A multicenter study of primary graft failure after infant heart transplantation: impact of extracorporeal membrane oxygenation on outcomes.

Primary graft failure is the major cause of mortality in infant HTx. The aim of this study was to characterize the indication and outcomes of infants requiring ECMO support due to primary graft failure after HTx. We performed a retrospective review of all infants (<1 yr) who underwent Htx from three institutions. From 1999 to 2008, 92 infants (<1 yr) received Htx. Sixteen children (17%) required ECMO after Htx due to low cardiac output syndrome. Eleven (69%) infants were successfully weaned off ECMO, and 9 (56%) infants were discharged with a mean follow-up of 2.3 ± 2.5 yr. Mean duration of ECMO in survivors was 5.4 days (2-7 days) compared with eight days (2-10 days) in non-survivors (p = NS). The five-yr survival rate for all patients was 75%; however, the five-yr survival rate was 40% in the ECMO cohort vs. 80% in the non-ECMO cohort (p = 0.0001). Graft function within one month post-Htx was similar and normal between ECMO and non-ECMO groups (shortening fraction = 42 ± 3 vs. 40 ± 2, p = NS). For infants, ECMO support for primary graft failure had a lower short-term and long-term survival rate vs. non-ECMO patients. Duration of ECMO did not adversely impact graft function and is an acceptable therapy for infants after HTx for low cardiac output syndrome.

beta-Adrenergic modulation of the inwardly rectifying potassium channel in isolated human ventricular myocytes. Alteration in channel response to beta-adrenergic stimulation in failing human hearts.

The beta-adrenergic modulation of the inwardly-rectifying K+ channel (IK1) was examined in isolated human ventricular myocytes using patch-clamp techniques. Isoproterenol (ISO) reversibly depolarized the resting membrane potential and prolonged the action potential duration. Under the whole-cell C1- -free condition, ISO applied via the bath solution reversibly inhibited macroscopic IdK1. The reversal potential of the ISO-sensitive current was shifted by approximately 60 mV per 10-fold change in the external K+ concentration and was sensitive to Ba2+. The ISO-induced inhibition of IK1 was mimicked by forskolin and dibutyrl cAMP, and was prevented by including a cAMP-dependent protein kinase (PKA) inhibitor (PKI) in the pipette solution. In single-channel recordings from cell-attached patches, bath applied ISO could suppress IK1 channels by decreasing open state probability. Bath application of the purified catalytic sub-unit of PKA to inside-out patches also inhibited IK1 and the inhibition could be antagonized by alkaline phosphatase. When beta-adrenergic modulation of IK1 was compared between ventricular myocytes isolated from the failing and the nonfailing heart, channel response to ISO and PKA was significantly reduced in myocytes from the failing heart. Although ISO inhibited IK1 in a concentration-dependent fashion in both groups, a half-maximal concentration was greater in failing (0.12 microM) than in nonfailing hearts (0.023 microM). These results suggest that IK1 in human ventricular myocytes can be inhibited by a PKA-mediated phosphorylation and the modulation is significantly reduced in ventricular myocytes from the failing heart compared to the nonfailing heart.

Role of endomyocardial biopsy in rejection surveillance after heart transplantation in neonates and children.

OBJECTIVES: The aim of this study was to retrospectively evaluate the sensitivity of noninvasive surveillance (physical examination, echocardiography) of rejection in accurately predicting histologically documented rejection episodes. Additionally, the usefulness of routine scheduled biopsy and its safety in pediatric patients was explored. BACKGROUND: Endomyocardial biopsy has been utilized as the standard for rejection surveillance after heart transplantation in adults, but its role in documenting clinically suspected rejection and in routine surveillance of pediatric patients has not been agreed upon. METHODS: Heart transplantation was performed in 14 neonates and 21 children. The immunosuppressive regimen consisted of cyclosporine, azathioprine and prednisone. All patients underwent routine noninvasive rejection surveillance that included clinical examination and echocardiography. In the neonates, biopsy was performed quarterly beginning 6 months after transplantation, after cessation of prednisone therapy. In the children, biopsy was performed 15 times in the 1st year. A minimum of five biopsy samples were interpreted using the Working Formulation for Heart Transplant Rejection. RESULTS: In the neonates, 37 biopsies were performed. Evidence of rejection was present in only three biopsy samples obtained during eight episodes (38%) of clinically suspected rejection. In 29 biopsies performed when rejection was not clinically suspected, each biopsy was free of cellular infiltrate. In the children, 291 biopsies were performed. Evidence of rejection was present in only seven biopsies (41%) from 17 episodes of clinically suspected rejection. Cellular rejection was discovered during routine rejection surveillance biopsies in asymptomatic patients in 23 (8.4%) of 274 biopsies. CONCLUSIONS: In neonates with clinically suspected rejection, endomyocardial biopsy identified which patients did not require rejection therapy. Endomyocardial biopsy surveillance did not detect any unsuspected cases of rejection. In children, noninvasive rejection surveillance was less reliable even in asymptomatic patients, suggesting that periodic endomyocardial biopsy should be utilized.

Impact of arrhythmia circuit cryoablation during Fontan conversion for refractory atrial tachycardia.

Refractory atrial arrhythmias in late postoperative Fontan patients are usually associated with residual hemodynamic abnormalities and result in significant morbidity and mortality. Surgical revision of the Fontan anastomosis may improve hemodynamics without eliminating tachycardia. This study sought to assess the impact of surgical cryoablation of the arrhythmia circuit at the time of Fontan conversion on the clinical recurrence of tachycardia. Sixteen consecutive atriopulmonary Fontan patients with refractory atrial arrhythmias underwent surgical conversion to lateral tunnel total cavopulmonary anastomosis (15) or Fontan revision (1 patient). The initial 4 patients underwent Fontan conversions alone, without specific arrhythmia surgery. The subsequent 12 patients underwent electrophysiologically guided cryoablation of the tachycardia circuits at the time of surgical conversion. The mean age at Fontan revision was 15.6 +/- 3.8 years. Cryoablation was directed to 3 identified major tachycardia circuits: the inferomedial right atrium, the superior rim of the prior atrial septal defect patch, and along the lateral right atrial wall. Transmural antitachycardia pacemakers were implanted in 11 of the 16 patients. There was no surgical mortality in either group, and all patients improved in functional classification. All patients not undergoing cryoablation experienced recurrent symptomatic tachycardia requiring antiarrhythmic therapy (median follow-up, 54 months) versus 2 of 12 patients receiving cryoablation (median follow-up, 25 months; p <0.02). Thus, surgical cryoablation of the arrhythmia circuit at the time of Fontan conversion is highly effective in the management of refractory atrial arrhythmias, and is superior to Fontan conversion alone.

Reversal of severe late left ventricular failure after pediatric heart transplantation and possible role of plasmapheresis.

Late acute cardiac graft failure carries a high mortality in adults. Vascular mediators and factors other than classic T-cell-mediated rejection may play a role in this process, and aggressive multimodality therapy may improve survival. We report experience with plasmapheresis in treating late severe acute left ventricular dysfunction in a group of pediatric heart transplant recipients. We retrospectively reviewed clinical records, echocardiograms, hemodynamics, coronary angiograms, biopsy specimens, and treatment regimens for 5 patients with 7 episodes of late-onset severe graft failure who recovered. Plasmapheresis was applied in all cases, in addition to methylprednisolone, cyclophosphamide, lympholytic agents, and aggressive supportive care including mechanical ventilation and hemofiltration. All patients presented with acute severe left ventricular dysfunction 1.4 to 7.9 years (mean 3.6) after orthotopic heart transplantation. Mean shortening fraction at presentation was 13 to 23% (mean 16), initial endomyocardial biopsy specimens were grade 0 to 3B, and immunofluorescence studies were negative. Treatment included plasmapheresis, cyclophosphamide, mechanical ventilation, hemofiltration, and inotropes. Clinical recovery was slow, with 4 to 8 weeks until left ventricular function normalized, and 2.2 to 9.4 (mean 4.6) weeks to hospital discharge. At follow-up (50 to 38 months, mean 24), all are alive. Two patients are well, whereas coronary vasculopathy developed in 3. Thus, survival may improve in patients with late graft failure with low biopsy score and plasmapheresis combined with multimodality therapy.

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.

Repair of congenital tracheal stenosis with a free tracheal autograft.

OBJECTIVES: Evaluate the results of a technique for repair of congenital tracheal stenosis by use of a free tracheal autograft. METHODS: Between January 1996 and July 1997, six infants with congenital tracheal stenosis resulting from complete tracheal rings underwent repair with a free tracheal autograft. Mean age at the time of repair was 4.9 months; mean weight was 5.4 kg. The approach was through a median sternotomy with cardiopulmonary bypass for respiratory support. The trachea was incised anteriorly through the area of stenosis, the midportion of the stenotic trachea was excised, and an end-to-end anastomosis was carried out posteriorly. The excised tracheal segment (1.3 to 2.2 cm long) was used as a free autograft to patch the lower trachea anteriorly. In four infants the autograft was augmented in the upper trachea with pericardium; in two patients with a shorter length of stenosis, the autograft completed the repair. Simultaneous pulmonary artery sling repair (4), ligation and division of patent ductus arteriosus (3), cricoid split (2), atrial and ventricular septal defect repair (1), and complete atrioventricular canal repair (1) were performed at the time of tracheal repair. RESULTS: The infants were extubated and discharged at a mean of 13 and 23 days postoperatively, respectively. One infant had recurrent tracheal stenosis related to the pericardial patch and required a tracheal stent and tracheostomy 4 months postoperatively. Our mean follow-up is 11 months. Bronchoscopic findings currently show widely patent tracheal lumina in all infants. CONCLUSIONS: The technique of free tracheal autograft with and without pericardial augmentation was successful in opening the airway of six infants with congenital tracheal stenosis and is currently our procedure of choice for children with this diagnosis.

Fontan conversion to cavopulmonary connection and arrhythmia circuit cryoblation.

OBJECTIVES: We review our surgical experience patients with atriopulmonary Fontan operations who had obstructive or arrhythmia indications for conversion to total cavopulmonary artery connections, arrhythmia circuit cryoablation, and placement of atrial antitachycardia pacemaker. METHODS: Fourteen patients (mean age 14 +/- 4 years) had conversion to total cavopulmonary artery connection 8 +/- 3 years after the original atriopulmonary Fontan operation primarily for atrial arrhythmias in 11, obstructive lesions in 2, and bradycardia with cyanosis in 1. Arrhythmia circuit cryoablation was performed on 11 patients and 10 had atrial antitachycardia pacemakers. Preoperative functional New York Heart Association class was IV in 9, III in 4, and II in 1. RESULTS: One patient had brain death (7%) presumably caused by resternotomy complications despite an excellent hemodynamic result. Another required reoperation for a maldeployed clamshell device after attempted fenestration closure. Average length of stay was 10 +/- 3 days; chest tubes were removed on day 7 +/- 3. There were no long-term deaths (mean follow-up 1.7 years, range 5 months to 5 years). Postoperative arrhythmias occurred in five patients, three of whom had successful termination by antitachycardia pacemaker and two who had pharmacologic control of their respective junctional ectopic and slow atrial tachycardia. All patients have improved to New York Heart Association class I or II. CONCLUSIONS: Total cavopulmonary artery conversion in association with arrhythmia circuit cryoablation and atrial antitachycardia pacemaker placement can be accomplished with low morbidity and mortality, oftentimes resulting in dramatic increases in functional class and control of life-threatening arrhythmias.

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.

Selective preoperative evaluation for possible N2 disease in carcinoma of the lung.

The efficacy of computed tomography and surgical mediastinal exploration in determining tumor resectability were retrospectively evaluated in 92 consecutive patients with non-small cell lung carcinoma. Status of mediastinal nodes was ultimately determined by surgical mediastinal exploration or thoracotomy. Patients were divided into three groups on the basis of chest roentgenography: Group I comprised 30 patients with peripheral T1 or T2 lesions with normal hilar and mediastinal shadows. Only one patient was found to have an involved node. Chest roentgenography had an accuracy rate of 96% and computed tomography, 93%. Thoracotomy is recommended without either computed tomography or surgical mediastinal exploration in this group. Group II comprised 47 patients with T1 or T2 lesions with an abnormal hilus, an abnormal mediastinal shadow, or either the hilus or mediastinum obscured by overlying parenchymal disease. Computed tomography revealed mediastinal nodes 1 cm or greater in size (abnormal node group) in 21 patients (45%) and smaller than 1 cm (normal node group) in 26 patients (55%). Surgical mediastinal exploration was performed in the abnormal node group and involved nodes were found in 17 of 21 patients (81%). In the normal node group, thoracotomy only was performed and no involved nodes were found. Computed tomography is recommended in all patients in Group II. Patients in the normal node group may be subjected to thoracotomy only and those in the abnormal node group should undergo surgical mediastinal exploration as the next diagnostic step before thoracotomy. Group III comprised 15 patients with grossly abnormal mediastinal shadows. Findings from computed tomography were abnormal in all 10 patients in whom it was done. Surgical mediastinal exploration was done in all 15 and yielded abnormal results in 14. It is recommended in this group that computed tomography is unnecessary and surgical mediastinal exploration should be the only diagnostic procedure. Thus, in potentially resectable non-small cell lung carcinoma, the use of computed tomography and surgical mediastinal exploration should be selective and should be determined by appropriate initial interpretation of the chest roentgenogram.

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.

Computed tomography in patients with esophageal perforation.

Contrast esophagram is the diagnostic procedure of choice in patients with clinically suspected perforation of the esophagus. In patients in whom the usual clinical signs or symptoms are unrecognized and in whom the diagnosis is obscure, the diagnosis of a perforated esophagus may be suggested by the finding of mediastinal fluid and air on CT. Three patients are reviewed. The perforations included one spontaneous, one from erosion of an esophageal carcinoma, and one iatrogenic. In two of the three patients, the diagnosis of perforated esophagus had not been made initially and in one patient the initial esophagram was interpreted as normal. Computed tomography of the chest in each patient led to the suspected diagnosis of perforated esophagus. Prompt appropriate surgical intervention followed. The findings of mediastinal fluid and more importantly mediastinal air on CT of the chest are strongly suggestive of esophageal perforation.

Total cavopulmonary conversion and maze procedure for patients with failure of the Fontan operation.

OBJECTIVES: Hemodynamic abnormalities and refractory atrial arrhythmias in patients late after the Fontan operation result in significant morbidity and mortality. We review our experience with conversion to total cavopulmonary artery connections and arrhythmia surgery. METHODS: Between 1994 and 2001, 40 patients underwent Fontan conversion and arrhythmia surgery. Significant hemodynamic lesions such as aortic aneurysm (n = 1), atrioventricular valve insufficiency (n = 8), and pulmonary arterioplasty (n = 9) were repaired concomitantly. Thirty-four patients were in New York Heart Association class III or IV. Mean age at the original Fontan operation was 7.5 +/- 6.5 years and mean age at Fontan conversion was 18.7 +/- 9.0 years. Arrhythmia surgery has evolved from isthmus cryoablation in 10 patients to right-sided maze in 16 patients for atrial reentry tachycardia. The maze-Cox III operation was used for 14 patients with atrial fibrillation. Atrial (n = 33) and dual-chamber (n = 5) pacemakers were placed. RESULTS: There has been no early mortality. Chest tubes were removed on postoperative day 9.0 +/- 6.0. Hospital stay was 11.8 +/- 6.6 days. Three patients required cardiac transplantation at 8 days, 9 months, and 33 months postoperatively. There was 1 death 2 years postoperatively from acute myocardial infarction. For the entire series, arrhythmia recurrence is 12.5%, with only 10% of patients receiving long-term antiarrhythmic medications; these patients were among the first 8 patients in our series. Most patients are in New York Heart Association class I or II. Bruce protocol in 12 patients showed increased tolerance (P <.05). CONCLUSIONS: Fontan conversion to total cavopulmonary connection with concomitant arrhythmia surgery is excellent therapy for patients whose Fontan repair has failed. Fontan conversion is safe, improves New York Heart Association class, improves exercise tolerance, and has a low incidence of recurrent arrhythmias.

Biventricular repair of hypoplastic right ventricle assisted by pulsatile bidirectional cavopulmonary anastomosis.

The right ventricle in patients with severe outflow obstruction or atresia and a small tricuspid valve often remains too hypoplastic even after optimal palliation to tolerate biventricular repair with closure of the atrial septal defect. In these patients, nonpulsatile cavopulmonary (Glenn) anastomosis has traditionally facilitated biventricular repair. In 1989, Billingsley and associates reported the addition of a bidirectional cavopulmonary anastomosis to the definitive biventricular repair in patients with hypoplastic right ventricle, pulmonary atresia, and intact ventricular septum. The atrial septal defect was left open with an adjustable snare for later closure. We report five patients with hypoplastic right ventricle (mean diastolic volume 48.4%, mean stroke volume 40.2% of predicted value) who had the atrial septal defect closed at the time of the biventricular repair. Four patients, who had the bidirectional cavopulmonary anastomosis supplementing the biventricular repair, had no evidence of excessive right atrial or superior vena cava hypertension postoperatively. One patient, who had atypical tetralogy of Fallot with tricuspid stenosis, developed recurrent pericardial tamponade and marked hepatomegaly following conventional tetralogy repair with closure of the atrial septal defect. These complications were controlled with the addition of bidirectional cavopulmonary anastomosis 2 months later. Postoperative hemodynamic or Doppler studies in these patients revealed pulsatile flow in the entire pulmonary artery system, including the artery distal to the Glenn anastomosis. This modification of biventricular repair allows primary closure of the atrial septal defect and provides pulsatile arterial flow in the entire pulmonary artery, even when the right ventricle is significantly hypoplastic.

Expanding indications for pediatric coronary artery bypass.

Pediatric coronary artery bypass has been done mostly for ischemic complications of Kawasaki disease. We reviewed our clinical experience between 1987 and 1994 with internal thoracic artery-coronary artery bypass in one infant and five children for varying indications. Indications for coronary bypass included Kawasaki disease (2), congenital left main coronary ostial stenosis, iatrogenic coronary cameral fistula, anomalous origin of the left coronary artery from the pulmonary artery, and single coronary artery traversing between the great arteries in a patient after cardiac transplantation. An additional cohort of 34 control patients of various ages and weights (1 day to 16.1 years, 2.6 kg to 62 kg) had angiographic measurements of the right coronary, left coronary, and left internal thoracic arteries with respect to the feasibility of performing coronary artery bypass. All six patients survived internal thoracic artery-left anterior descending coronary artery bypass without evidence of perioperative myocardial infarction. Postoperative angiographic studies in five and color Doppler echocardiography in one showed graft patency. Retrospective angiographic measurements in the 34 control patients showed that internal thoracic and coronary arteries are proportionately quite large in neonates and infants compared with those in older children and adolescents. Internal thoracic artery-coronary artery bypass should be considered for the expanding indications presented herein and when emergency intraoperative life-threatening situations present themselves. Long-term patency and reoperation rates have yet to be determined.

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.

Transposition of the great arteries with intact ventricular septum. Arterial switch in the first month of life.

Twenty-three infants with simple transposition of the great arteries and intact ventricular septum were operated on from October 1983 to October 1986. The age at operation in 22 infants ranged from 2 to 21 days and in one was 35 days (mean 9.82 +/- 6.86 days). The infants were evaluated with cardiac catheterization at 1 to 27 days of age. Twenty-two infants had balloon atrial septostomy, and 22 received prostaglandin E1 infusion. The left ventricular diastolic wall thickness, assessed by M-mode echocardiograms, varied between 2.8 and 4 mm. There were two hospital deaths in this group of 23 infants (mortality 8.6%), and there were no late deaths. All surviving patients are doing well clinically. One patient had asymptomatic nonsustained ventricular tachycardia necessitating phenytoin. Postoperative echocardiographic assessment performed on 15 patients at 0.93 +/- 0.61 years of age and cardiac catheterization and angiographic studies on seven patients at 1.07 +/- 0.13 years after operation revealed excellent ventricular performance, good semilunar valve function, and mild gradient at the right ventricular outflow with a mean right ventricular pressure of 37.4 +/- 4.1 torr.

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.

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.