three patients, an aortopexy was performed at the ages of 5 months (one infant) and 10 months (two infants). The obstructive respiratory signs disappeared postoperatively in the following weeks. It has been observed that tracheal stenosis in aplasia or hypoplasia of the right lung may be caused by the dislocated aortic arch. An aortopexy can release the airway compression in such cases. Because of associated malformations, a careful evaluation of the airway and vessels is recommended.     

Relief of tracheal compression by aortopexy

We have performed aortopexy in 12 children with tracheal compression. Six infants had compression secondary to a vascular anomaly (group 1), and the other 6 had previous repair of esophageal atresia (group 2). Eleven of the 12 children are alive after a mean follow-up of 36 months. In group 1, 1 patient died and 3 patients (50%) experienced recurrent respiratory distress. Five infants sustained a major postoperative complication, and the average postoperative hospital stay was 25 days. In group 2, however, aortopexy was uniformly successful. There were no deaths, no postoperative complications, and no cases of postoperative respiratory distress, and the mean postoperative hospital stay was only 10 days. For children with reflex apnea after repair of esophageal atresia, aortopexy is lifesaving and can be performed with minimal morbidity and mortality. Great caution is indicated in children with tracheal compression from other causes.     

Aortopexy reduces anastomosis stress after repair of coarctation

Restenosis after repair of coarctation with hypoplastic distal aortic arch is an important complication. Complete removal of ductal tissue, resection of isthmus area, and side-to-side arterioplasty of the distal aortic arch leads to a wide distance between the aortic arch and descending aorta; therefore, the anastomosis may remain under tension, increasing risk of restenosis. To reduce the tension, aortopexy of the descending aorta was used. The operative technique and the results in 16 neonates and infants are presented.     

Bronchial compression by posteriorly displaced ascending aorta in patients with congenital heart disease

BACKGROUND: We encountered several patients with posteriorly displaced ascending aorta and bronchial compression associated with congenital heart disease. We describe the helical computed tomography (CT) findings and explore the mechanism of airway compression. METHODS: We retrospectively reviewed the clinical data and CT findings of 8 patients with posterior displacement of the ascending aorta. The bronchial stenosis was quantified on reformatted images perpendicular to the main-stem bronchi. On an axial image at the level of main bronchi, we measured depth of retrosternal space, interaortic distance, and aorto-spinal distance. To compare with control, we measured the same variables in 10 control patients. RESULTS: In 7 patients, the main bronchus on the side of the aortic arch was squeezed between the ascending and descending aorta and showed slit-like stenosis. The right pulmonary artery was elongated around the ascending aorta in 5 patients and showed slit-like stenosis in 3. Patients with posterior displacement had significantly larger retrosternal space, smaller interaortic distance, and smaller aorto-spinal distance than did the control group. Aortopexy was undertaken in 3 patients. Follow-up computed tomograms of 2 patients showed improvement. CONCLUSIONS: The posteriorly displaced ascending aorta may compress the main bronchus on the side of the aortic arch and right pulmonary artery against the descending aorta or spine. Even if the bronchial compression is mild with tolerable airway symptoms, these patients must be closely observed. When airway symptoms are severe, aortopexy should be considered.     

One-stage total repair of aortic arch anomaly using regional perfusion.

OBJECTIVE: Primary repair of aortic arch obstructions and associated cardiac anomalies is a surgical challenge in neonates and infants. Deep hypothermic circulatory arrest prolongs myocardial ischemia and might induce cerebral and myocardial dysfunction. METHODS: From March 2000 to December 2005, 69 neonates or infants with aortic arch anomaly underwent one-stage biventricular repair with continuous cerebral perfusion in the presence of a nonworking beating heart using the dual perfusion technique on the innominate artery and aortic root. Preoperative diagnoses of arch anomaly comprised aortic coarctation (n=54) or an interrupted aortic arch (n=15). Combined anomalies were ventricular septal defect (n=52), anomalous origin of the right pulmonary artery from ascending aorta (n=3), hypoplastic left heart syndrome (n=2), truncus arteriosus (n=2), atrioventricular septal defect (n=2), double outlet right ventricle (n=1), total anomalous pulmonary venous return (n=1), partial anomalous pulmonary venous return (n=1), and aortic stenosis (n=1). RESULTS: The mean regional perfusion time was 27.8+/-9.8 min. There was no operative mortality. Postoperative low cardiac output was present in four patients (5.8%). A neurologic complication was noted in one patient (1.5%) who developed transient chorea, but recovered completely. During 32.8+/-17.5 months of follow-up, one late death (1.5%) occurred. There was neither reoperation associated with arch anomaly nor recoarctation except in one patient. One patient developed left main bronchial compression necessitating aortopexy. CONCLUSIONS: One-stage total arch repair using our regional perfusion technique is an excellent method that may minimize neurologic and myocardial complications without mortality. Our surgical strategy for arch anomaly has a low rate of residual and recurrent coarctation when performed in neonates and infants.     

Pericardial flap aortopexy for tracheomalacia

Tracheomalacia is frequently associated with esophageal atresia and vascular compression of the thoracic viscera. Suture aortopexy, as first described by Gross, has become the most commonly used surgical procedure for alleviating the symptoms of tracheomalacia. External stenting, implantation of cartilage graft, and tracheal resection have been tried in severe cases not amenable to aortopexy. A standard aortopexy was attempted in an infant who had undergone division of the posterior portion of a double aortic arch. Because of very tight posterior attachments of the mediastinal structures, the aortic arch could not be brought up to the sternum without undue tension. A 3 x 2 cm flap of pericardium was formed, based at the aortic root. The free end was then sutured to the undersurface of the sternum, thereby pulling the aorta toward the sternum under gentle, controlled tension. The patient was easily extubated and has remained symptom free. Pericardial flap aortopexy is a relatively simple procedure with minimal risk to the aorta or trachea. It may be the preferred initial procedure in the surgical management of tracheomalacia.     

Initial Experience of Modified Four-Branched Graft Technique and Antegrade TEVAR in Acute Type A Aortic Dissection

Background: We report the initial experience of modified four-branched graft technique for proximal aorta and arch repair, feasibly combined with antegrade thoracic endovascular aortic repair (TEVAR) to extend distal aortic reconstruction in acute type A aortic dissection.Methods: From 2011 to 2013, 12 consecutive patients with acute type A aortic dissection were indicated for arch surgery and underwent surgical replacement of proximal aorta, arch replacement or debranching procedure, and concomitant TEVAR for distal aortic repair.Results: A good surgical field was obtained in all patients. No major complications developed but two hospital deaths were attributed to end-organs damage preoperatively. Good and fast remodeling of thoracic descending aorta was demonstrated in 11 patients in postoperative CT imaging and no aneurysmal dilatation of visceral aorta had been observed in 10 patients during follow-up periods.Conclusion: Modified four-branched graft technique facilitated proximal aorta and arch repair, and provided excellent neurological outcome and favorable short-term results. Single-stage operation combined with antegrade TEVAR is feasible and effective to extend the repair down to the descending aorta, and thus achieved good remodeling of thoracic descending aorta.     

Thoracoscopic aortopexy for vascular compression of the trachea: approach from the right

Tracheomalacia resulting from vascular compression of the trachea may require aortopexy for symptomatic relief. Several operative approaches have been described for infants and children. The authors describe the technique of aortopexy by means of a right-sided thoracoscopic method as the initial approach to relieve tracheal compression in 2 children. Intraoperative bronchoscopy is mandatory to assess the adequacy of aortopexy prior to the completion of suture placement. This procedure was very successful in relieving tracheal compression in a 17-month-old boy with an aberrant innominate artery take-off and in a 2-year and 7-month-old boy with a history of esophageal atresia/tracheoesophageal fistula repair and severe tracheomalacia. A right-sided thoracoscopic approach to aortopexy in infants with severe tracheomalacia may be successfully performed as the initial operative intervention in infants with vascular compression of the trachea.     

Repair of persistent truncus arteriosus with interrupted aortic arch.

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

Malignant pleural mesothelioma in a 14-year-old boy with right aortic arch

We report successful surgery for malignant pleural mesothelioma (MPM) in a 14-year-old boy with right aortic arch. Pleural biopsy by video-assisted thoracic surgery yielded a diagnosis of MPM, epithelial type. As the disease was not changed after combination chemotherapy with three cycles of cisplatin and gemcitabine, we performed left extrapleural pneumonectomy, including resection of the pericardium and diaphragm for MPM, and aortopexy for right aortic arch. The postoperative course was uneventful, and the patient has remained alive without disease for 10 months postoperatively.     

Vascular ring in an extremely low birth weight infant

Vascular rings due to aortic arch anomalies can be a major cause of tracheoesophageal obstruction in infants. But there is no report of vascular ring, in an extremely low birth weight (ELBW) infant. This is a report of an infant with birth weight of 560 g, who has not diagnosed vascular ring until 6 months of age because of asymptomatic process by prolonged tracheal intubation. The patient, was treated for double aortic arch with tracheomalacia surgically by simple division of the atretic left arch and the ligamentum arteriosus, aortopexy and tracheostomy before reaching full recovery at 3 years of age. We reported our management of vascular rings in the ELBW infant. This case suggested that the difficulty of extubation in ELBW infants in spite of the improvement of respiratory condition should raise a suspicion of the tracheal compression by other lesions such as vascular rings.     

Cardiovascular causes of airway compression

Compression of the paediatric airway is a relatively common and often unrecognized complication of congenital cardiac and aortic arch anomalies. Airway obstruction may be the result of an anomalous relationship between the tracheobronchial tree and vascular structures (producing a vascular ring) or the result of extrinsic compression caused by dilated pulmonary arteries, left atrial enlargement, massive cardiomegaly, or intraluminal bronchial obstruction. A high index of suspicion of mechanical airway compression should be maintained in infants and children with recurrent respiratory difficulties, stridor, wheezing, dysphagia, or apnoea unexplained by other causes. Prompt diagnosis is required to avoid death and minimize airway damage. In addition to plain chest radiography and echocardiography, diagnostic investigations may consist of barium oesophagography, magnetic resonance imaging (MRI), computed tomography, cardiac catheterization and bronchoscopy. The most important recent advance is MRI, which can produce high quality three-dimensional reconstruction of all anatomic elements allowing for precise anatomic delineation and improved surgical planning. Anaesthetic technique will depend on the type of vascular ring and the presence of any congenital heart disease or intrinsic lesions of the tracheobronchial tree. Vascular rings may be repaired through a conventional posterolateral thoracotomy, or utilizing video-assisted thoracoscopic surgery (VATS) or robotic endoscopic surgery. Persistent airway obstruction following surgical repair may be due to residual compression, secondary airway wall instability (malacia), or intrinsic lesions of the airway. Simultaneous repair of cardiac defects and vascular tracheobronchial compression carries a higher risk of morbidity and mortality.     

Surgical treatment of coarctation and interrupted aortic arch complex in infants

One hundred forty-two consecutive neonates or early infants with coarctation and interrupted aortic arch complex who underwent biventricular repair at the Fukuoka Children's Hospital between January 1991 and December 2000 were reviewed. One-stage repair was performed in 33 patients (35%) with coarctation complex and in 41 patients (85%) with interrupted aortic arch complex. The overall mortality rate was 6.1% in one-stage repair and 6.6% in two-stage repair of coarctation complex patients and 9.8% in one-stage repair and 28.6% in two-stage repair of interrupted aortic arch complex patients. The recoarctation rate was 5.3% in coarctation complex and 2.1% in interrupted aortic arch complex. All patients with recoarctation underwent successful catheter intervention and required no reoperation. In conclusion, one-stage repair of interrupted aortic arch and coarctation complex with the anterior approach resulted in good outcomes. Then descending aorta cannulation through a median sternotomy combined with the cerebral perfusion technique enables complete avoidance of circulatory arrest and is a useful technique. However, a two-stage procedure can be useful in the patients whose condition has deteriorated substantially or in whom intracardiac anomalies are severe.     

Pulmonary autograft patch aortoplasty for reconstruction of an interrupted aortic arch associated with an aortopulmonary window

We present here an infant with an aortopulmonary window associated with an interrupted aortic arch. Single-stage repair with a pulmonary autograft patch for augmentation of the interrupted aortic arch repair was performed. Transpulmonary patch closure was used to repair the aortopulmonary window. A computed tomography scan at the 1-month follow-up demonstrated a reconstructed aortic arch without obstruction, kinking, or any sign of bronchial or branch pulmonary artery compression.     

Current outcomes of one-stage surgical correction for Berry syndrome

Objectives

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

Remodeling of the aortic root and reconstruction of the bicuspid aortic valve

BACKGROUND: Currently, isolated reconstruction of a regurgitant bicuspid aortic valve can be performed with adequate early results. Dilatation of the proximal aorta is known to be associated with this valve anomaly and may be partially responsible for the development of primary regurgitation or secondary failure of valve repair. We have used repair of the bicuspid valve with remodeling of the aortic root as an alternative to insertion of a composite graft. METHODS: Between October 1995 and May 1999, 16 patients (12 men, 4 women, aged 35 to 73 years) were seen with a regurgitant bicuspid aortic valve and dilatation of the proximal aorta of more than 50 mm. All patients underwent repair of the valve using either coapting sutures alone (n = 12) or in combination with triangular resection of a median raphe (n = 4). Using a Dacron graft, the aortic root was remodeled and the ascending aorta (n = 16) and proximal arch (n = 4) replaced. RESULTS: No patient died. The postoperative degree of aortic regurgitation was less than grade II in all patients. Valve function has remained stable in all patients between 2 and 43 months postoperatively. CONCLUSIONS: Reconstruction of the regurgitant bicuspid valve in the presence of proximal aortic dilatation is feasible with good results by combining the root remodeling technique with valve repair.     

Individual temperature management during thoracoabdominal aortic aneurysm repair using separate perfusion of upper and lower torso

We report thoracoabdominal aortic aneurysm repair using separate perfusion of upper and lower torso that can control temperature of each organ individually. This novel modality can maintain mild hypothermic organ perfusion in upper torso and protect the heart under empty beating, while lower torso is further cooled to protect the spinal cord and visceral organs. Therefore this technique may be useful for patients with heart disease who require complex reconstruction of the intercostal arteries or visceral branches. We used this technique successfully in a patient who has a history of surgical repair of the aortic arch and the abdominal aorta. A 70-year-old male who had a history of abdominal aortic aneurysm repair and aortic arch aneurysm repair using stented elephant trunk underwent Crawford's type II thoracoabdominal aortic aneurysm repair. Three pairs of the intercostal arteries and 4 visceral branches were reconstructed using this technique successfully.     

Tracheobronchial compression of vascular origin. Review of experience in infants and children

BACKGROUND: Tracheobronchial compression of vascular origin is an uncommon but important cause of respiratory distress in infants and children. We reviewed our surgical experience with 31 children, presenting vascular tracheobronchial compression. METHODS: Thirty-one children, with a median age of seven months, were operated on for airway and/or oesophageal compression secondary to vascular anomalies. Diagnostic findings, mainly established by bronchoscopy and angiography, revealed several pathologic disorders such as right aortic arch with aortic diverticulum in 10, double aortic arch in five, innominate artery compression in six, arteria lusoria in four, and pulmonary artery sling in one patient. Five children developed tracheobronchial compression after previous repair of a complex congenital heart disease. Clinical symptoms on admission were dominantly stridor, recurrent airway infection, dyspnea, respiratory distress and upper digestive complaints such as dysphagia and gastroesophageal reflux. RESULTS: Surgical relief was successful in 30 patients. Only one child with palliated tetralogy of Fallot and pulmonary artery sling died because of intractable extensive tracheobronchial stenosis. Late follow-up over three to 90 months showed a symptom-free evolution in most patients, including one reoperation for recurrent compression and one late death. CONCLUSIONS: Tracheobronchial compression by vascular structures in childhood is uncommon and may be masked by nonspecific respiratory symptoms, resulting in an often delayed diagnosis. Once imaging studies have clearly delineated the causal pathologic vascular structures, surgical correction is often straightforward and effective, in spite of the common presence of tracheobronchial malacia.