Anomalous origin of the left coronary artery. A twenty-year review of surgical management.

Children with anomalous origin of the left coronary artery from the pulmonary artery are at risk for myocardial infarction and death. Surgical management of this condition in children has evolved significantly during the past 20 years. Between 1970 and 1990, a total of 20 of these patients underwent surgical intervention at two institutions. Age at operation ranged from 3 weeks to 11 years (mean, 26 months). Twelve patients had congestive heart failure, three were in cardiogenic shock, and two had cardiac murmurs. Operative techniques included ligation (n = 9), subclavian artery anastomosis (n = 5), aortic implantation (n = 3), internal mammary artery anastomosis (n = 1), intrapulmonary tunnel from aortopulmonary window to coronary artery (n = 1), and cardiac transplantation (n = 1). The three deaths in the series occurred at 3 weeks, at 2 months, and at 9 years after ligation. There have been no deaths after establishment of a two coronary artery system or after transplantation. Two of the five patients who had subclavian artery anastomosis to the anomalous coronary artery have severe anastomotic stenosis and collateralization. For patients with anomalous origin of the left coronary artery from the pulmonary artery, we recommend direct aortic implantation of the anomalous coronary artery at the time of diagnosis. Intrapulmonary tunnel from aortopulmonary window to coronary artery, or aorta-coronary bypass with internal mammary artery are recommended for children in whom aortic implantation is not anatomically feasible. Left coronary artery ligation is not indicated for these patients; those who have survived ligation should be considered for elective establishment of a two coronary artery system because of the risk of late death.

Technical modifications for improved results in total anomalous pulmonary venous drainage.

To delineate factors that contribute to improved surgical outcome in patients with total anomalous pulmonary venous drainage, we reviewed the records of 52 consecutive patients. Venous drainage was supracardiac in 25 (48%), cardiac in 12 (23%), infracardiac in 10 (19%), and mixed in five (10%). Preoperative pulmonary venous obstruction was present in 18 patients (35%). Median age at the time of repair was 35 days and weight, 3.7 kg. Repair was performed with deep hypothermia, low-flow cardiopulmonary bypass, and occasional short periods of circulatory arrest. In patients with coronary sinus drainage, the veins were tunneled to the left atrium through an enlarged atrial septal defect, with a mortality of 8% (1/12) and no postoperative stenosis. The approach in patients with supracardiac, infracardiac, and mixed drainage varied with time. In 16 patients, the condition was managed by apical or right-sided exposure of the common vein, anastomosis of the common vein to the left atrium with continuous sutures, and primary closure of the atrial septal defect (type I repair). In the other 24 patients the common vein was approached from the right side through the right atrium and the interatrial septum. Common vein-left atrium anastomosis was performed with interrupted sutures and a piece of pericardium used to augment the anastomosis, prevent common vein distortion, and close the atrial septal defect (type II repair). Mortality in type I repair was 25% (4/16) and in type II repair, 4% (1/24). Follow-up was 7.86 +/- 4.0 years with no late deaths. Postoperative stenosis occurred in five of 14 (36%) patients who had type I repair versus two of 23 (9%) who had type II repair. Multivariate analysis showed that type I repair was a positive risk factor for hospital mortality (p = 0.05) and restenosis (p = 0.04). The technique of transatrial exposure of the common venous chamber, interrupted suturing of the common vein to the left atrium, and pericardial patch augmentation significantly improves survival and decreases risk of restenosis.

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.

Heart transplantation in neonates and in children.

Between May 1988 and July 1991, 28 neonates and children underwent orthotopic heart transplantation at Children's Memorial Hospital in Chicago. Indications for heart transplantation were hypoplastic left heart syndrome (10), dilated cardiomyopathy (13), aortic stenosis with endocardial fibroelastosis (1), complex D-transposition of the great arteries after Senning repair (1), L-transposition of the great arteries with single ventricle after shunt (1), cor biloculare, pulmonary atresia, and situs inversus after Fontan (1), and chronic rejection after heart transplantation for hypoplastic left heart syndrome (1). The age at time of transplantation ranged from 2 days to 17 years (mean, 5.3 +/- 6.1 years). Early deaths were from intraoperative donor right ventricular failure (2) and acute rejection after a second transplant procedure at 21 days (1), for an in-hospital mortality rate of 10.7%. Immunosuppression was with cyclosporine, azathioprine, and prednisone, with an attempt to discontinue the prednisone in neonates at age 6 months as guided by endomyocardial biopsy. Rejection episodes were treated with methylprednisolone pulse (34) or with OKT3 (4). Endomyocardial biopsy (in patients older than 6 months) was used extensively, and acute rejection was diagnosed in 29 of 301 biopsies. Three late deaths occurred (mean follow-up, 16.3 +/- 11.8 months): one of acute rejection at 13 months, one of viral pneumonia at 7 months, and one of intraabdominal sepsis as a complication of peritoneal dialysis at 5 months. Actuarial survival at 2 years is 77% +/- 9% (standard error of the estimate). Heart transplantation for neonates and for children can be performed with acceptable operative mortality. Intermediate results with triple therapy immunosuppression and an intensive rejection surveillance regimen relying on endomyocardial biopsy are encouraging.

Ventricular septal defect with tricuspid pouch with and without transposition. Anatomic and surgical considerations.

In a 10-year review, patients operated on for ventricular septal defect and tricuspid valve pouch were divided into two groups, because the effect of the tricuspid valve pouch is influenced by which ventricle has the higher pressure. Group I comprised patients with ventricular septal defect without transposition of the great arteries and group II, ventricular septal defect with transposition. In 72 of 392 group I patients, the septal tricuspid valve leaflet was incised to expose the edges of the hidden ventricular septal defect to accomplish proper anatomic repair. Forty-eight patients had a tricuspid valve pouch, the diagnosis being established by angiography, echocardiography, or at operation. Ages at operation ranged from 5 months to 22 years and the pulmonary-systemic flow ratio ranged from 1 to 3.4, with 16 being less than 1.5. In one patient the pouch produced a 40 mm Hg pressure gradient in the right ventricular outflow tract. At operation, through a transatrial approach, the tricuspid valve pouch was opened radially, the actual ventricular septal defect patched, and the tricuspid valve leaflet repaired. There were no deaths, no significant intraoperative or postoperative morbidity, and no tricuspid valve dysfunction. The average postoperative hospital stay was 4.8 days. In group II, six of 83 patients operated on for transposition with ventricular septal defect had significant left ventricular outflow tract obstruction from the tricuspid valve pouch. Five of six had a Mustard procedure, two requiring a left ventricular-pulmonary artery conduit, and in two of the six the ventricular septal defect was closed through the pulmonary artery. One patient had heart transplantation after a Mustard repair and tricuspid valve replacement. The sixth patient in group II had a successful arterial switch at 9 years of age, after the presence of left ventricular outflow tract obstruction was proved to be due to the pouch. The presence of a tricuspid valve pouch in group I may lead the surgeon to close false small openings produced by the pouch rather than the actual ventricular septal defect. Incising the pouch is safe and essential for proper exposure and secure closure of the true defect. In group II, the systemic right ventricular pressure can push the pouch into the left ventricular outflow tract, causing significant obstruction, and may contribute to tricuspid valve insufficiency after atrial baffle repair. Arterial switch is preferred because it returns the obstructive tricuspid valve pouch and abnormal tricuspid leaflet to the lower pressure pulmonic right ventricle.

Surgical options in subaortic stenosis associated with endocardial cushion defects.

Over a 15-year period, 12 patients with endocardial cushion defects undergoing correction had subaortic stenosis requiring operative intervention. Ages ranged from 4 months to 17 years (mean, 7 +/- 6 years) and subaortic gradients from 15 to 100 mm Hg (mean, 60 +/- 25 mm Hg). Subaortic stenosis was due to discrete fibromuscular tissues in 7 patients, mitral valve malattachment in 3, and tunnel outflow in 2. In 2, the subaortic stenosis was clinically significant at the time of endocardial cushion defects repair, whereas in 10 it was noted 2 to 14 years postoperatively (mean, 6.3 +/- 5 years). Surgical relief of subaortic stenosis was accomplished by resection of muscle tissues in 7, apicoaortic conduit insertion in 2, modified Konno procedure (aortic valve preserved) in 2, and lifting of malattached mitral valve from the outflow in 1. There was no early death and one late death (infected conduit). Severe mitral insufficiency developed in the patient who had the mitral valve lifted and necessitated valve replacement. Postoperative echocardiographic gradient in 9 patients ranged from 0 to 36 mm Hg (mean, 10.5 +/- 14 mm Hg). Clinically significant subaortic stenosis has not developed in any patient in 15 years of follow-up (mean, 5 +/- 4 years). We conclude that in subaortic stenosis associated with endocardial cushion defects, resection is effective for discrete obstruction, whereas a modified Konno procedure is preferable for obstruction due to tunnel outflow or mitral valve malattachment.

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

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

Pericardial patch tracheoplasty for severe tracheal stenosis in children: intermediate results.

Between 1982 and 1990, 15 children have undergone pericardial patch tracheoplasty, 13 for complete tracheal rings and 2 for acquired tracheal stenosis. Eleven had complete tracheal rings from the thoracic inlet or the cricoid to the carina. Diagnosis was by bronchoscopy (15), computed tomography (10), and magnetic resonance imaging (3). As first described by Idriss in 1984, the surgical technique uses a median sternotomy approach, extracorporeal circulation, bronchoscopic guidance, and the use of pericardium as a tracheal patch. There was one perioperative death of mediastinitis (operative survival, 93%). There were two late deaths caused by complications of tracheostomy (1) and direct laryngoscopy and bronchoscopy (DLB) (1). Follow-up is complete in 12 intermediate-term survivors and ranges from 0.4 to 8.2 years (mean, 4.2 +/- 2.9 years). Five children required multiple DLBs with dilation and excision of granulation tissue; one of these patients underwent tracheoplasty revision 5 months after the initial procedure for residual tracheal rings, and four of these children have had tracheostomies, two temporary and two currently (4 months, 7 years). Seven children had widely patent tracheas observed at follow-up DLB with little or no residual stenosis after tracheoplasty. All survivors are currently asymptomatic, except for the two children with tracheostomies. Pericardial patch tracheoplasty offers effective therapy with relatively low operative mortality for infants and children with severe long segment tracheal stenosis. On intermediate-term follow-up, nearly all survivors are essentially free of symptoms of residual tracheal stenosis.

Surgical management of the conal (supracristal) ventricular septal defect.

Surgical management of the conal (supracristal) ventricular septal defect differs significantly from the management of the perimembranous (infracristal) ventricular septal defect. The absence of a portion of the conal septum can lead to prolapse of the right cusp of the aortic valve, which predisposes these patients to aortic insufficiency. Between January 1980 and December 1989, 36 children with conal ventricular septal defect underwent intracardiac repair. Diagnosis was by echocardiography, cardiac catheterization, and intraoperative exploration. Preoperative evaluation showed that 26 patients (72%) had aortic valve prolapse and 16 (44%) had aortic insufficiency. Pulmonary-to-systemic flow ratios ranged from 1:1 to 3.5:1 (mean 2.0:1.0). Ten patients (27%) were believed to have clinical congestive heart failure. Age at the time of operation ranged from 2 weeks to 18 years (mean 5.5 years). Operative exposure was through the pulmonary artery (26), aorta (4), right ventricle (3), or right atrium (3). Simultaneous aortic valve suspension for aortic insufficiency was performed in four patients. Operative survival was 100%. Follow-up is complete in all patients and ranges from 0.5 to 9 years (mean 4.3 years). All patients are in normal sinus rhythm. No residual ventricular septal defects have been identified. Twenty-three of 36 patients (64%) have no evidence of aortic insufficiency; 12 of 36 (33%) have trivial or mild aortic insufficiency. One patient with initial severe aortic insufficiency underwent repeat aortic valvuloplasty 3 years after ventricular septal defect closure and aortic valve suspension. No patients have required aortic valve replacement. Surgical management of the conal ventricular septal defect differs from that of the perimembranous ventricular septal defect in two critical aspects. The operative approach should be through the pulmonary artery. This allows the best exposure of the remaining conal septum and the pulmonary and aortic valve leaflets, facilitating closure of the defect without injury to the valves or conduction system. Conal ventricular septal defects should undergo early closure, regardless of shunt volume, to prevent progressive aortic insufficiency.

Intermediate term results of infant orthotopic cardiac transplantation from two centers.

Orthotopic cardiac transplantation has become established for selected infants with severe forms of congenital heart disease. This study reviews the combined experience and intermediate term results of infants undergoing orthotopic cardiac transplantation from Children's Memorial Hospital, Chicago, and Kosair Children's Hospital, Louisville. From June 1986 through December 1989, 20 orthotopic cardiac transplantations were performed in 19 patients. Sixteen patients had variants of hypoplastic left heart syndrome. One infant had anomalous origin of the left coronary artery with severe ischemic cardiomyopathy. Two infants had aortic stenosis with endocardial fibroelastosis, and one had extracorporeal membrane oxygenation as a bridge to transplantation. Immunosuppression included cyclosporine, azathioprine (Imuran), and corticosteroids with an effort to wean the patients from steroids by 6 months to 2 years. Three early deaths resulted--from technical errors in two patients and from hyperacute rejection in one patient at 3 days. Four late deaths have occurred. Two patients died at 2 and 13 months of acute rejection. One patient died at 15 months of acute rejection after retransplantation. One patient died at 7 months of respiratory syncytial viral pneumonia. The remaining 12 patients are surviving 5 to 47 months (means 20 months) after orthotopic cardiac transplantation. Rejection surveillance in the first 6 months is by clinical signs supplemented by echocardiography, electrocardiography, and cell cycle analysis; endomyocardial biopsy is used after 6 months of age. For the cumulative series, 24 episodes of suspected rejection have been treated during 277 at-risk patient months with intravenous methylprednisolone (Solu-Medrol) (n = 18) and monoclonal antibody (OKT3) (n = 6), for an incidence of 1.04 episodes of rejection per patient per year. Serious posttransplantation infections including endocarditis, catheter sepsis, meningitis, and colonic perforation were successfully treated in four patients. Subjectively, their quality of life is excellent as shown by normal growth and developmental milestones and a low hospital readmission rate (1.4 episodes per patient per year). These encouraging intermediate term results warrant continued application of infant orthotopic cardiac transplantation for severe forms of congenital heart disease.

Living-related lobar lung transplantation in beagle puppies.

Unilateral lung transplantation has provided effective short-term therapy in adults with end-stage lung disease. Rejection continues to be the most common cause of transplant failure. Living-related lung transplantation may decrease the recipient immune response. The purpose of this study is to test the technical and physiological feasibility of living-related lobar lung transplantation from adult beagles into beagle puppies in a chronic model. Twenty purebred adult beagle donors underwent left thoracotomy with harvest of the left lower lobe using cold perfusion of the pulmonary artery and cold immersion. Twenty recipient purebred beagle puppies from the same colony underwent left thoracotomy, left pneumonectomy, and implantation of the donor adult lobe. Anastomoses were performed in sequence: pulmonary vein to left atrium, bronchus, pulmonary artery. Postoperative immunosuppression was with Cyclosporine. Respiratory function of the implanted lobe was evaluated by pulmonary angiography and during balloon occlusion of the right pulmonary artery with arterial blood gases 1 month after transplantation. Ten recipient puppies died of rejection (4), infection (3), or bronchial dehiscence (3) prior to angiography. Seventeen pulmonary angiograms in 10 surviving animals showed normal left pulmonary arterial blood flow (2), diminished left pulmonary arterial flow (5), and occluded left pulmonary artery (3). In two recipients balloon occlusion of the right pulmonary artery was performed and respiratory function was maintained solely by the transplanted lobe for 2 days in one recipient and for 30 minutes at 1 month and 2 months postimplant in a second recipient. Living-related lung transplantation of adult beagle lobes into pneumonectomized beagle puppies is technically feasible. High mortality rates in a chronic model are related to infection and rejection.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac transplantation for hypoplastic left heart syndrome: a modified technique [see comment].

A modified technique of infant orthotopic cardiac transplantation with arch reconstruction using bicaval cannulation is described, and the results in 4 infants with hypoplastic left heart syndrome are presented. This technique minimizes donor myocardial ischemic time and recipient circulatory arrest time.

An alternative approach to the surgical management of physiologically corrected transposition with ventricular septal defect and pulmonary stenosis or atresia.

A modified approach to the surgical management of corrected transposition of the great vessels with ventricular septal defect and pulmonary stenosis or atresia was used successfully in two patients. The procedure consisted of performing a venous switch operation, directing the blood flow from the morphologically left ventricle (right-sided chamber) into the aorta through the ventricular septal defect and inserting a valved conduit between the left-sided morphologically right ventricle and the pulmonary artery. This approach has several advantages when compared with the traditional surgical management, which consists of closure of the ventricular septal defect and a left ventricular (right-sided chamber) to pulmonary artery conduit. It uses the morphologically left ventricle as the systemic pumping chamber, thereby minimizing long-term ventricular failure. It allows closure of the defect from the right ventricular side of the septum, thus decreasing the prevalence of complete atrioventricular block. It also avoids use of the tricuspid valve as the systemic atrioventricular valve and therefore decreases the chance of postoperative valve regurgitation.

Massive primary chylopericardium: a case report.

A large pericardial effusion was discovered in an asymptomatic 12-year-old boy admitted for an elective orthopedic procedure. On physical examination, heart rate was 96 and blood pressure was 130/70 without paradox. The neck veins were not distended, but heart tones were distant. Chest roentgenogram (CXR) showed an enlarged cardiac silhouette. Echocardiogram showed a massive pericardial effusion compressing the right atrium, with depressed ventricular contractility. Pericardiocentesis yielded 450 mL of chylous fluid. A percutaneous pericardial drain was placed and drained another 400 mL of chyle. Pericardial fluid reaccumulated even though the patient was on a low-fat diet, and 1 week after admission left thoracotomy was performed with partial pericardiectomy and pericardial window. There was 1 L of chyle in the pericardial sac; frozen section of the pericardium showed lymphangiectasia. Chest tube drainage diminished rapidly and the patient was discharged. Follow-up CXR at 1 week showed fluid in both pleural spaces requiring bilateral tube thoracostomies again draining chyle. Even with total parenteral nutrition (TPN), 500 mL/d of chyle drained from the pleural tubes. Right thoracotomy with ligation of the thoracic duct was performed after 1 week of TPN. Pleural drainage abruptly dropped, and there has been no reaccumulation in either the pleural spaces or pericardium at 6-month follow-up. This case dramatically supports early thoracic duct ligation and partial pericardiectomy as the treatment of choice for primary massive chylopericardium.

Exit block in pediatric cardiac pacing. Comparison of the suture-type and fishhook epicardial electrodes.

Ninety-seven consecutive permanent epicardial pacemaker implantations were performed with either suture-type (group I, n = 52) or fishhook electrodes (group II, n = 45). In addition to epicardial fixation of the electrodes at the collar area, the suture-type was further secured in place by tying the electrode's suture at its exit to another nonabsorbable pledget-supported suture. Acute thresholds and slew rates were not significantly different between the two groups. Although the R wave was lower in group I (8.4 +/- 3.5 mV) than in group II (11.8 +/- 6, p less than 0.01), no sensing problems occurred. The larger surface area of suture-type electrode led to lower resistance in group I (271 +/- 61 omega) compared with group II (356 +/- 72, p less than 0.001); however, the difference in pacemaker generator longevity did not appear significant (group I, n = 12, mean 4.7 +/- 1.6 years; group II, n = 4, mean 5 +/- 0.6 years). The incidence of exit block was significantly higher in group II (40%, 18/45) than in group I (8%, 4/52, p less than 0.01). The length of the stimulation tip and better fixation of the suture-type electrode probably accounted for the observed difference in the incidence of exit block between the two electrodes.

Transposition of the great arteries: a comparison of results of the mustard procedure versus the arterial switch.

Sixty infants with transposition of the great arteries and intact ventricular septum underwent primary surgical correction in the first 3 months of life. Twenty-three patients had a Mustard procedure (group 1) and 37 patients, an arterial switch operation (group 2). The mean age at the time of repair was 42 +/- 31 days for group 1 and 8 +/- 6 days for group 2 (p less than 0.001). The mean weight at the time of repair was 3.6 +/- 0.7 kg for group 1 and 3.4 +/- 0.5 kg for group 2 (p = not significant). Operative mortality was 8.7% (2/23) in group 1 and 8.1% (3/37) in group 2 (p = not significant). The incidence of arrhythmias in the early postoperative period was 39% (9/23) in group 1 and 11% (4/37) in group 2 (p less than 0.01). All patients were followed a mean of 4.8 +/- 2.4 years in group 1 and 2.6 +/- 1.4 years in group 2 (p less than 0.001). Postoperative catheterization has been performed in 86% (18/21) of group 1 operative survivors and 50% (17/34) of group 2 operative survivors. Ejection fraction of the systemic ventricle was 79% +/- 15% of predicted normal in group 1 and 98% +/- 6% in group 2 (p less than 0.005). The incidence of late arrhythmias was 57% (12/21) in group 1 and 3% (1/34) in group 2 (p less than 0.001). There have been 2 late deaths in group 1 and 1 late death in group 2 (p = not significant).(ABSTRACT TRUNCATED AT 250 WORDS)

Direct tricuspid closure versus atrial partitioning in Fontan operation for complex lesions.

Thirty-three patients with complex lesions undergoing the Fontan operation needed either direct tricuspid closure (group 1, 14 patients) or atrial partitioning (group 2, 19 patients). In group 1, the tricuspid patch was sutured to the annulus leaving the coronary sinus draining to the systemic venous atrium. In group 2, atrial partitioning was accomplished with either a Dacron or a polytetrafluoroethylene patch, leaving the coronary sinus draining to the pulmonary venous atrium. Intraoperative distention of the left side was used to check for residual defects. In group 1, complete heart block developed in 5 patients (36%) and patch disruption, in 4 patients (29%). There were 3 late deaths (21%), which were due to sudden death, sepsis caused by Candida, and liver failure. In group 2, no patient had heart block, and patch disruption developed in 1 patient (5%). There was 1 early death (5%) and 2 late deaths (11%), which were due to sepsis caused by Candida and renal failure. Our experience suggests that atrial partitioning is a better approach than direct tricuspid patch closure in patients with complex lesions undergoing the Fontan operation.

Vascular anomalies causing tracheoesophageal compression. Review of experience in children.

Two hundred four infants and children (mean age 13 months) have undergone operation for the relief of tracheoesophageal obstruction resulting from vascular anomalies. One hundred thirteen patients had complete vascular rings (group I), 61 with double aortic arch and 52 with right aortic arch with a left ligamentum. Nine patients had a pulmonary artery sling (group II), 71 had innominate artery compression (group III), and 11 had miscellaneous anomalies (group IV). Patients were admitted with respiratory distress, stridor, apnea, dysphagia, or recurrent respiratory infections. Diagnosis was established by barium esophagogram in group I; barium esophagogram, bronchoscopy, and computed tomography or angiography in group II; bronchoscopy in group III; and barium esophagogram or angiography in group IV. The operative approach was through a left thoracotomy in group I, II and IV (93% of these patients) and through a right thoracotomy for group III (96% of these patients). The operative mortality rate was 4.9% and there were seven late deaths (3.4%). There have been no operative deaths in patients with isolated vascular anomalies in the past 28 years. Follow-up data from 1 month to 20 years (mean 8.5 months) were available on 159 patients; 141 (92%) were essentially free of symptoms, and 12 (8%) had residual respiratory problems. Five of six patients in group II having a lung scan postoperatively had a patent left pulmonary artery. A strong index of suspicion is necessary to avoid the complications of vascular rings in children. Barium swallow is the best single diagnostic technique for patients with complete vascular rings. A bronchoscopic study is required to diagnose innominate artery compression. Angiograms or computed tomographic scans are used to confirm the diagnosis of pulmonary artery sling. Left thoracotomy provides excellent exposure for all vascular rings except the displaced innominate artery, for which a right thoracotomy is the best approach.