Short-term outcomes in premature neonates adhering to the philosophy of supportive care allowing for weight gain and organ maturation prior to cardiac surgery.

BACKGROUND: Prematurity is a recognized risk factor for morbidity and mortality following cardiac surgery. The purpose of this study was to examine short-term outcomes following cardiac surgery in premature neonates adhering to our institutional philosophy of supportive care allowing for weight gain and organ maturation. METHODS: Retrospective review of all neonates undergoing cardiac surgery from January 2002 to May 2008. A total of 810 neonates (<30 days of age) were identified. Prematurity defined as less than 36 weeks of gestation. Neonates undergoing ductus arteriosus ligation alone were excluded. In all, 63 neonates comprised the premature group. Term group comprised 244 randomly selected term neonates in a 1:4 ratio. Outcome variables were compared between the 2 groups. RESULTS: Median gestation 34 weeks, range 24 to 35 weeks. Defects: 2 ventricle, normal arch (41% premature vs 44% term; P = .7), 2 ventricle, abnormal arch (24% vs 22%; P = .8), single ventricle, normal arch (21% vs 15%; P = .2), single ventricle, abnormal arch (14% vs 19%; P = .4). Premature neonates were older and smaller at surgery. Cardiopulmonary bypass procedures were performed less frequently in premature neonates (49% vs 69%; P = .004). Length of mechanical ventilation at our institution (6 days [0.5-54) vs 4 days [0.5-49); P = .06); postoperative hospital stay at our institution (17 days [1-161) vs 15 days [0-153); P = .06); and mortality (16% vs 11%; P = .2) was not different between the 2 groups. CONCLUSION: Early outcome seems independent of weight, prematurity, cardiopulmonary bypass, and type of first intervention. Importantly, there was no statistical difference in mortality between the 2 groups, regardless of how they were treated. Further long-term follow-up is needed in this patient population.

Neurodevelopmental outcomes in children with Down syndrome and congenital heart defects.

Trisomy 21, the chromosomal condition responsible for Down syndrome (DS, OMIM #190685), is the most common identifiable genetic cause of intellectual disability. Approximately half of all children with DS are born with a significant congenital heart defect (CHD), the most common of which is an atrioventricular septal defect (AVSD). As children with comorbid DS and CHD increasingly survive cardiac surgery, characterization of their early developmental trajectories is critical for designing early interventions to maximize individual potential. Herein, the developmental domains (cognitive, language, and motor) of children with DS and AVSD (DS + AVSD, n = 12) were compared to children with DS and a structurally normal heart (DS - CHD, n = 17) using the Bayley Scales of Infant and Toddler Development III. The DS + AVSD cohort mean age was relatively the same as controls with DS - CHD, 14.5 ± 7.3 months compared with 14.1 ± 8.4 months, respectively. Although the motor domain was the only domain that showed a statistically significant difference between groups (P < 0.05), both cognitive standard scores (P = 0.63) and language composite standard scores (P = 0.10) were lower in the DS + AVSD cases compared with the DS - CHD controls although it is not statistically significant. Since this is the first study to examine the early developmental outcomes of children with DS + AVSD, the findings may be useful for clinicians in providing anticipatory guidance.

Red blood cell transfusion for infants with single-ventricle physiology.

The purpose of this study was to assess how red blood cell (RBC) transfusions impact hemodynamic parameters in infants with single-ventricle lesions. This was a retrospective chart review. The setting was a pediatric cardiac intensive care unit at a tertiary care children's hospital. Fifty-nine patients <1 year of age with single-ventricle physiology who received a blood transfusion between December 2007 and April 2009 were analyzed. They received a total of 183 transfusions. Exclusion criteria included transfusions given within 72 h of cardiac surgery or transfusions given to patients with active bleeding. There were no interventions. The study population was divided into terciles based on pretransfusion hemoglobin (Hgb) concentration. The pretransfusion Hgb concentration in group A was 7.8 to 12.3 gm/dl, in group B was 12.4 to 13.2 gm/dl, and in group C was 13.3 to 15.7 gm/dl. Heart rate, blood pressure, arterial saturation, and cerebral near-infrared spectroscopy (cNIRS) values before transfusion, as well as at 1, 2, 4, 8, and 12 h after transfusion, were collected. There was significant improvement in diastolic blood pressure, arterial saturation, and cNIRS in group A after 12 h. Transfusions given in group B also resulted in improvement in diastolic blood pressure and arterial saturation, with less robust response of cNIRS. In group C, only arterial saturation values increased significantly. RBC transfusions can improve hemodynamics and markers of oxygen delivery in infants with single-ventricle physiology, but further studies are needed to determine an optimal Hgb level in this population. Interventions to increase Hgb above this level may be of limited benefit.

Negative pressure ventilation as a therapy for post-operative complications in a patient with single ventricle physiology.

OBJECTIVE: To report the use of negative pressure ventilation as a therapeutic rescue in a patient with post-operative complications of Kawashima procedure. DESIGN: Case report. SETTING: Paediatric cardiac critical care unit in a tertiary care teaching hospital. PATIENT: Nine month-old patient with single ventricle physiology developed complications after Kawashima procedure that were worsened by use of positive pressure ventilation. INTERVENTION: Utilisation of negative pressure ventilation due to its unique cardiopulmonary interactions and haemodynamic effects. MEASUREMENTS AND MAIN RESULTS: There was gradual and persistent improvement in the passive diastolic pulmonary blood flow through the Kawashima circuit along with improvement in oxygenation and ventilation. The patient was successfully weaned off ventilator support and discharged to home. CONCLUSION: Patients with single ventricle physiology depend on passive diastolic blood flow to the lungs through surgical pathways such as Fontan or Kawashima circuits. Prolonged use of positive pressure ventilation can significantly affect this forward flow and result in haemodynamic compromise. Use of negative pressure ventilation with its unique cardiopulmonary interactions can be an effective rescue as demonstrated in our case.

Force frequency relationship of the human ventricle increases during early postnatal development.

Understanding developmental changes in contractility is critical to improving therapies for young cardiac patients. Isometric developed force was measured in human ventricular muscle strips from two age groups: newborns (<2 wk) and infants (3-14 mo) undergoing repair for congenital heart defects. Muscle strips were paced at several cycle lengths (CLs) to determine the force frequency response (FFR). Changes in Na/Ca exchanger (NCX), sarcoplasmic reticulum Ca-ATPase (SERCA), and phospholamban (PLB) were characterized. At CL 2000 ms, developed force was similar in the two groups. Decreasing CL increased developed force in the infant group to 131 +/- 8% (CL 1000 ms) and 157 +/- 18% (CL 500 ms) demonstrating a positive FFR. The FFR in the newborn group was flat. NCX mRNA and protein levels were significantly larger in the newborn than infant group whereas SERCA levels were unchanged. PLB mRNA levels and PLB/SERCA ratio increased with age. Immunostaining for NCX in isolated newborn cells showed peripheral staining. In infant cells, NCX was also found in T-tubules. SERCA staining was regular and striated in both groups. This study shows for the first time that the newborn human ventricle has a flat FFR, which increases with age and may be caused by developmental changes in calcium handling.

Near infrared spectroscopy changes with pericardial tamponade.

OBJECTIVE: Report on the use of near infrared spectroscopy (NIRS) for the recognition of pericardial tamponade after neonatal congenital heart surgery. DESIGN: Case report. SETTING: Pediatric cardiac intensive care unit. PATIENT: Seven-day-old term infant with double inlet left ventricle, hypoplastic aortic arch, and ascending aorta, postoperative day (POD) 3 from the Norwood procedure. INTERVENTION: After an initial stable early postoperative course, the patient's cerebral saturation decreased from the 50s on POD 2, to the 40s in the early morning hours of POD 3. By 9 am on POD 3, the cerebral saturation decreased further to the mid 30s. No change in pulsoximetry, arterial blood gas acid base balance, or blood pressure occurred. An echocardiogram was performed due to the progressive decline in cerebral saturation values. A 10-mm circumferential pericardial effusion was diagnosed. The effusion was drained without incident. Cerebral saturation returned to the 50s, the patient had no further complications and was discharged to home on POD 15. CONCLUSION: Pericardial tamponade is a well-known complication of open heart surgery in children, and early recognition of this can be difficult. This case report demonstrated cerebral saturation to be decreased as impending pericardial tamponade developed. Along with the commonly used markers of tamponade, near infrared spectroscopy measurement of cerebral saturation may also be of benefit in recognizing this life-threatening condition.

A Novel Case of Marfan Syndrome in an Infant With Hypoplastic Left Heart Syndrome.

This report describes a case of hypoplastic left heart syndrome (HLHS) and Marfan syndrome presenting in conjunction, and highlights how a connective tissue disorder may alter medical and surgical management of newborns with HLHS.

Self-reported functional health status following interrupted aortic arch repair: A Congenital Heart Surgeons' Society Study.

OBJECTIVES: Improved survival after congenital heart surgery has led to interest in functional health status. We sought to identify factors associated with self-reported functional health status in adolescents and young adults with repaired interrupted aortic arch. METHODS: Follow-up of survivors (aged 13-24 years) from a 1987 to 1997 inception cohort of neonates included completion of functional health status questionnaires (Child Health Questionnaire-CF87 [age <18 years, n = 51] or the Short Form [SF]-36 [age ≥18 years, n = 66]) and another about 22q11 deletion syndrome (22q11DS) features (n = 141). Factors associated with functional health status domains were determined using multivariable linear regression analysis. RESULTS: Domain scores of respondents were significantly greater than norms in 2 of 9 Child Health Questionnaire-CF87 and 4 of 10 SF-36 domains and only lower in the physical functioning domain of the SF-36. Factors most commonly associated with lower scores included those suggestive of 22q11DS (low calcium levels, recurrent childhood infections, genetic testing/diagnosis, abnormal facial features, hearing deficits), the presence of self-reported behavioral and mental health problems, and a greater number of procedures. Factors explained between 10% and 70% of domain score variability (R2 = 0.10-0.70, adj-R2 = 0.09-0.66). Of note, morphology and repair type had a minor contribution. CONCLUSIONS: Morbidities associated with 22q11DS, psychosocial issues, and recurrent medical issues affect functional health status more than initial morphology and repair in this population. Nonetheless, these patients largely perceive themselves as better than their peers. This demonstrates the chronic nature of interrupted aortic arch and suggests the need for strategies to decrease reinterventions and for evaluation of mental health and genetic issues to manage associated deteriorations.

Successful Salvage of an Extracardiac Fontan in the Setting of Purulent Mediastinitis using Antibiotic-Impregnated Beads.

Post-cardiotomy mediastinitis is an especially serious complication after the implantation of prosthetic vascular grafts. Standard of care is irrigation, debridement, and removal of all prosthetic material present in the surgical field. The use of antibiotic impregnated beads at the site of infection has been reported in the salvage of vascular grafts in the adult population. We present the case of a 3-year-old child with hypoplastic left heart syndrome who developed mediastinitis following the Fontan operation. In a nontraditional approach, the Fontan conduit, which was surrounded by gross purulence, was successfully salvaged with the adjunctive use of vancomycin-impregnated beads.

Long-term noninvasive arrhythmia assessment after total anomalous pulmonary venous connection repair.

BACKGROUND: Pediatric patients with a history of atrial surgery are at risk for the development of sinus node dysfunction and atrial arrhythmias. However, there has been no comprehensive, long-term, electrophysiologic study of patients who have undergone repair of total anomalous pulmonary venous connection. METHODS: We evaluated school-aged and adolescent survivors of isolated total anomalous pulmonary venous connection repair from January 1983 to December 1996 to assess for sinus node dysfunction, atrioventricular block, and atrial and ventricular arrhythmias. Assessment was limited to an electrocardiogram, 24-hour Holter monitor, and exercise stress test. RESULTS: Twenty-nine children were evaluated 11.2 +/- 3.6 years after their initial operative repair. The mean age at repair was 36.0 +/- 43.0 days. Electrophysiologic studies revealed evidence of sinus node dysfunction, including sinus bradycardia, sinus pauses, and chronotropic impairment, in most of the patients. Twenty-nine percent of patients showed chronotropic impairment on exercise testing. Atrioventricular conduction abnormalities occurred in 2 patients. Single atrial and ventricular premature complexes were frequent, but complex tachyarrhythmias were less common. There was 1 patient who had nonsustained supraventricular tachycardia and 2 patients who had nonsustained ventricular tachycardia. No statistically significant relationships were found between hypothesized variables and arrhythmia outcomes. CONCLUSIONS: Survivors of total anomalous pulmonary venous connection repair appear to have a high incidence of signs of sinus node dysfunction and a low incidence of atrioventricular block in follow-up. Significant atrial and ventricular arrhythmias appear to be uncommon. Despite a favorable overall long-term outcome, these patients warrant ongoing clinical follow-up for arrhythmia surveillance.

Quality of Life for Historic Cavopulmonary Shunt Survivors.

BACKGROUND: Beginning with Dr William Glenn in 1958, 90 patients with congenital heart lesions underwent cavopulmonary (Glenn) shunts over a 30-year period. In 2015, the follow-up data on this original cohort were reported. The study focuses on the current quality of life of this cohort. METHODS: Of the original 91 cavopulmonary shunt survivors, 14 (ages: 26-59; average length of postsurgical follow-up: 38.4 years) completed the Rand 36-Item Short-Form Health Survey, volume 2 (SF-36v2) quality-of-life survey at clinical office visits, over the phone or via regular mail. Diagnoses included tricuspid atresia (n = 9), double inlet left ventricle (n = 1), d-transposition of great arteries (n = 1), Ebstein's anomaly (n = 1), tetralogy of Fallot (n = 1), and pulmonary atresia with hypoplastic right ventricle (n = 1). Norm-based comparison of cavopulmonary shunt survivors to the general population was performed using a one-sample t test. RESULTS: The 36-question health survey, SF-36v2, provided physical and mental health summary measures standardized to achieve a mean of 50. The 14 survivors' physical composite score was 47.18 (standard deviation [SD]: 8.24; P = .22) and mental composite score was 52.71 (SD: 5.64; P = .095). CONCLUSION: The cavopulmonary shunt is now a widely used surgical palliation for single-ventricle patients and can be associated with physical and mental health outcomes similar to the general US population. Cavopulmonary shunt survivors' SF-36v2 survey results do not demonstrate a statistically significant difference from the general US population in physical or mental health measures. The results of this study contribute to the discussion of quality of life for patients with congenital heart disease by following up with the oldest known survivors.

Half a Century's Experience With the Superior Cavopulmonary (Classic Glenn) Shunt.

BACKGROUND: Five decades after its introduction, the Glenn shunt remains an integral step for patients undergoing single-ventricle palliation. We performed a longitudinal follow-up of the original cohort of patients who underwent Glenn shunt. METHODS: We performed a retrospective study of the original cohort of patients who underwent Glenn shunt at Yale between 1958 and 1988. Electronic medical records and chart review up to current era were used to collect data. RESULTS: Ninety-one patients underwent a Glenn shunt at an average age of 6.6 ± 2.5 years, of which 89 were classic Glenn shunts. Median overall survival was 43 years (range, 2 to 56; 95% confidence interval [CI], 39.5 to 46.5) while median survival from the Glenn shunt was 31.4 years (range, 0 to 45; 95% CI, 23.9 to 38.9). Forty-six patients died, 7 in the early postoperative period and 39 late deaths. Twenty-six patients were lost to follow-up. Nineteen patients remain alive with active clinical follow-up, 6 of whom still live with their classic Glenn shunt without conversion to bidirectional Glenn. Twenty-six patients (31%) developed pulmonary arteriovenous fistula with 11 patients (42%) requiring coil embolization. No patient developed thrombosis of the Glenn shunt. There were a total of 28 patients who developed arrhythmias, mostly in the tricuspid atresia group (n = 16), with the majority being atrial tachyarrhythmias (48%). Sixteen patients required permanent pacemaker placement for sinus node dysfunction. CONCLUSIONS: The Glenn shunt continues to provide excellent staged palliation in single-ventricle patients and a bridge to two-ventricle repair. Arrhythmias and pulmonary arteriovenous fistulas were common among single-ventricle cohort. Quality-of-life evaluation of the surviving patients would be an important outcome measure for future investigation.

Early Extubation After Repair of Tetralogy of Fallot and the Fontan Procedure: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.

BACKGROUND: There is increasing interest in reducing the duration of mechanical ventilation after pediatric cardiac operations. However, the contemporary use of an early extubation strategy and its effect on clinical outcomes is poorly understood. METHODS: Data from The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010 to 2013) were analyzed to determine the association of early extubation with postoperative length of stay (PLOS). Two operations were analyzed: complete repair of tetralogy of Fallot (TOF) in patients older than 45 days and the Fontan procedure. Centers were stratified into tertiles by frequency of early extubation, defined as less than 6 hours after leaving the operating room. Patients extubated after 48 hours were excluded. RESULTS: Among 92 centers, early extubation was performed in 31.5% (478 of 1,519) of children undergoing TOF repair and in 69.8% (1,153 of 1,653) of those undergoing the Fontan procedure. Early extubation after TOF repair was associated with heavier weight at operation (p < 0.001) and fewer preoperative risk factors (p = 0.016). After adjustment for covariates, average PLOS after TOF repair was shorter for centers in the highest tertile of early extubation rate than for the lowest tertile centers, which have low early extubation rate (p = 0.04). No association was found between center early extubation rate and PLOS for Fontan procedures (p = 0.08). CONCLUSIONS: Early extubation is common after repair of TOF and the Fontan procedure. A high institutional rate of early extubation after TOF repair is associated with shorter PLOS. Further analysis is needed to understand what effect early extubation might have on other meaningful measures such as resource use.

Cost-utility analysis of salvage cardiac extracorporeal membrane oxygenation in children.

BACKGROUND: Indications for extracorporeal membrane oxygenation therapy have expanded to include cardiopulmonary arrest and support after congenital heart surgery. Data from a national registry have reported that cardiac patients have the poorest survival of all extracorporeal membrane oxygenation recipients. Concerns have been raised about the appropriateness of such an aggressive strategy, especially in light of the high costs and potential for long-term neurologic disability. We reviewed our experience with salvage cardiac extracorporeal membrane oxygenation to determine the cost-utility, which accounts for both costs and quality of life. METHODS: Medical records of patients with congenital heart disease receiving salvage cardiac extracorporeal membrane oxygenation between January 2000 and May 2004 were reviewed. Charges for all medical care after the institution of extracorporeal membrane oxygenation were determined and converted to costs by published standards. The quality-of-life status of survivors was determined with the Health Utilities Index Mark II. RESULTS: Salvage cardiac extracorporeal membrane oxygenation was instituted in 32 patients (18 for cardiopulmonary arrest and 14 for cardiac failure after heart surgery) at a median age of 2.0 months (range, 4 days to 5.1 years). Congenital heart disease was present in 27 (84%). The mean duration of extracorporeal membrane oxygenation support was 5.1 +/- 4.1 days. Survival to hospital discharge was 50%, including 1 patient bridged to heart transplantation. Survival to 1 year was 47%. The mean score of the Health Utilities Index for the survivors was 0.75 +/- 0.19 (range, 0.41-1.0). The median cost for hospital stay after the institution of extracorporeal membrane oxygenation was USD 156,324 per patient. The calculated cost-utility for salvage extracorporeal membrane oxygenation in this population was USD 24,386 per quality-adjusted life-year saved, which would be considered within the range of accepted cost-efficacy (< USD 50,000 per quality-adjusted life-year saved). CONCLUSIONS: Salvage cardiac extracorporeal membrane oxygenation results in reasonable survival and is justified on a cost-utility basis.

Late neurodevelopmental outcome after repair of total anomalous pulmonary venous connection.

Objective We sought to define the neurodevelopmental status of school-aged survivors of total anomalous pulmonary venous connection repaired during infancy. Methods All school-aged survivors of total anomalous pulmonary venous connection repair performed at a single institution were eligible. Thirty children returned for neurologic examination and neurodevelopmental testing. Results The median age at total anomalous pulmonary venous connection repair was 16 days (range, 1-141 days), and age at testing was 11 years (range, 6-19 years). Pulmonary venous return was supracardiac in 14 patients, infracardiac in 12 patients, cardiac in 3 patients, and mixed in 1 patient. Preoperative obstructed total anomalous pulmonary venous connection was present in 6 patients. Circulatory arrest was used in all repairs, with a median duration of 35 minutes (range, 17-55 minutes). At follow-up, microcephaly (head circumference <5%) was present in 28%. Neuromuscular examination was suspect or abnormal in 27%. Mean Full-scale IQ (95.3 +/- 18.5) and Verbal IQ (98.6 +/- 20.2) were not different from population norms, but Performance IQ (92.3 +/- 16.9) was significantly lower than population norms ( P = .02). Fine motor skills and visual-motor coordination were significantly impaired ( P < .01 for Grooved Pegboard and Test of Visual-Motor Integration). Patients with total anomalous pulmonary venous connection also had difficulty with tests of attention (Test of Everyday Attention for Children, P < .01), but results of tests of memory function were not significantly different from population norms. Conclusions School-aged survivors of infant total anomalous pulmonary venous connection repair exhibit a significant incidence of neurodevelopmental difficulties. Fine motor function, visual-motor integration, and attention are the most commonly affected domains. Evaluation of these children is indicated to identify those who are at risk for learning disabilities and who could benefit from early intervention.

Nesiritide in infants and children with congestive heart failure.

OBJECTIVES: Nesiritide (synthetic B-type natriuretic peptide) has been shown to be effective in the management of acute decompensated heart failure in adults. The role of nesiritide in pediatric heart failure has not been examined. In the present study, we reviewed our initial experience with nesiritide in children with primary heart failure or low cardiac output after heart surgery. METHODS: Nesiritide was administered in an open-label fashion to patients with heart failure who were already receiving inotropic and diuretic therapy. Between July 2003 and August 2004, 30 patients aged 5 days to 16.7 yrs (median age, 4.6 months) received nesiritide therapy. Diagnoses included single-ventricle congenital defect (n = 5), two-ventricle congenital defect (n = 13), heart transplant (n = 5), and dilated cardiomyopathy (n = 7). Sixteen patients were started on nesiritide within 2 wks of corrective or palliative heart surgery. The majority of subjects (n = 24) received an initial bolus dose. Continuous infusion dosage ranged between 0.005 and 0.02 microg.kg.min. Nesiritide was discontinued for possible side effects in two patients (arrhythmia and hypotension). Duration of therapy ranged from 1 to 24 days (median, 4 days). RESULTS: Administration of nesiritide was associated with improvement in fluid balance from positive 0.8 +/- 1.9 mL.kg.hr at baseline to negative 0.3 +/- 1.8 mL.kg.hr after 24 hrs of therapy (p = .02). There was a nonsignificant trend toward a reduction in right atrial pressure (9.2 +/- 3.9 vs. 11.2 +/- 4.1, p = .08). CONCLUSIONS: Nesiritide is well tolerated in children with heart failure and is associated with improved diuresis. Further prospective studies will be needed to compare nesiritide with other vasoactive agents and examine the cost-efficacy of this therapy.

Anomalous Origins of Coronary Arteries From the Pulmonary Artery: A Comprehensive Review of Literature and Surgical Options.

Anomalous origins of coronary arteries from the pulmonary artery are rare malformations in which the coronary arteries originate from pulmonary artery sinuses or branches. The consequences are variable although, in most cases, these anomalies lead to severe coronary hypoperfusion and ventricular dysfunction. Surgical correction is indicated once the diagnosis is established due to high early mortality associated with the disease. In nearly all cases, the anomalous artery can be excised from its pulmonary origin, mobilized, and reimplanted directly into the ascending aorta using different surgical techniques. In rare circumstances, technical modifications must be used to restore a normal dual coronary perfusion. The emphasis of this article is to provide a collective review of surgical options published in the literature.

Persistent Pulmonary Hypertension in a Neonate With Transposition of Great Arteries and Intact Ventricular Septum: A Case Report and Review of the Literature.

Transposition of the great arteries (TGA) with intact ventricular septum (IVS) has very favorable short- and long-term surgical outcome. Although rare, when associated with persistent pulmonary hypertension (PPH), it exhibits significant mortality risk and management challenges. We report the case of a neonate with TGA with IVS and PPH who underwent successful early surgical repair with emphasis on clinical management and review of the literature.

Surgical management of competing pulmonary blood flow affects survival before Fontan/Kreutzer completion in patients with tricuspid atresia type I.

OBJECTIVES: To determine the association between surgical management of pulmonary blood flow (PBF) at initial and staged procedures with survival to Fontan/Kreutzer operation (Fontan) in patients with tricuspid atresia. METHODS: Infants aged <3 months with tricuspid atresia type I (n = 303) were enrolled from 34 institutions (1999-2013). Among those who underwent surgical intervention (n = 302), initial procedures were: systemic to pulmonary artery shunt (SPS; n = 189; 62%); pulmonary artery banding (PAB; n = 50; 17%); and superior cavopulmonary connection (SCPC; n = 63; 21%). Multiphase parametric-hazard models were used to analyze competing outcomes. RESULTS: Risk-adjusted 6-year survival was lower after SPS (85%; P = .04) versus PAB (93%) or SCPC (93%). Survival after SPS when the main pulmonary artery (MPA) was closed (n = 21) or banded (n = 4) was 60%, versus 93% without MPA intervention (P = .02). After SPS, survival before SCPC was lower with an open ductus arteriosus (n = 7; 76% vs 97%; P = .02). Similarly, after SPS, risk-adjusted survival was similar to that for patients who had an initial PAB or SCPC when MPA intervention was avoided and the ductus arteriosus either closed spontaneously before SPS, or was closed during SPS. For all patients reaching SCPC (n = 277), survival to Fontan was not significantly influenced by whether PBF persisted through the MPA. CONCLUSIONS: Tricuspid atresia patients with SPS represent a high-risk subgroup. Avoiding an open ductus arteriosus and concomitant MPA intervention during SPS may help mitigate the risk associated with SPS. The presence of antegrade PBF through the MPA, at initial and staged operations, did not significantly influence survival to Fontan operation.