Nonconfluent Pulmonary Arteries Research Articles

Pulmonary Arterial Reconstruction for Pulmonary Coarctation in Early Infancy

Pulmonary atresia with pulmonary coarctation may complicate diminished and unbalanced pulmonary development. The aim of this study is to assess the outcome of pulmonary arterial reconstruction with cardiopulmonary bypass in early infancy for sufficient and balanced pulmonary development. We performed a retrospective review of 15 patients with pulmonary coarctation younger than 4 months of age who underwent pulmonary arterial reconstruction between 2001 and 2005. The mean age and weight were 42.2 days and 3.62 kg, respectively. The patient population included 5 biventricular repair candidates and 10 Fontan candidates. To evaluate the pulmonary arterial development, the preoperative and postoperative pulmonary arterial index and minimum diameter of the pulmonary artery were compared. No early or in-hospital deaths occurred, and there was no nonconfluent pulmonary artery development or segmental mal-development after a mean follow-up period of 14.9 months. Immediate pulmonary flow regulation was required in 2 patients because of excessive pulmonary flow. The mean pulmonary arterial index increased significantly from 103 mm2/m2 to 343 mm2/m2, and the mean minimum diameter of the pulmonary artery increased significantly from 2.02 mm to 4.45 mm. Four biventricular repair candidates completed definitive repair, and 2 required surgical reintervention in the pulmonary artery. Six Fontan candidates completed the Glenn procedure, and 1 completed the Fontan procedure. Three required surgical reintervention in the pulmonary artery. Two late deaths occurred after the Glenn procedure because of ventricular dysfunction and respiratory infection. Pulmonary arterial reconstruction in early infancy provides sufficient and balanced pulmonary arterial development for pulmonary atresia with pulmonary coarctation.

Surgical Outcome of Heterotaxy Syndrome in a Single Institution

Heterotaxy syndrome encompasses a wide range of anatomical variants including poor pulmonary vascular development. Surgical outcome remains poor in this difficult subgroup. Between April 1996 and November 2004, 27 patients with visceral heterotaxy were enrolled in this study. The median age at presentation was 25 days. There were 11 patients with asplenia and 16 with polysplenia. There were 5 deaths (4 in patients with asplenia, 1 in a patient with polysplenia). All patients with asplenia were scheduled for single-ventricle repair, and 5 completed a modified Fontan procedure. Of the 16 patients with polysplenia, 8 completed 2-ventricle repair and 6 had a modified Fontan. In polysplenia, actuarial survival was 93.8% at 4 months, and remained constant thereafter. In asplenia, survival was 81.8% at 1 month, and 53.0% at 1 year. A significant correlation was found between nonconfluent pulmonary arteries and mortality. The medium-term result in polysplenia was satisfactory, but not in asplenia. Nonconfluent pulmonary arteries carry a high risk of mortality, and a strategy to create pulmonary vascular confluence and satisfactory pulmonary blood flow is mandatory.

Successful early surgical recruitment of the congenitally disconnected pulmonary artery

Background Our purpose was to document our experience with early recruitment of congenitally disconnected pulmonary arteries and to assess subsequent pulmonary artery growth and function. Methods Patients born in the 10-year period from 1989 to 1999 with a disconnected pulmonary artery diagnosed in infancy and treated in our unit were studied. To be included patients had nonconfluent pulmonary arteries with one or both completely disconnected from the main pulmonary artery. This series did not include patients with acquired stenosis causing occlusion of a pulmonary artery. Echocardiography, cardiac catheterization, MRI, lung perfusion scans, and intraoperative assessment were used to gauge pulmonary artery growth and function. Results Seven patients with a disconnected pulmonary artery associated with intracardiac conotruncal congenital cardiac disease underwent successful early surgical recruitment of the affected pulmonary artery at 3 months of age or younger. Median follow-up from date of first operation was 4.2 years (range, 1.6 to 13.4). All 7 patients had postrecruitment lung perfusion scans showing a mean of 44% (range, 27% to 78%) of total pulmonary flow through the affected lung. Significant growth in the diameter of the recruited native pulmonary artery was demonstrated in all patients. There were no deaths reported in our series to date. Conclusions The rare possibility of a congenitally disconnected pulmonary artery needs to be considered in all patients with a conotruncal cardiac anomaly. To facilitate surgical correction, ensure subsequent growth of the pulmonary artery, and optimize associated lung development, early diagnosis and surgical recruitment is recommended.

Management of Pulmonary Atresia with Ventricular Septal Defect.

It has been nearly 40 years since Kirklin, at the Mayo Clinic in 1964, performed the first surgical repair of pulmonary atresia with ventricular septal defect using a nonvalved extracardiac conduit, which he created out of the patient's pericardium, and this patient continues to do well. In the subsequent four decades, great advances have been made in the diagnosis, with regard to the often very complex anatomy of pulmonary blood supply that this extremely heterogeneous group of patients manifest, and their subsequent surgical management. Unifocalization procedures have permitted eventual complete correction in patients with nonconfluent pulmonary artery segments, and advances in infant surgery have enabled most patients to achieve correction in the early childhood years. Although the ideal extracardiac conduit has yet to be developed, necessitating periodic conduit replacement in corrected patients, the late results in this group of patients are generally excellent, with many now achieving survival well into the adulthood years with good quality of life. Continued advances in surgery and interventional catheterization techniques hold great promise for ever-improving outcomes in children being born with this complex congenital cardiovascular malformation.

Echocardiographic evaluation, management and outcomes of bilateral arterial ducts and complex congenital heart disease: 16 years' experience.

Bilateral arterial ducts represent an uncommon form of pulmonary or systemic blood supply associated with complex congenital cardiac disease. We sought to determine the role of high-resolution cross-sectional echocardiography and color flow Doppler ultrasonography in assessing this condition, as well as to describe the management and outcome in a group of patients. A retrospective review was conducted of 11 newborns identified over a 16-year period as having bilateral arterial ducts. Pulmonary atresia associated with non-confluent pulmonary arteries was the dominant lesion, with the heterotaxy syndrome also frequently being recognized. Echocardiography best identified the source of blood supply to either the pulmonary or systemic circulations, allowing differentiation from collateral vessels. Stenosis of the right or left pulmonary artery at the initial site of ductal insertion needs careful evaluation on follow-up. Management of patients with this condition remains a challenge, as indicated by the poor outcomes observed in our series.

Outcomes and impact of a nonconfluent pulmonary artery originating from the distal end of the arterial duct in 43 patients

Outcomes and impact of a nonconfluent pulmonary artery originating from the distal end of the arterial duct in 43 patients

Double inlet right ventricle versus other types of double or common inlet ventricle: its clinical characteristics with reference to the Fontan procedure.

To determine the clinical features in patients with double inlet right ventricle (DIRV) and separate atrioventricular (AV) valves in terms of the Fontan procedure. Surgical procedures were carried out in 31 patients with this rare malformation. Their clinical characteristics were compared with similar features in 45 patients with double inlet left ventricle (DILV), 93 with common inlet right ventricle (CIRV), and 20 with common inlet left ventricle (CILV). Pulmonary atresia with the right and the left pulmonary arteries being confluent was seen in 58% of patients with DIRV, and pulmonary atresia with non-confluent pulmonary arteries in 15% (P=0.0001). The incidence of these findings was significantly higher when compared with DILV (P=0.0001). Reflecting these morphologic features, constructions of systemic-to-pulmonary shunts and extensive plasty to the pulmonary arteries were more frequently needed in patients with DIRV than in the others (P=0.04, 0.0001). The AV valves have become moderately or severely regurgitant in three with DIRV (10%). The Fontan procedure has thus been employed in 21 with DIRV (64%), this incidence being significantly higher than that seen in the CIRV (37%) group (P=0.01). Survival rate of 95% at 10 years in this group was as excellent as that in the DILV group, and obviously better than that in either CIRV or CILV (P=0.002). Although multiple palliative procedures might be frequently needed to maintain pulmonary perfusion, the Fontan circulation can be justifiably established in patients with DIRV, and regurgitation across the AV valves was not very common.

Truncus arteriosus repair: outcomes, risk factors, reoperation and management.

Truncus arteriosus (TA) continues to be associated with significant morbidity and mortality, but there have been clinically significant improvements with early repair. Sixty patients underwent physiological correction of TA between November 1978 and January 2000. The average age was 76 days (range, 3 days--20 months). Associated cardiac anomalies were frequently encountered, the most common being severe truncal valve regurgitation (n=7), interrupted aortic arch (n=6), coronary artery anomalies (n=6), non-confluent pulmonary arteries (n=4), and total anomalous pulmonary venous return (n=1). Truncal valve replacement was performed initially or subsequently in seven patients with severe regurgitation (mechanical prostheses in six patients and a cryopreserved aortic homograft in one patient). Right ventricle--pulmonary artery continuity was established with an aortic (n=16) or pulmonary homograft (n=32) in 48 patients, a Dacron polyester porcine valved conduit in five, a non-valved polytetrafluoroethylene (PTFE) tube in three, direct anastomosis to the right ventricle with anterior patch arterioplasty in three, and a bovine jugular venous valve conduit in one patient. There were ten hospital deaths (17%; 70% confidence limit, 7--25%). Multivariate and univariate analyses demonstrated a relationship between hospital mortality and associated cardiac anomalies. In the 43 patients without these associated cardiac anomalies, the early survival was 91% (group I). In the 17 patients with one or more of these risk factors, the survival was 71% (group II, P=0.002). There was one late death. Twenty-three patients (46%) required reoperation for right ventricular outflow tract (RVOT) obstruction at a mean follow-up time of 59.1 months. In 23 patients, the RVOT reconstruction was performed with a PTFE monocusp, and six patients had of a variety of replacement conduits inserted. Postoperatively, there were 34 (68%) patients in New York Heart Association functional class I and 16 (32%) in class II. Twenty-eight surviving patients are reported as doing well without any medication. The freedom of reoperation in the 39 hospital survivors (group I) without risk factors was 64% at 7 years; and 36% at 10 years in the 11 patients (group II) surviving with risk factors. Associated cardiac anomalies were risk factors for death after the repair of TA. In the absence of these associated lesions, TA can be repaired with an excellent surgical outcome in the neonatal and early infancy period.

Complete repair of tetralogy of Fallot in the neonate: results in the modern era.

To review more than a decade of experience with complete repair of tetralogy of Fallot (TOF) in neonates at the University of Michigan; to assess early and late survival, perioperative complications, and the incidence of reoperation; and to analyze patient, procedural, and morphologic risk factors to determine their effects on outcome. Palliation of TOF with systemic-to-pulmonary artery shunts has been the accepted standard for symptomatic neonates and infants. Complete repair has traditionally been reserved for infants older than 6 months of age because of the perception that younger and smaller infants face an unacceptably high surgical risk. A retrospective review from August 1988 to November 1999 consisted of 61 consecutive symptomatic neonates with TOF who underwent complete repair. Thirty-one patients had TOF with pulmonary stenosis, 24 had TOF with pulmonary atresia, and 6 had TOF with nonconfluent pulmonary arteries. The mean age at repair was 16 +/- 13 days, and the mean weight was 3.2 +/- 0.7 kg. Before surgery, 36 patients were receiving an infusion of prostaglandin, 26 were mechanically ventilated, and 11 required inotropic support. Right ventricular outflow tract obstruction was managed with a transannular patch in 49 patients and a right ventricle-to-pulmonary artery conduit in 12. Cardiopulmonary bypass time averaged 71 +/- 26 minutes. Hypothermic circulatory arrest was used in 52 patients (mean 38 +/- 12 minutes). After cardiopulmonary bypass, the average intraoperative right/left ventricular pressure ratio was 55% +/- 13%. There were no new clinically apparent neurologic sequelae after repair. The postoperative intensive care unit stay was 9.1 +/- 8 days, with 6.8 +/- 7 days of mechanical ventilation. There was one hospital death from postoperative necrotizing enterocolitis on postoperative day 71 and four late deaths, only one of which was cardiac-related. Actuarial survival was 93% at 5 years. Follow-up was available for all 60 hospital survivors and averaged 62 months (range 1-141 months). Twenty-two patients required a total of 24 reoperations at an average interval of 26 months after repair. Indications for reoperation included right ventricular outflow tract obstruction (19), branch pulmonary artery stenosis (11), severe pulmonary insufficiency (4), and residual ventricular septal defect (1). The 1-month, 1-year, and 5-year freedom from reoperation rates were 100%, 89%, and 58%, respectively. Complete repair of TOF in the neonate is associated with excellent intermediate-term survival. Although the reoperation rate is significant, this is to be expected with the complex right ventricular outflow tract and pulmonary artery anatomy seen in symptomatic neonates and the need for conduit replacement in patients with TOF with pulmonary atresia.

Right-sided aortic arch with bilateral ductus: a rare case of nonconfluent pulmonary arteries without associated cardiac anomalies

Right-sided aortic arch with bilateral ductus: a rare case of nonconfluent pulmonary arteries without associated cardiac anomalies

Contribución del Doppler color al diagnóstico de ductus arterioso bilateral

We report a neonate with distal bilateral ductus arteriosus origin of nonconfluent pulmonary arteries which was only diagnosed accurately by 2-D echo Doppler color flow mapping in the context of situs ambiguous, univentricular A-V connection and pulmonary atresia with normal systemic/pulmonary venous drainage. The color flow appearance of this singular vascular anatomy was enhanced after prostaglandin administration, which, allowed us to see an image resembling a "moustache morphology". At 36 months of age, and after a successful bilateral 4 mm modified Blalock Taussig shunt implantation by sequential approach, the patient is waiting for the next surgical step which will include a restoration of the confluence of the pulmonary arteries with bidirectional Glenn operation. Brief embryological and differential diagnosis comments are discussed.

5.6 The fontan operation in patients with non-confluent pulmonary arteries

5.6 The fontan operation in patients with non-confluent pulmonary arteries

Echocardiographic assessment of pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect

The purpose of this study was to determine the role of high resolution two-dimensional echocardiographic imaging and color flow Doppler study in assessing the pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect (VSD). Although echocardiography is a well established technique for assessing central pulmonary arteries in pulmonary atresia VSD and for determining the presence or absence of a patent arterial duct, few data are available on its role in patients whose source of blood supply is from collateral vessels. Forty-two patients aged a few hours to 19 months (mean 29 days) were prospectively assessed by high resolution echocardiography, including color flow Doppler study, during a 4-year period ending in 1994, before any intervention other than intravenous administration of prostaglandins. Angiographic confirmation was available in 29 patients, including 18 (95%) of 19 with aortopulmonary collateral channels. A patent arterial duct was correctly identified as the sole source of pulmonary blood supply in 23 patients, whereas aortopulmonary collateral channels were detected in 19, with one of these having a small patent arterial duct and collateral channels. The patent arterial duct originated from the undersurface of the aorta in 16 (67%) of 24 patients and from the base of the brachiocephalic trunk in 7 (33%) of 24. All patients with a patent ductus as the sole source of pulmonary blood supply had confluent pulmonary arteries. Nonconfluent pulmonary arteries were present in six patients, with all but one having aortopulmonary collateral channels as the sole source of pulmonary flow. Aortopulmonary collateral channels were direct in 17 (89%) of 19 patients, whereas in 2 (11%) of 19, both direct and indirect collateral channels were present. Color flow Doppler study was accurate in determining the presence or absence, the side and the origin of the collateral channels in all patients, with the correct number being determined in 12 (67%) of 18. "Wash-in" to the hilar pulmonary arteries (retrograde color flow) was seen in 12 (92%) of 13 patients with collateral channels and confluent pulmonary arteries. Failure to identify a tiny central pulmonary artery occurred in one patient. High resolution imaging and color flow Doppler study provide good appreciation of the source of pulmonary blood supply in neonates and young infants with pulmonary atresia VSD.

Pulmonary homograft implantation for ventricular outflow tract reconstruction: Early phase results

The pulmonary valve homograft (PH) has been reported to have potential advantages over the aortic valve homograft, including a larger diameter, a thinner wall, and decreased intrinsic calcification. From January 16, 1986, to July 14, 1987, eight consecutive patients underwent repair of congenital cardiac anomalies using a cryo-preserved PH. Patients ranged in age from 18 months to 32 years. Diagnoses included tetralogy of Fallot with pulmonary atresia (3 patients); tetralogy with absent pulmonary valve (1 patient); corrected transposition with pulmonic stenosis (1 patient); transposition of the great arteries, ventricular septal defect, and pulmonic stenosis (2 patients); and double-outlet right ventricle with pulmonic stenosis (1 patient). The PH was implanted orthotopically in the patient with absent pulmonary valve, and in the other 7 it was placed as a valved extracardiac conduit. Two of the tetralogy patients with severe bifurcational pulmonary stenosis and another with nonconfluent pulmonary arteries and origin of the left pulmonary artery from a patent ductus arteriosus had their repairs facilitated using the branching pulmonary arterial portion of the PH. There were no hospital or posthospital deaths. Postrepair right ventricular to left ventricular systolic pressure ratios were a mean of 0.35 at 18 hours postoperatively (range, 0.21–0.61). All patients were studied with Doppler and echocardiography after repair. The mean gradient across the PH was 9 mm Hg (range, 2–27 mm Hg), and no pulmonary valve incompetence was present. One patient (12.5%) required reoperation seven months after repair for conduit revision due to compression by the sternum and is now well. We conclude that the PH is a good conduit for ventricular outflow tract reconstruction, and the short-term results are satisfactory. The bifurcational portion of the donor pulmonary arteries has proved to be useful in the repair of associated pulmonary arterial anomalies. Because of its ready availability when procured in conjunction with the aortic homograft valve, and because of increased demand on our tissue bank for the use of aortic homograft valves, we have adopted the PH as our valve of choice for ventricular outflow tract reconstructive procedures.

Use of pulmonary arterial unifocalization into cryopreserved homograft pulmonary arterial bifurcation grafts to facilitate complete correction of pulmonary atresia with ventricular septal defect, nonconfluent (absent) central pulmonary arteries and multiple major aortopulmonary collateral arteries

AbstractThree children with pulmonary atresia and ventricular septal defect with absent central pulmonary arteries underwent successful complete correction following preliminary staging procedures for unifocalization and deletion of multiple major aortopulmonary collateral arteries. The unifocalization procedures were accomplished using valveless pulmonary arterial cryopreserved allografts. This technique was found to facilitate and simplify the final repair. All patients survived the complete correction. The patients have been followed for 1½-4 years following the final surgery. One child died two years after repair from pneumonia complicated by asthma. One patient appears to have progression of pulmonary vascular disease despite the complete repair, and one patient continues to do well both symptomatically and hemodynamically. The results suggest that this method of unifocalization solves many of the problems inherent to more classical techniques, but the overall prognosis of patients in this diagnostic entity has yet to be completely defined.

Intermediate results after complete repair of tetralogy of Fallot in neonates

From July 1988 through September 1993, 30 neonates with symptomatic tetralogy of Fallot underwent complete repair. Sixteen patients had tetralogy and pulmonary stenosis, 9 had pulmonary atresia, 3 had nonconfluent pulmonary arteries, and 2 had both pulmonary atresia and nonconfluent pulmonary arteries. The median age at operation was 11 days (mean ± standard error of the mean, 12.6 ± 2.9 days), with a mean weight of 3.1 ± 0.1 kg (range 1.5 to 4.4 kg). Preoperatively, 14 patients were receiving an infusion of prostaglandin, 13 were mechanically ventilated, and 6 required inotropic support. Right ventricular outflow tract obstruction was managed by a limited transannular patch in 25 patients, infundibular muscle division with limited resection in 15, and insertion of a right ventricle-pulmonary artery valved aortic homograft conduit in 5 patients. Follow-up was complete at a median interval of 24 months (range 1 to 62 months). There were no hospital deaths and two late deaths, for 1-month, 1-year, and 5-year actuarial survivals of 100%, 93%, and 93%, respectively. The hazard function for death had a rapidly declining single phase that approached zero by 6 months after the operation. Both late deaths occurred in patients with tetralogy of Fallot and pulmonary atresia who had undergone aortic homograft conduit reconstruction, so that the only independent risk factor for death was the use of a valved homograft conduit (p ≤ 0.005). Eight patients required reoperation, resulting in 1-month, 1-year, and 5-year freedom from reoperation rates of 100%, 93%, and 66%, respectively. Indications for reoperation were branch left pulmonary artery stenosis in 5 patients, residual right ventricular outflow tract obstruction in 2 patients, and severe pulmonary insufficiency in 1 patient. Independent risk factors for reoperation included an intraoperative pressure ratio between the right and left ventricles of 0.75 or greater ( p = 0.01), Doppler residual left pulmonary artery stenosis of 15 mm Hg or more, or Doppler right ventricular outflow tract obstruction gradient of 40 mm Hg or more at hospital discharge ( p = 0.002 and 0.02, respectively). This series demonstrates the safety of early hemodynamic repair of symptomatic tetralogy of Fallot in neonates. It also emphasizes the importance of relieving all sources of right ventricular outflow tract obstruction at the initial operation, particularly that located at the site of insertion of the ductus arteriosus, which may be difficult to diagnose in the neonate before ductal closure occurs. The safety and efficacy of valved aortic homograft conduits in neonates requires further investigation. (J T horac C ardiovasc S urg 1995;109:332-44)

Modified fontan operation with reconstruction of the nonconfluent pulmonary artery

A modification of the Fontan operation for tricuspid atresia with a nonconfluent pulmonary artery after a Glenn shunt is reported. Confluence was established posteriorly using an inverted right atrial flap and anteriorly with a xenopericardial patch.

Pediatric lung transplantation

From July 1990 to April 1993, 36 lung transplantations in 33 patients were performed in our pediatric transplant program (0.25 to 23 years, mean age 10.3 years). Eight children had been continuously supported with a ventilator for 3 days to 4.5 years before transplantation and three were supported by extracorporeal membrane oxygenation. Indications for lung transplantation in this pediatric population included the following: cystic fibrosis (n = 13), pulmonary hypertension, and associated congenital heart disease (n = 10), pulmonary atresia, ventricular septal defect and nonconfluent pulmonary arteries (n = 3), pulmonary fibrosis (n = 6), and acute respiratory distress syndrome (n = 1). Three children underwent retransplantation for acute graft failure (n = 2) or chronic rejection (n = 1). Pulmonary fibrosis was related to complications of treatment of acute of myelogenous leukemia with bone marrow transplantation in two children and to bronchiolitis obliterans, bronchopulmonary dysplasia, interstitial pneumonitis, and Langerhans cell histiocytosis in four others. Thirteen children underwent lung transplantation and concomitant cardiac repair. Bilateral lung transplantation, ventricular septal defect closure and pulmonary homograft reconstruction of the right ventricular outflow tract to the transplanted lungs was performed in three children by means of a new technique that avoids the need for combined heart-lung transplantation. Two patients had ventricular septal defect closure and single lung transplant for Eisenmenger's syndrome, two had ligation of a patent ductus arteriosus and transplantation, three additional children underwent atrial septal defect closure and lung transplantation, and two underwent lung transplantation for congenital pulmonary vein stenosis. Eight early deaths and three late deaths occurred (actuarial 1-year survival 62%). Lung transplantation in children has been associated with acceptable early results, although modification of the adult implantation technique has been necessary. Lung transplantation and repair of complex congenital heart defects is possible; heart-lung transplantation may only be required for patients with severe left heart dysfunction and associated pulmonary vascular disease. Bronchiolitis obliterans remains a major concern for long-term graft function in pediatric lung transplant recipients.

A case of bilateral ductus arteriosus associated with pulmonary atresia and nonconfluent pulmonary artery

A case of bilateral ductus arteriosus associated with pulmonary atresia and nonconfluent pulmonary artery

Results of a policy of primary repair of truncus arteriosus in the neonate

Although the early mortality for repair of truncus arteriosus has decreased in the modern era, routine correction in the neonate has not been widely adopted. To assess the results of our protocol of early repair, we reviewed 46 neonates and infants undergoing repair of truncus arteriosus at the University of Michigan Medical Center from January 1986 to January 1992. Their ages ranged from 1 day to 7 months (median 13 days) and weights from 1.8 kg to 5.4 kg (mean 3.1 kg). Repair was performed beyond the first month of life in only 8 patients, because of late referral in 7 and severe noncardiac problems in 1. Associated cardiac anomalies were frequently encountered, the most common being interrupted aortic arch (n = 5), nonconfluent pulmonary arteries (n = 4), hypoplastic pulmonary arteries (n = 4), and major coronary artery anomalies (n = 3). Truncal valve replacement was performed in 5 patients with severe regurgitation, 3 of whom also had truncal valve systolic pressure gradients of 30 mm Hg or more. The truncal valve was replaced with a mechanical prosthesis in 2 patients and with a cryopreserved homograft in 3 patients. Right ventricle-pulmonary artery continuity was established with a homograft in 41 patients (range 8 mm to 15 mm), a valved heterograft conduit in 4 (range 12 mm to 14 mm), and a nonvalved polytetrafluoroethylene tube in the remaining patient (8 mm). There were 5 hospital deaths (11%, 70% confidence limits 7% to 17%). Multivariate and univariate analyses failed to demonstrate a relationship between hospital mortality and age, weight, or associated cardiac anomalies. Only 1 death occurred among 9 patients with interrupted aortic arch or nonconfluent pulmonary arteries. Hospital survivors were followed-up from 3 months to 6.3 years (mean 3 +/- 0.4 years). Late noncardiac deaths occurred in 3 patients, all within 4 months after the operation. Actuarial survival was 81% +/- 6% at 90 days and beyond. Despite the prevalence of major associated conditions, early repair has resulted in excellent survival. We continue to recommend repair promptly after presentation, optimally within the first month of life.