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Truncus arteriosus is a congenital heart defect with high resource use, cost, and mortality. Value assessment (outcome relative to cost) can improve quality of care and decrease cost. This study hypothesized that truncus arteriosus repair at a high-volume center would result in better outcomes at lower cost (higher value) compared with a low-volume center.This study retrospectively analyzed a multicenter cohort of neonates undergoing truncus arteriosus repair (2004 to 2015) by using the Pediatric Health Information Systems database. Multivariate quantile, logistic, and negative binomial regression models were used to evaluate total hospital cost, in-hospital mortality, ventilation days, intensive care unit length of stay (LOS), hospital LOS, and days of inotropic agent use by center volume (high-volume3/year) and age at repair while adjusting for sex, ethnicity, race, genetic abnormality, prematurity, low birth weight, concurrent interrupted arch repair, and truncal valve repair.Of 1024 neonates with truncus arteriosus, 495 (48%) were treated at high-volume centers. Costs at the 75th percentile were lower at high-volume vs low-volume centers by $28,456 (P = .02) at all ages at repair. Patients at high-volume centers had lower median postoperative ventilation days (5 days vs 6 days; P.001), intensive care unit LOS (13 days vs 19 days; P.001), hospital LOS (23 days vs 28 days; P = .02), and inotropic agent use (3 days vs 4 days; P = .004). In-hospital mortality did not differ by center volume.In neonates undergoing truncus arteriosus repair, costs are lower and outcomes are better at high-volume centers, thus resulting in higher value at all ages of repair. Value-based interventions should be considered to improve outcomes and decrease cost in truncus arteriosus care.

Surgery for truncus arteriosus has an early mortality of 3% to 20%, with a long-term survival of approximately 75% at 20 years. Nowadays, truncus arteriosus repair is mostly done in the neonatal period together with a single-staged repair of concomitant cardiovascular anomalies. There are many challenging subgroups of patients with truncus arteriosus, including those with clinically significant truncal valve insufficiency, an interrupted aortic arch, or a coronary artery anomaly. In fact, truncal valve competency appears to be the most important factor influencing the outcomes after truncus arteriosus repair. The use of a conduit during truncus arteriosus repair invariably requires reoperation on the right ventricular outflow tract. Through improvements in perioperative techniques over time, many children are now living well into adulthood after repair of truncus arteriosus, albeit with a high rate of reoperation. Despite this, the long-term outcomes of truncus arteriosus repair are good, with many patients being asymptomatic and with a quality of life comparable to the general population.

Background Literature describing morbidity and mortality after truncus arteriosus repair is predominated by single-center reports. We created and analyzed a multicenter dataset to identify risk factors for late mortality and right ventricle-to-pulmonary artery (RV-PA) conduit reintervention for this patient population. Methods We retrospectively collected data on children who underwent repair of truncus arteriosus without concomitant arch obstruction at 15 centers between 2009 and 2016. Cox regression survival analysis was conducted to determine risk factors for late mortality, defined as death occurring after hospital discharge and greater than 30 days after operation. Probability of any RV-PA conduit reintervention was analyzed over time using Fine-Gray modeling. Results We reviewed 216 patients with median follow-up of 2.9 years (range, 0.1 to 8.8). Operative mortality occurred in 15 patients (7%). Of the 201 survivors there were 14 (7%) late deaths. DiGeorge syndrome (hazard ratio [HR], 5.4; 95% confidence interval [CI], 1.6 to 17.8) and need for postoperative tracheostomy (HR, 5.9; 95% CI, 1.8 to 19.4) were identified as independent risk factors for late mortality. At least one RV-PA conduit catheterization or surgical reintervention was performed in 109 patients (median time to reintervention, 23 months; range, 0.3 to 93). Risk factors for reintervention included use of pulmonary or aortic homografts versus Contegra (Medtronic, Inc, Minneapolis, MN) bovine jugular vein conduits (HR, 1.9; 95% CI, 1.2 to 3.1) and smaller conduit size (HR per mm/m2, 1.05; 95% CI, 1.03 to 1.08). Conclusions In a multicenter dataset DiGeorge syndrome and need for tracheostomy postoperatively were found to be independent risk factors for late mortality after repair of truncus arteriosus, whereas risk of conduit reintervention was independently influenced by both initial conduit type and size.

Background The purpose of this study was to determine whether aortic biomechanical properties are abnormal in children with repaired truncus arteriosus (TA) and to concurrently evaluate left ventricular (LV) function post-repair utilizing a novel platform for regional ventricular function. Methods Cardiac magnetic resonance (CMR) studies from 26 children (mean age: 15.6 ± 7.2 years) post-TA repair were compared with 20 normal controls (mean age: 14.7 ± 2.6 years). Parameters of aortic stiffness (pulse wave velocity and relative area change) were measured. Flow hemodynamic metrics (aortic regurgitant fraction, peak systolic flow, and peak systolic velocity) and LV function (volumetric data, ejection fraction, regional wall strain) were also compared. Results Ascending aortic pulse wave velocity was elevated and relative area change was decreased in TA patients compared with controls. Patients post-TA repair demonstrated elevated end diastolic and end systolic volumes in addition to decreased regional wall strain and increased mechanical dyssynchrony. LV functional changes were independent of aortic biomechanical properties. Conclusions Children with repaired TA have increased ascending aortic stiffness and altered LV function as measured by CMR imaging. Longitudinal studies and advanced CMR assessments are warranted to better determine the long-term potential for late aortic complications and to optimize both the medical and surgical management of these patients after TA repair.

Background Truncus arteriosus is associated with coronary anomalies. We identified coronary artery lesions in patients undergoing repair of truncus arteriosus, defined the impact of lesions on mortality, and studied the effect of surgical intervention of coronary lesions. Methods A retrospective review identified 107 patients with truncus repair (1995-2019). Coronary lesions were categorized as ostial stenosis, intramural, juxtacommissural origin, and single coronary. Survival analysis characterized survival after truncus repair and studied the association of coronary lesions and mortality. Results Among 107 patients with truncus repair 34 patients had at least 1 coronary lesion. Median follow-up time was 7 years, with 85% 5-year survival. Coronary lesions including ostial stenosis, intramurality, and juxtacommissural origin were associated with increased mortality, whereas single coronaries did not impact survival. Eleven patients had 1 coronary lesion and 6 patients with 2 coronary lesions had similar (80% and 83%, respectively) 5-year survival. Eight patients with 3 coronary lesions had 24% 5-year survival (P = .0003). Among patients with 1 or 2 lesions, surgical intervention on the coronary lesions tended to be associated with longer 5-year survival (100% vs 62%, respectively; P = .06). All patients with 3 lesions underwent coronary artery intervention, with 24% 5-year survival. Conclusions Impact of coronary lesions on mortality after truncus repair increases with the number of lesions. Coronary artery intervention may be associated with improved time-related survival among patients with 1 or 2 lesions. Patients with the most complex anomalies (3 lesions) have poor survival and warrant ongoing study of repair techniques.

Background Multiple conduits for right ventricular outflow tract reconstruction exist, although the ideal conduit that maximizes outcomes remains controversial. We evaluated long-term outcomes and compared conduits for right ventricular outflow tract reconstruction in children with truncus arteriosus. Methods Records of patients who underwent truncus arteriosus repair at our institution between 1981 and 2018 were retrospectively reviewed. Primary outcomes included survival and freedom from catheter reintervention or reoperation. Secondary analyses evaluated the effect of comorbidity, operation era, conduit type, and conduit size. Results One hundred patients met inclusion criteria. Median follow-up time was 15.6 years (interquartile range, 5.3-22.2). Actuarial survival at 30 days, 5 years, 10 years, and 15 years was 85%, 72%, 72%, and 68%, respectively. Early mortality was associated with concomitant interrupted aortic arch (hazard ratio, 5.4; 95% confidence interval, 1.7-17.4; P = .005). Median time to surgical reoperation was 4.6 years (interquartile range, 2.9-6.8; n = 58). Right ventricle to pulmonary artery continuity was established with an aortic homograft (n = 14), pulmonary homograft (n = 41), or bovine jugular vein conduit (n = 36) in most cases. Multivariate analysis revealed longer freedom from reoperation with the bovine jugular vein conduit compared with the aortic homograft (hazard ratio, 3.1; 95% confidence interval, 1.3-7.7; P = .02) with no difference compared with the pulmonary homograft. Larger conduit size was associated with longer freedom from reoperation (hazard ratio, 0.7; 95% confidence interval, 0.6-0.9; P Conclusions The bovine jugular vein conduit is a favorable conduit for right ventricular outflow tract reconstruction in patients with truncus arteriosus. Concomitant interrupted aortic arch is a risk factor for early mortality.

Interrupted right aortic arch is a rare congenital cardiovascular anomaly typically associated with other forms of congenital heart disease. We report two cases of interrupted right aortic arch associated with isolated left pulmonary artery and bilateral ductus arteriosus in the first case and with truncus arteriosus in the second case. Computed tomography was complementary to echocardiography in delineating the anatomic details in both cases, informing surgical repair with reconstruction of a left aortic arch in the first case and right arch in the second case.

Truncus arteriosus with the absence of one branching pulmonary artery and presence of major aortopulmonary collateral arteries is rare. A small patient, with a birth weight of 2,219 g, was successfully repaired by a staged approach, after banding of the left pulmonary artery and unifocalization of major aortopulmonary collateral arteries.

Background Clinical indications and outcomes for children requiring multiple extracorporeal membrane oxygenation (ECMO) runs during a single hospital admission are rarely reported. We review indications, cannulation techniques, and outcomes for infants requiring multiple ECMO runs after congenital heart surgery. Methods Demographic and ECMO-related data for all children requiring multiple ECMO runs during a single hospital admission from1995 to 2008 were extracted from our institution's ECMO database. Survival was defined as survival to hospital discharge. Demographic and ECMO data were compared between survivors and nonsurvivors using the Mann-Whitney test for continuous and Fisher's exact test for categorical data. Results Twenty children (5% of 391 ECMO runs) with a median age of 5 days (range, 4 to 180 days) and weight of 3.22 kg required repeat ECMO support for persistent cardiopulmonary dysfunction despite successful weaning from an index ECMO run. Of the two-ventricle repair group, tetralogy of Fallot, truncus arteriosus, and transposition constituted the majority of cardiac diagnoses (75%), whereas single-ventricle physiology made up the rest (25%). The median total ECMO duration was 191 hours (range, 77 to 485 hours), and median hospital length of stay was 41 days (range, 6 to 297 days). Five (25%) patients survived to discharge. When survivors were compared with nonsurvivors, there were no significant differences in demographics, single or biventricular repair, indication for ECMO support, or need for surgical reoperation while on ECMO. However, the incidence of renal failure was higher in nonsurvivors than in survivors after multiple ECMO runs (12 versus 2.0; p = 0.03). Conclusions Survival in children undergoing congenital heart surgery requiring multiple ECMO runs is low, but 1 in 4 patients will survive to discharge. If a correctable lesion or ventricular impairment is present, repeat ECMO support may be justified. Development of renal failure is associated with nonsurvival in these patients.

Outcomes for truncus arteriosus repair are impacted significantly by the severity of truncal valve dysfunction. When satisfactory repair of the regurgitant truncal valve is unattainable, replacement is required. Given our experience in children with stentless porcine xenografts in the aortic position and the incidence of early valve failure for aortic homografts in infants, we replaced a severely regurgitant truncal valve with a full-root porcine xenograft in a 3-month-old infant. The initial and early result are encouraging, suggesting that the stentless porcine xenograft may be considered an option in cases where primary repair of the truncal (or aortic) valve is not possible.

The association between truncus arteriosus and total anomalous pulmonary venous return is exceedingly rare. Here described is a neonate presenting with a common arterial trunk and a previously unreported "scimitar-like" mixed total pulmonary venous return who underwent a successful one-stage repair. The unprecedented anatomic pattern of the systemic and pulmonary venous connection and the original surgical strategy are discussed.

Background Truncus arteriosus (TA) is an uncommon congenital cardiac lesion that portends an exceedingly poor prognosis if not repaired. The objective of this study was to assess the clinical and developmental outcomes in a prospective cohort of patients who underwent TA repair. Methods All patients who underwent a TA repair between 1996 and 2012 were included. Follow-up clinical, neurologic, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database. Functional developmental outcomes were assessed at 21.1 ± 2.5 months of age with the Adaptive Behavior Assessment System-II, General Adaptive Composite (GAC) score. Survival and outcomes were compared between those with and without chromosomal abnormalities (CA). Survival and freedom from reintervention were assessed by Kaplan-Meier analysis. Results The study comprised 36 infants (19 male). CA was identified in 13, with 22q11.2 deletion in 10 patients. Patients underwent TA repair at a median age of 10 days; 5 patients underwent concomitant interrupted arch repair. There were 8 deaths, 2 of which occurred in the hospital. The 5-year survival was 79.4%. Survival was similar between those with and without CA. At 5 years, freedom from reoperation was 77.2%. The mean GAC was higher in the patients without CA (93.6 ± 12.8 vs 76.1 ± 13.1, p = 0.0016). Conclusions Patients with surgically repaired TA continue to have significant postoperative mortality. Reoperation and cardiac catheterization are eventualities for a quarter of patients in the first 5 years of life. Functional developmental outcome in patients without CA is good, although it is significantly impaired in those with CA.

Background. Patients with both interrupted aortic arch (IAA) and truncus arteriosus (TA) have worse outcomes than those with either lesion in isolation. We determined outcomes and associated factors in this rare group. Methods. From 1987 to 1997, 50 (11%) of 472 neonates with IAA were identified with TA. Site of aortic arch interruption was distal to the left subclavian artery in 16% and between the left common carotid and subclavian artery in 84%. From the common arterial trunk, the pulmonary arteries arose from a main pulmonary trunk in 46%, common orifice in 22%, and separate orifices in 32%. At presentation, truncal valve stenosis was present in 12% and regurgitation in 22%. Results. There were 34 deaths, with a single early hazard phase. Overall survival from admission was 44%, 39%, and 31% at 6 months, 1 year, and 10 years, respectively. One patient had primary cardiac transplantation and 4 died without any intervention. The IAA repair alone was performed in 7 patients, with single stage repair of both IAA and TA in 38 patients. Associated factors for overall time-related death include female gender (p < 0.001), type III TA (p < 0.001) and one institution (low-risk; p < 0.001). Results improved somewhat over time (p < 0.001). At 5 years after IAA repair only 28% were alive without arch repair intervention, and at 5 years after TA repair only 18% were alive without conduit reoperation. Conclusions. The combination of IAA and TA carries high early mortality, with high risk of reinterventions in survivors. One stage repair of both TA and IAA is the optimal management.

Background Allograft conduits (AC) are used for reconstruction of the right ventricular outflow tract (RVOT) in children with RVOT hypoplasia or atresia and for pulmonary valve replacement in children undergoing Ross aortic valve replacement (AVR). Children undergoing the Ross AVR are generally older and have their AC inserted in the orthotopic position as opposed to the heterotopic position used for most cases of complex RVOT obstruction. The orthotopic position of the AC combined with the fact that the AC in the Ross patients are larger and can be oversized are the three factors that increase the right ventricular-pulmonary artery (RV-PA) conduit durability in the Ross AVR group. A retrospective evaluation of our experience with use of AC in non-Ross patients for reconstruction of the RVOT was conducted to determine the risk factors for failure. Methods Between January 1985 and December 2003, 117 non-Ross patients received AC (94 pulmonary and 23 aortic) for RVOT reconstruction. Median age at implantation was 8 months (mean 4.3 ± 7.1 years; range, 6 days to 43 years). There were 57 children (49%) less than 12 months of age. Endpoints were AC failure (valve explant, balloon dilatation), and AC dysfunction (AC stenosis >40 mm Hg and AC insufficiency more than 2+). There were no device-related deaths. Results Overall patient survival was 80% at 15 years. Freedom from AC failure was 60% at 5 years and 43% at 15 years. Freedom from failure was worse in infants (42% and 34% at 5 and 15 years, respectively). Freedom from AC dysfunction was 40% at 5 years and 23% at 15 years. Freedom from dysfunction was worse in infants (21% and 16% at 5 and 15 years, respectively). Univariate analysis identified younger patient age, smaller AC size, diagnosis of truncus arteriosus, and the presence of aortic AC as risk factors for AC dysfunction and failure. Multivariate analysis identified smaller AC size and the presence truncus arteriosus as risk factors for AC dysfunction and failure. Conclusions Right ventricular outflow tract reconstruction with an AC in non-Ross patients has poor performance at midterm follow-up with AC dysfunction and failure of, respectively, 60% and 40% for the entire group and 79% and 58% in the infant group at 5 years. An alternate conduit for this application must be considered.

The extant nomenclature for truncus arteriosus (TA) is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A modified Van Praagh (VP) classification is proposed involving three main categories of TA: TA with confluent or near confluent pulmonary arteries (large aorta type, VP A1, A2), TA with absence of one pulmonary artery (VP A3), and TA with interrupted aortic arch or coarctation (large pulmonary artery type, VP A4). A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Background . Despite improved surgical results for truncus arteriosus, overall mortality rates, remain higher than those reported for other complex congenital heart diseases, especially with truncal valve regurgitation or an interrupted aortic arch. Methods . Seventeen patients had complete repair of the truncus arteriosus at the Cleveland Clinic Foundation between August 1993 and June 1997. The age at operation ranged from 2 days to 4.5 years. Associated abnormalities included interrupted aortic arch in 3 patients and abnormal coronary artery anatomy in 3. Four patients had more than moderate truncal valve insufficiency requiring concomitant truncal valve repair. Results . There were no early deaths and only one late death at a mean follow-up of 24 months. The death occurred 3 months postoperatively and resulted from refractory pulmonary vascular obstructive disease in a patient who was referred at 1 year of age. Reoperation was required in 4 patients. Conclusions . Even in the presence of associated anomalies complete repair was performed with a low mortality rate. Truncal valve repair can be performed safely in the neonate with good results.

The single semilunar valve in patients with truncus arteriosus frequently is dysplastic and dysfunctional. Truncal valve insufficiency has been associated with poor outcome. Although the management of truncal valve insufficiency has evolved over the years, approaches to this problem vary considerably and remain a serious dilemma in many cases.We reviewed the records of 89 patients with unrepaired truncus arteriosus and mild (n = 37), moderate (n = 33), or severe (n = 19) truncal valve insufficiency who were admitted to our institution between 1975 and 1995. Eight patients (7 neonates) with moderate or severe insufficiency died before surgical intervention, and 4 patients underwent palliative pulmonary artery banding. The remaining 77 patients underwent repair. The median age at repair was 3.2 months (range, 2 days to 15 years; 83% infants), and it decreased from 4 months between 1975 and 1985 to 1 month between 1986 and 1995. Truncal valve replacement (mechanical = 6, allograft = 4) was performed in 10 patients, and 5 patients underwent valve repair.All 4 patients who underwent pulmonary artery banding died either early or late. The hospital (or 30-day) mortality rate after repair was 34% (26/77). At a median follow-up of 10 years, 11 hospital survivors had died, with overall 1- and 10-year actuarial survival rates of 56% and 48%, respectively, and poorer survival among patients with severe truncal valve insufficiency (p = 0.02). Late truncal valve replacement (n = 24) had been performed in 21 patients. Freedom from truncal valve replacement was better in patients with mild truncal insufficiency than in those with moderate or severe preoperative insufficiency (p0.001). Four late deaths were related directly to reoperation for truncal valve replacement or to prosthetic valve dysfunction. Three of the 4 neonates who received allograft root replacements died within 7 months of repair, and severe allograft valve insufficiency requiring replacement 1 year after operation developed in the fourth.The prospects for patients with truncal valve insufficiency have been improving over time. Nevertheless, the results in patients with severe insufficiency continue to be poor.

Although it has gained much interest in other surgical specialties, the application of near-infrared spectroscopy to assess cerebral perfusion during cardiac surgery is relatively new. Regional cerebral oxygen saturation (rSO2) is a function of cerebral oxygen supply and demand. Continuous monitoring of the rSO2 permits early detection of cerebral ischemia allowing for prompt intervention. The following is a description of a repair of truncus arteriosus with type A interrupted aortic arch during which continuous cerebral oximetry assisted with the positioning of the arterial cannula avoiding a prolonged episode of cerebral ischemia.

Background. This study describes the results of techniques using the autologous truncal wall and part of the pulmonary artery for correction in anticipation of the growth of the pulmonary tract in patients with truncus arteriosus. Methods. Seven consecutive patients with truncus arteriosus were reviewed. The posterior wall of the pulmonary tract was obtained by anastomosing the lower edge of the truncal arteriotomy to the upper corner of the ventriculotomy from the truncus in types I and II. Anterior translocation of the pulmonary artery was performed in a type III. A pericardial patch with or without a monocusp was placed to complete the right ventricular outflow tract. Results. There were two hospital deaths, one of which was unrelated to a cardiac problem. Postoperative right-to-left ventricular peak pressure ratio was less than 0.55. There was one left pulmonary stenosis due to monocusp adherence in the late postoperative period. The sizes of the pulmonary tract at anastomosis were between 107% and 166% of the normal value between 7 months and 3.8 years of follow-up. Conclusions. The use of autologous arterial wall instead of a conduit is recommended for the repair of truncus arteriosus to expect growth of the pulmonary tract.

Early progressive pulmonary homograft insufficiency developed in an 11-month-old infant after repair of truncus arteriosus because of dilatation secondary to the presence of residual distal pulmonary artery stenosis and hypoplasia. Before repair, the pulmonary artery branches were discontinuous, with the right pulmonary artery being somewhat hypoplastic and originating from the trunk, and the left pulmonary artery supplied by a modified Blalock-Taussig shunt created in the newborn period. At repair, a pulmonary homograft was used to connect the branches. Progressive cardiomegaly and oxygen dependance occurred 3 weeks postoperatively. Cardiac catheterization showed systemic right ventricular pressure, severe homograft insufficiency, and residual distal pulmonary artery stenosis and hypoplasia. On reoperation at 3 months postoperatively, the homograft annulus diameter increased from 14 mm to 16 mm. Dilatation and insufficiency probably occurred because the right ventricle and homograft distal to the obstruction functioned as a unit during systole. The problem might have been minimized with the use of aortic homograft, which is thicker, or annular reinforcement with a synthetic material.

Bronchovenous fistula is occasionally encountered after traumatic lung injury or, in neonates, due to ventilation injuries with high ventilatory pressures. We report a case of massive air embolism associated with a bronchopulmonary venous communication in an infant post-repair of truncus arteriosus. Selective ventilation of the opposite lung for 3 days sealed the fistula.

Background Truncus arteriosus (TA) with interrupted aortic arch (IAA) is a rare combination of complex anomalies. We report long-term follow-up after one-stage repair of TA associated with IAA. Methods From April 1985 to August 2007, 16 patients with TA associated with IAA underwent one-stage repair. Results There were 2 (12.5%) early deaths and no late deaths. Follow-up was 92.9% complete (13 of 14 patients), with 1 patient lost to follow-up. Median duration of follow-up was 18.2 years (range, 2.1 to 21.9 years). Functional status in all patients was good. Thirteen patients underwent 25 surgical reoperations and 5 interventional procedures (3 aortic arch balloon angioplasties and 2 pulmonary artery balloon angioplasties). Overall freedom from any reoperation was 69.2% at 1 month, 54.5% at 3 years, 30% at 5 years, 11.1% at 10 years, and 0% at 15 years after the initial operation. Freedom from aortic reoperation was 76.9% at 1 month, 72.7% at 3 years, 70% at 5 years, 66.7% at 10 years, and 57.1% at 15 years; while freedom from right ventricular-to-pulmonary artery conduit replacement was 84.6% at 1 month, 63.6% at 3 years, 40% at 5 years, 11.1% at 10 years, and 0% at 15 years. Finally, freedom from truncal valve reoperation was 100% at 5 years, 88.9% at 10 years, and 85.7% at 15 years. Conclusions One-stage repair of TA-IAA can be undertaken with good long-term results. Despite a significant reoperation rate, patients' functional status remains good at the long-term follow-up.

Neonatal surgical repair for truncus arteriosus historically involves removing the pulmonary arteries from the truncal root, closing the ventricular septal defect, and creating right ventricular to pulmonary artery continuity. Unfortunately, early reintervention is frequently required for conduit failure and proximal branch pulmonary artery stenosis. We present a technique that preserves the pulmonary artery architecture, keeping the pulmonary arteries in their native position. This technique has been applied to 16 patients and appears to decrease proximal branch pulmonary artery stenosis, thereby extending conduit longevity and increasing the freedom from early reintervention.

Background Surgical repair of interrupted aortic arch (IAA) remains challenging and is associated with significant mortality and incidence of late arch obstruction, as recently reported by the Congenital Heart Surgeons' Society (CHSS). In particular, the CHSS reported that any technique other than direct anastomosis with patch augmentation is a risk factor for arch reintervention. The experience at Texas Children's Hospital with IAA repair using an aortic arch advancement technique without a patch was examined. Methods Between July 1995 and December 2005, 60 patients underwent IAA repair using aortic arch advancement without a patch. Selective cerebral perfusion was used in 25 patients (42%). Cox proportional hazards models were used to analyze 20 variables to determine risk factors for death, arch reintervention, and left ventricular outflow tract (LVOT) reintervention. Results Median age was 8 days (range, 2 to 271 days) and weight was 3.0 kg (range, 1.7 to 6.1 kg). IAA types were A in 18 (30%) and B in 42 (70%). Associated anomalies were multiple congenital anomalies in 30 (50%) patients, DiGeorge syndrome in 21 (35%), LVOT obstruction in 26 (43%), a single ventricle in 11 (18%), and truncus arteriosus in 6 (10%). Mean follow-up was 3.0 ± 2.6 years. Five-year freedom from aortic arch reintervention was 100%. Survival at 30 days, 1 year, and 5 years was 93%, 78%, and 76%, respectively. Since July 2000, two of 32 patients have died for an overall survival of 94%. Risk factors for death are older age, multiple congenital anomalies, DiGeorge syndrome, and bicuspid aortic valve. Selective cerebral perfusion was an independent protective variable for survival. Survival for an IAA patient with a ventricular septal defect and no complicating cardiac anomalies was 100%. Conclusions Aortic arch advancement without a patch can be applied to IAA patients, with the expectation of a minimal need for arch reintervention. This technique affords an excellent survival, to which selective cerebral perfusion may be a contributing factor.

A technique with autologous tissue for the correction of type III truncus arteriosus is described. The truncal root was excised as a cylinder that incorporated pulmonary arteries and that was translocated anterior to the ascending aorta. The proximal section of the cylinder was closed and the pulmonary tract was reconstructed with anastomosis of a widely opened distal section to the right ventricle. Autologous pericardium was sutured to the entire surface of the pulmonary tract.

Background The excellent results for repair of truncus arteriosus reported in some centers have not applied to patients with associated interrupted aortic arch. This work aims at understanding the discrepancy of results in our own experience. Patients and Methods Ten patients among 83 consecutive children with truncus arteriosus repaired from 1987 to September 2004 who had aortic arch interruption were analyzed, with particular emphasis on clinical presentation and outcome. The comprehensive Aristotle complexity score was calculated for each patient. The Kaplan-Meier method was used to estimate survivals. Results Preoperative mechanical ventilation was necessary in 5 of the 10 patients; 2 of them were moribund. Associated major lesions were as follows: severe (n = 2) and moderate (n = 4) truncal valve regurgitation, coronary artery anomalies (n = 3) and Di-George's syndrome (n = 4). The comprehensive Aristotle score was at least 20 in 6 patients. There were 5 operative deaths (5 of 10); early mortality was 50% (95% confidence limits: 19% to 81%). These deaths occurred in patients with Aristotle score of 20 or greater (5 of 6=83%). All 4 patients who had no moderate or severe truncal valve regurgitation survived the intervention. Survival was a low 37.5% ± 16.1% from 1 year on compared with a high 95.5% ± 2.5% for the 73 patients without aortic arch interruption. Conclusions This study confirms the predictive value of the Aristotle score, hospital mortality being significantly correlated with the highest Aristotle score ( p = 0.024). To improve outcome in these high-risk patients, preoperative management should be optimized, repair should not be delayed, and regurgitant truncal valve should be repaired or replaced.

Background Current techniques for repair of the right ventricular outflow tract (RVOT) may require interposition of a valved conduit between the right ventricle and the pulmonary artery bifurcation. Recently, the Contegra conduit (Medtronic, Inc.) was introduced as an alternative xenograft tissue for RVOT reconstruction. Promising early hemodynamic and clinical results have been reported so far, but still less is known about mid-term adverse outcome. Methods A total of 38 Contegra valved conduits (12 to 22 mm) were implanted from October 1999 to June 2004, in 36 children less than 5 years old and in 2 patients 8 and 21 years old. Diagnosis included the following: tetralogy of Fallot (n = 21); pulmonary atresia (n = 4); double outlet right ventricle + pulmonary stenosis (n = 3); d-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis (n = 3); truncus arteriosus (n = 3); and other complex malformations (n = 4). Results There was no mortality following initial surgery and no valved-conduit–related early morbidity. Early postoperative echocardiographic assessment after 3 months demonstrated favorable hemodynamics in all patients. However, during further follow-up, 5 conduits had to be replaced because of severe stenosis at the level of the distal anastomosis (2 of them had moderate to severe dilatation of the conduit proximally to the valve). Excessive intimal peel formation and severe perigraft scarring reaction were observed in all cases. One child died before surgery. Conclusions The Contegra valved conduit is an interesting concept for reconstruction of the RVOT. However, because of unpredictable incidence of supravalvar stenosis during mid-term results, we cannot recommend routine use of this material.

Background Pulmonary ventricle (PV) to pulmonary artery (PA) conduits have made possible the correction of many complex congenital cardiac anomalies. Methods Between April 1964 and January 2001, 1270 patients underwent operation with conduit placement from the PV to PA. The present study evaluates late outcome of 1095 patients (612 males, 483 females) having an operation before July 1992. Mean age was 9.6 ± 8.2 years old. Diagnoses included pulmonary atresia/tetralogy of Fallot (459), transposition of the great arteries (TGA) (232), truncus arteriosus (193), double outlet right ventricle (DORV) (121), corrected TGA (49), septated univentricular heart (36), and other (5). A porcine-valved Dacron conduit was used in 730, homograft in 239, and non-valved conduit in 126. Results Early mortality decreased from 23.5% prior to 1980 to 3.7% for the most recent decade. Mean follow-up was 10.9 years (maximum, 29 years). Actuarial survival for early survivors at 10 and 20 years was 77.0% ± 1.5% and 59.5% ± 2.6%. On univariate analysis, clinical and hemodynamic factors associated with late mortality were male gender, older age at operation, higher post-repair PV/systemic ventricle (SV) pressure ratio, higher distal PA pressure, and longer bypass time ( p ≤ 0.01 for all). On multivariate analysis, independent risk factors for late mortality were male gender, older age at operation, diagnosis of TGA, corrected TGA, truncus, or univentricular heart, and PV/SV pressure ratio ≥ 0.72 ( p ≤ 0.03 for all). Freedom from reoperation for conduit failure at 10 and 20 years was 55.5% ± 2.0% and 31.9% ± 2.7%. On multivariate analysis, independent risk factors for conduit failure were homograft conduit, diagnosis of TGA, younger age at operation, and smaller conduit size ( p ≤ 0.007 for all). Reoperation for one conduit replacement was performed in 306 patients, two conduit replacements in 55 patients, three in 6 patients, and four in 3 patients. Overall early mortality for conduit replacement in this series was 4.9%; it was 1.7% for patients operated on from 1989 through 1992. At follow-up, 84% of survivors were in NYHA class I or II. Conclusions Operations that include conduit placement and replacement can be performed with low early mortality. Younger age at operation was associated with improved late survival. The diagnosis of TGA was associated with increased risk for conduit failure, and the durability of the homograft, in this series, was inferior to the porcine-valved Dacron conduit. Quality of life was excellent for most patients despite the need for reoperation.

Background . Repair of truncus arteriosus in the neonatal and early infant periods has become standard practice at many centers. We reviewed our recent experience with repair of truncus arteriosus in neonates, with a focus on early and intermediate outcomes. Methods . From July 1992 to December 1999, 65 patients 1 month of age or less underwent primary complete repair of truncus arteriosus. Median age was 10 days, and median weight was 3.2 kg. Major associated anomalies included moderate or severe truncal valve regurgitation in 15 patients (23%), interrupted aortic arch in 8 (12%), coronary artery abnormalities in 12 (18%), and nonconfluence of the pulmonary arteries in 3 (5%). Median durations of cardiopulmonary bypass and cardioplegic arrest were 172 minutes and 90 minutes, respectively. Circulatory arrest was employed only in 7 patients undergoing concomitant repair of interrupted arch. Reconstruction of the right ventricular outflow tract was achieved with an aortic (n = 39) or pulmonary (n = 26) allograft valved conduit (median diameter, 12 mm). Replacement (n = 6) or repair (n = 5) of a regurgitant truncal valve was performed in 11 patients, and interrupted arch was repaired in 8. Results . There were three early deaths (5%). Early reoperations included reexploration for bleeding in 3 patients, emergent replacement of a pulmonary outflow conduit that failed acutely in 1 patient, and placement of a permanent pacemaker in 1. Mechanical circulatory support was required in 1 patient. During the median follow-up of 32 months, there were two deaths. The Kaplan-Meier estimate of survival was 92% at 1 year and beyond. The only demographic, diagnostic, or operative factors significantly associated with poorer survival over time were operative weight of 2.5 kg or less ( p = 0.01) and truncal valve replacement ( p = 0.009). Actuarial freedom from conduit replacement among early survivors was 57% at 3 years. Conclusions . Repair of truncus arteriosus in the neonatal period can be performed routinely with excellent survival, even in patients with major associated abnormalities.

Background . Truncal valve insufficiency has been a significant short- and long-term risk factor for repair of truncus arteriosus. Recent reports have documented the virtues of truncal valve repair. The purpose of this report is to review our experience with truncal valve repair and illustrate our techniques. Methods . Between 1995 and 2000, 8 patients had interventions for severe truncal valve insufficiency at primary repair (3 patients) or in conjunction with conduit replacement (5 patients). One neonate had truncal valve replacement at initial repair early in the experience. The other 7 patients had truncal valve repair, 3 by valvar suture techniques. The remaining 4 patients had leaflet excision and annular remodeling in 3 (coronary reimplantation was required in 2) and commissure resuspension in 1 patient. Results . Trivial to mild truncal valve insufficiency is present in the patients who had leaflet excision and annular remodeling (n = 3) and commissure resuspension (n = 1). Of the 3 patients who had valvar suture truncal valve repair, there was one death and 2 patients required acute valve replacement. The 7 survivors are doing well 1 month to 6 years postoperatively. Conclusions . Truncal valve repair by valvar suture techniques has not been successful in our practice. Truncal valve remodeling by leaflet excision and reduction annuloplasty is an effective method for truncal valve repair. When leaflet excision of a coronary sinus of Valsalva is required, coronary artery translocation can be accomplished.

Background . In the absence of reliable national data, we have collected results of all operations for congenital heart defects from five departments to assess mortality rates and compare them among surgeons and departments. Methods . Data relating to all operations (2,718) carried out at the five centers during a period from April 1, 1997 through March 31, 1999. Clearly defined criteria were agreed for the classification of patients into various subgroups. Results . The overall hospital mortality was 4.4% (95% confidence intervals 3.7%–5.3%). Mortality for open operations was 12.6% in neonates, 5.1% in infants, and 3.5% in children. Mortality rates were 1.1% for coarctation, 0.4% ventricular septal defect, 4.1% atrioventricular septal defect, 2.9% Fallot, 0.9% switch, and 15.6% truncus arteriosus. Although individual surgeons' mortality rates ranged from 1.8% to 7.5%, none of the 12 surgeons' data were above 95% confidence intervals. For individual surgeons, the change in mortality rates between the 2 years ranged between −3.3% and +3.8%. Conclusions . With 2 years' data available, estimates of mortality rates are more precise as reflected by tighter confidence intervals. There were relatively small data sets for individual hospitals and surgeons, which made statistical evaluation difficult. For setting standards, data from more departments for a longer period will be required. Statistical methods alone cannot be used as a sole arbiter of what is considered acceptable performance.

Background . We have used the Medtronic Freestyle bioprosthesis as a right ventricular to pulmonary artery conduit recently in an attempt to overcome some of the problems associated with homografts and stented xenografts. The aim of this study was to review the performance of this prosthesis. Methods . Prospectively collected data for patients having Freestyle bioprostheses implanted as a right ventricular to pulmonary artery conduit were reviewed to assess clinical outcome and echocardiographic results. Results . Thirteen patients aged 13 days to 22.5 years (median, 7.9 years) underwent either primary repair (n = 5) or change of conduit (n = 8) using the Freestyle bioprosthesis. One neonate with truncus arteriosus died postoperatively of pulmonary hypertension. One conduit was explanted 27 months after repair of neonatal truncus arteriosus. There has been no incidence of significant prosthetic regurgitation, thromboembolism, or endocarditis at mean follow-up of 10.1 months (range, 2 weeks to 29 months). Conclusions . The Medtronic Freestyle valve is a reliable pulmonary valve substitute in the short term. Early results justify continued clinical use of the device in this setting with close follow-up.

We report the successful use of nifedipine in the treatment of acute pulmonary hypertension in an infant after a cardiac operation. This patient had undergone total surgical correction of his truncus arteriosus malformation. He had signs of severe pulmonary artery hypertension unresponsive to hyperventilation, oxygenation, sedation, and a myriad of vasodilators. Nifedipine, 0.2 mg/kg every 4 hours, effectively treated his pulmonary artery hypertension and allowed for a smooth post operative course and positive outcome.

We present an intramural left main coronary artery, unexpectedly encountered during aortic root replacement in a truncus arteriosus patient. Given the severely limited orifice, we opted to unroof the intramural portion of the left main coronary artery prior to implantation as a button. Until now there have been no reports in the literature describing unroofing an intramural coronary artery prior to reimplantation in an aortic root replacement operation.

Background . Early complete repair of truncus arteriosus with homograft reconstruction of the right ventricular outflow tract was performed and long-term results were evaluated. Methods . Review of 46 consecutive patients with truncus arteriosus who underwent primary correction between June 1987 and May 1997 was performed. Ages ranged from 21 days to 7.2 years (median, 62 days) and weights from 1.8 to 21.5 kg (median, 3.4 kg). Ten patients were operated on before 1 month of age, 20 between 1 and 3 months, 13 between 3 months and 1 year, and 3 at an older age. Associated cardiac conditions were encountered frequently, the most common being coronary artery anomalies (n = 16), truncal valve incompetence (n = 12), and interrupted aortic arch (n = 5). The right ventricular outflow tract was reconstructed with an aortic (n = 28) or a pulmonary homograft (n = 18). Results . There were two hospital deaths (4.3%). Both patients had severe truncal valve regurgitation and interrupted aortic arch together with other unfavorable conditions. Survival in uncomplicated truncus arteriosus was 100%. Follow-up was from 3 months to 10 years (mean, 36 months). There was one late death 4 months after the initial repair, presumably because of cardiac reason. Actuarial survival was 93% at 4 months and beyond. Actuarial freedom of reoperation in the 27 hospital survivors with aortic homografts was 43% at 75 months; it was 73% at 62 months in the 17 patients surviving with pulmonary homografts. Conclusions . Neonatal or early infancy complete repair is the treatment with the best potential for survival. The homograft remains our conduit of choice to establish continuity between the right ventricle and the pulmonary artery. Management of severe truncal valve incompetence remains a surgical challenge.

An infant with truncus arteriosus, complete atrioventricular canal, and total anomalous pulmonary venous connection successfully underwent one-stage complete repair. Residual mitral valve regurgitation required reoperation after 12 days. The patient is doing well at 6 months' follow-up. Echocardiography demonstrates no residual defects, competent atrioventricular valves, and normal pulmonary pressure. This case illustrates the potential for successful one-stage repair even of associated complex heart defects involving venous, intracardiac, and arterial pathways.

Between January 1987 and July 1992, 641 infants (less than 1 year of age) underwent cardiac surgical procedures through a median sternotomy incision at the UCLA Medical Center. In 36 (5.6%), to achieve cardiac decompression, the chest was left open after the operation, or was re-opened immediately postoperatively because of low cardiac output. The incidence of cardiac decompression was 31% ( 4 / 13 ) after the Norwood procedure and 24% ( 7 / 29 ) after truncus arteriosus repair. Opening of the chest reduced intrathoracic pressure and allowed complete expansion of the lungs. Delayed sternal closure was carried out in 27 patients at a mean of 5 days (range, 2 to 14 days) postoperatively. By the time of chest closure, left atrial pressure had decreased from a mean of 12 ± 1.4 to 8.4 ± 0.8 mm Hg ( p < 0.004), and inotropic drug support with dopamine and dobutamine had also decreased significantly. Thirteen (36%) patients died of low cardiac output and multiorgan failure (4 of them after delayed chest closure) that was complicated by sepsis in 2. The incidence of sternal wound infection was relatively low at 5.6% ( 2 / 36 ); 1 patient died of generalized sepsis complicating multiorgan failure and the second case occurred in a patient who survived long term after sternectomy. With optimal ventilatory and inotropic drug support and meticulous wound care, delayed sternal closure may improve the survival of infants in low cardiac output after cardiac surgical procedures.

Small cryopreserved allografts for use in neonates and infants are increasingly difficult to find. We describe a technique for surgically reducing the size of the more readily available large-diameter allografts to make them conform to size requirements appropriate for neonates and infants. This technique involves a longitudinal incision of the allograft from its muscular annulus to its distal orifice with the excision of a single valve leaflet. The two commissural posts of the excised leaflet and the wall of the allograft are then reapproximated with fine suture. Four patients ranging in age from 14 days to 11 months (mean age, 5.2 months) and ranging in weight from 3 to 8.4 kg (mean weight, 5.2 kg) underwent reconstruction of the right ventricular outflow tract using this surgical technique. Two patients underwent repair of truncus arteriosus (age, 14 days and 16 days) and two patients underwent Rastelli operations (age, 9 months and 11 months). The mean follow-up time was 22.5 months (range, 14 to 28 months). All 4 patients are alive and well. One patient requires digoxin and furosemide for moderate quadricuspid truncal valve insufficiency. Serial echocardiography documents mild allograft stenosis in 2 patients and trivial to mild allograft insufficiency in 2 patients. Because handling characteristics and lower bleeding risks render allograft conduits preferable to synthetic conduits and, furthermore, because conduit insertion of any type in the neonate will eventually be outgrown and require replacement, we find this technique to be justifiable as a therapeutic option. In the short term, the technique results in excellent functional results and provides an alternative to synthetic conduits when an appropriate sized allograft is unavailable.

Neonatal repair of truncus arteriosus is being performed in a number of centers, often with the use of small homograft conduits. The fate of the homograft and the risk of replacement were the subjects of this study. Between January 1987 and October 1991, 43 infants aged less than 3 months (range, 3 to 90 days) underwent primary repair of truncus arteriosus including implantation of a valved homograft conduit (diameter, 7 to 12 mm). Twenty-nine had follow-up of more than 6 months (range, 6 to 65 months; mean, 21.9 months). After a mean period of 31 months (range, 8 to 65 months), 7 patients showed obstruction with right ventricular pressures at least 75% systemic and underwent either a conduit change (n = 5) or a patch augmentation (n = 2). Mean cardiopulmonary bypass time at reoperation was 99 minutes; mortality was zero. Five other children are known to have a right ventricular pressure of 50% to 60% systemic, 2 having undergone balloon dilation. Statistical comparison of the patients with conduit reoperation or high right ventricular pressure (n = 12) with the rest of the population (n = 17) revealed an elevated pulmonary artery to right ventricular pullback gradient on postoperative day 1 after the repair (7.7 versus 1.3 mm Hg; p = 0.001) and choice of an aortic over a pulmonary homograft (100% versus 64.7%; p = 0.065) as significant risk factors. Age and weight at repair, postoperative pulmonary artery pressure, length of follow-up, and size of the homograft showed no significant differences between the two groups. A postoperative pulmonary artery to right ventricular pressure gradient is a risk factor for conduit replacement within 2 years, possibly reflecting subtle technical factors or small branch pulmonary arteries. Very small homografts (7 to 9 mm in diameter) are not, but, most interestingly, aortic homografts are a significant risk factor for early replacement. The use of small pulmonary homografts should be encouraged.

Although the clinical use of the subcoronary free-sewn allograft aortic valve was first described by Ross and Barratt-Boyes independently in 1962, the use of an allograft valved conduit for right ventricular outflow tract reconstruction in congenital cardiac surgery was not considered until February 14, 1966. With the description of this successful application by Ross and Somerville, the corrections of many previously inoperable cardiac lesions soon followed.

Evaluation was made of 17 patients who underwent conduit reconstruction of the right ventricular outflow tract (for anomalies other than truncus arteriosus) at the Texas Heart Institute between December, 1965, and June, 1974. Fifteen patients survived the operation and have shown substantial clinical improvement. Several different conduits were used, principally an allograft aorta with the aortic valve and mitral leaflet attached, a woven Dacron prosthesis containing a xenograft (porcine) valve, and a valveless Dacron tube graft. Allograft conduits are sometimes difficult to procure and keep and tend to calcify with passage of time. Woven Dacron prostheses are favored because they are readily available in a wide range of sizes. We believe it is not necessary for the conduit to contain a valve unless the patient has pulmonary hypertension, in which case we use a xenograft (porcine) valve because this valve does not require the long-term use of anticoagulants, a difficult regimen to manage in children. The largest possible prosthesis must be used; otherwise right ventricular hypertension will persist. Indications for conduit reconstruction include anomalous coronary arteries crossing the right ventricular outflow tract, discontinuity of the pulmonary arteries, and pulmonary atresia with a ventricular septal defect. Our current method of managing pulmonary atresia with ventricular septal defect (pseudotruncus arteriosus) includes palliative shunting to relieve hypoxemia during infancy and to permit full development of the pulmonary arteries for eventual total correction at a more optimal age when a larger conduit may be used.

The use of aortic allografts in the repair of congenital cardiac lesions has increased as a result of both the advent of cryopreservation and the effects of increased donor availability secondary to infant transplantation. During the period 1986 through 1987, 38 cryopreserved aortic allografts were placed for right ventricle-pulmonary artery discontinuity. Size of the allografts ranged from 11 to 26 mm (mean size, 19 mm), and age of the patients ranged from 6 weeks to 26 years (mean age, 5 years). Twenty-one patients had primary placement of aortic allografts, and 17 underwent replacement of previous conduits. There were 5 hospital deaths (13%) overall, only 1 among the 10 patients younger than 6 months of age with truncus arteriosus, and none among the 17 patients having conduit replacement. A large conduit could be placed with a low incidence (10.5%) of postoperative hemorrhage related to the conduit. The aortic allograft is our conduit of choice for both conduit replacement and primary repair of right ventricle-pulmonary artery discontinuity.

An 18-year-old patient is reported who had simultaneous occurrence of aortopulmonary septal absence, complete interruption of the aortic arch, and patent ductus arteriosus. This is the only known patient who has had total anatomical correction of this combination of defects. Correction was accomplished by staged procedures; the first stage consisted of obliteration of the patent ductus and establishment of aortic continuity; this was followed one year later by the second stage, which included a unique method for repair of absence of the aortopulmonary septum. The surgical desirability and embryological implications of criteria for differentiating truncus arteriosus from aortopulmonary septal defect are presented. Follow-up physiological evaluations and the patient's death from the effects of increasing pulmonary vascular disease (despite early symptomatic improvement) approximately two years after completion of the repair underscore the importance of early diagnosis and operative intervention.

Infants with truncus arteriosus present a difficult management issue. Because of the high operative mortality, repair is often delayed beyond the first 3 to 6 months of age. We reviewed our experience with 11 neonates and young infants with truncus arteriosus undergoing repair (median age, 21 days). Five patients also had major truncal valve insufficiency, and 2 required valve replacement. Right ventricle-pulmonary artery continuity was established with a porcine valved conduit in 3 patients and an aortic or pulmonary homograft in 8. There was 1 operative death (9%; 70% confidence limits, 3%-22%) and 1 late death over a mean follow-up of 21 months (range, 4 to 32 months). Eight of the 9 late survivors are growing normally. Echocardiographic examination revealed normal ventricular function in all patients (mean shortening fraction, 39%). Doppler assessment demonstrated trivial prosthetic or homograft valve regurgitation in 7 patients and mild to moderate obstruction in 5 patients. This recent experience with repair of truncus arteriosus indicates that the operative risk is low even in the neonate. Repair in the first month of life should be recommended before the development of critical congestive heart failure or irreversible pulmonary vascular disease.

A method for surgically limiting pulmonary blood flow in the critically ill neonate with truncus arteriosus is described. Two recent cases utilizing this technique are presented. Comparisons are made between this and other palliative surgical procedures used in truncus arteriosus.

Correction of truncus arteriosus can be performed in the older child with low mortality. However, many infants are severely symptomatic with increased pulmonary blood flow and require banding of the pulmonary artery at an age when correction has a high mortality. The pulmonary artery band can complicate the usual technique of the subsequent repair. An alternative approach following pulmonary artery banding is to interrupt the pulmonary artery–truncus continuity with a patch from within the truncus and then place the external valved conduit onto the pulmonary artery in an end-to-side manner.

A surgical procedure for palliation of infants with truncus arteriosus and excessive pulmonary blood flow (types I, II, and III) is described. The technique involves the production of ostial stenosis of the pulmonary artery from within the truncus using cardiopulmonary bypass. This method has advantages over pulmonary artery banding, which in general has produced disappointing results.

A new modified procedure for correction of types II and III truncus arteriosus defects is described. This method of intraluminal aortic patch placement to partition the pulmonary and systemic circulation has certain distinct technical and anatomical advantages in patients with this type of congenital defect. Further, as the patient grows, it may prove to be a more versatile procedure.

The association of truncus arteriosus with interrupted aortic arch represents a formidable surgical challenge. Two successful repairs have been reported, but none for the past ten years. This report presents a third successful repair using a technique that allows the widely patent ductus arteriosus to maintain continuity between the truncus (with pulmonary arteries detached) and the descending aorta. Right ventricle-pulmonary artery continuity is established in the usual way with a porcine-valved conduit. While long-term potential difficulties with this approach are recognized, it appears to give satisfactory initial palliation and to be an acceptable method of treatment for this combination of defects. The embryology and the anatomy of the lesion are briefly discussed.