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Address for reprints: Christopher W. Mastropietro, MD, FCCM, Department of Pediatrics, Indiana University School of Medicine, Cardiac Intensive Care Unit, Riley Hospital for Children, 705 Riley Hospital Drive, Riley Phase 2, RI4119C, Indianapolis, IN 46202.
Division of Critical Care, Department of Pediatrics, Columbia University College of Physicians & Surgeons, Morgan Stanley Children's Hospital of New York, New York, NY
Division of Cardiac Intensive Care, Department of Pediatrics, University of Central Florida College of Medicine, The Heart Center at Arnold Palmer Hospital for Children, Orlando, Fla
Division of Cardiac Critical Care, Department of Pediatrics, Zucker School of Medicine at Hofstra/Northwell, Cohen Children's Medical Center of NY, New Hyde Park, NY
Divisions of Cardiology and Critical Care Medicine, Department of Pediatrics, Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Ill
We sought to describe characteristics and operative outcomes of children who underwent repair of truncus arteriosus and identify risk factors for the occurrence of major adverse cardiac events (MACE) in the immediate postoperative period in a contemporary multicenter cohort.
Methods
We conducted a retrospective review of children who underwent repair of truncus arteriosus between 2009 and 2016 at 15 centers within the United States. Patients with associated interrupted or obstructed aortic arch were excluded. MACE was defined as the need for postoperative extracorporeal membrane oxygenation, cardiopulmonary resuscitation, or operative mortality. Risk factors for MACE were identified using multivariable logistic regression analysis and reported as odds ratios (ORs) with 95% confidence intervals (CIs).
Results
We reviewed 216 patients. MACE occurred in 44 patients (20%) and did not vary significantly over time. Twenty-two patients (10%) received postoperative extracorporeal membrane oxygenation, 26 (12%) received cardiopulmonary resuscitation, and 15 (7%) suffered operative mortality. With multivariable logistic regression analysis (which included adjustment for center effect), factors independently associated with MACE were failure to diagnose truncus arteriosus before discharge from the nursery (OR, 3.1; 95% CI, 1.3-7.4), cardiopulmonary bypass duration >150 minutes (OR, 3.5; 95% CI, 1.5-8.5), and right ventricle-to-pulmonary artery conduit diameter >50 mm/m2 (OR, 4.7; 95% CI, 2.0-11.1).
Conclusions
In a contemporary multicenter analysis, 20% of children who underwent repair of truncus arteriosus experienced MACE. Early diagnosis, shorter duration of cardiopulmonary bypass, and use of smaller diameter right ventricle-to-pulmonary artery conduits represent potentially modifiable factors that could decrease morbidity and mortality in this fragile patient population.
One-fifth of children who underwent repair of truncus arteriosus suffered major adverse cardiac events. We identified potentially modifiable risk factors for the occurrence of these events.
In a multicenter cohort, 20% of children suffered MACE after repair of truncus arteriosus. Diagnosis after nursery discharge, cardiopulmonary bypass duration >150 minutes, and right ventricle-to-pulmonary artery conduit diameter >50 mm/m2 were identified as independent and potentially modifiable risk factors for MACE in these children.
See Commentaries on pages 2399, 2402, and 2404.
Over the past 15 years, survival after surgery for congenital heart disease has steadily improved.
For children born with a common arterial trunk, or truncus arteriosus, surgical mortality between 2005 and 2009 reported from the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) was 9.2%.
Another study from the STS-CHSD reported the need for extracorporeal membrane oxygenation (ECMO) after repair of truncus arteriosus to be 9.4%, which was high relative to other operations, and mortality in this subset was 71%.
Previous studies have identified moderate to severe truncal valve regurgitation or interrupted aortic arch (IAA) to be risk factors for mortality and complications after repair of truncus arteriosus.
Outcomes of repair of common arterial trunk with truncal valve surgery: a review of the Society of Thoracic Surgeons Congenital Heart Surgery Database.
These coexistent lesions, however, represent a relatively small proportion of patients. Between 2000 and 2009, 572 patients were entered into the STS-CHSD as having surgical repair of truncus arteriosus, yet only 22 underwent concomitant repair of their truncal valve, 34 underwent concomitant repair of IAA, and 5 underwent concomitant repair of both additional lesions.
Outcomes of repair of common arterial trunk with truncal valve surgery: a review of the Society of Thoracic Surgeons Congenital Heart Surgery Database.
Of the remaining 511 patients, 45 (9%) died. Thus, a considerable portion of children with truncus arteriosus suffer early mortality despite the absence of IAA or concomitant truncal valve surgery.
To date, most data on operative outcomes after repair of truncus arteriosus are from single-center studies.
These data, although useful, are often difficult to interpret and generalize because of variations in preoperative, surgical, and postoperative management that exist between centers. We aim to describe characteristics and operative outcomes of children who underwent repair of truncus arteriosus using a contemporary multicenter data set. From these data, we aim to identify risk factors for poor outcome after repair of truncus arteriosus, defined as the occurrence of major adverse cardiac events (MACE) in the postoperative period. We hypothesized that analysis of data compiled from multiple centers that includes variables not recorded in the STS-CHSD would allow us to identify previously unrecognized risk factors for poor outcome after repair of truncus arteriosus.
Methods
Study Population
We performed a retrospective review of all patients who underwent primary surgical repair of truncus arteriosus at 15 tertiary care pediatric referral centers between 2009 and 2016. A list of participating institutions is provided in Table E1. The study was approved by the institutional review boards at all centers and was performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. Because of the retrospective nature of the data collected, the need for informed consent was waived.
The following patients were excluded from the analysis:
•
Children who underwent pulmonary artery banding but died before repair
•
Children with hemitruncus (ie, right pulmonary artery coming off the aorta) or pseudotruncus (ie, pulmonary atresia with major aortopulmonary collaterals)
•
Children who underwent concomitant repair of truncus arteriosus with IAA or aortic arch obstruction (Van Praagh type A4), which is designated as a Society of Thoracic Surgeons–European Association for Cardio-Thoracic Surgery mortality category 5 procedure, in contrast to repair of truncus arteriosus (Van Praagh type A1-A3), which is a Society of Thoracic Surgeons–European Association for Cardio-Thoracic Surgery mortality category 4 procedure
(data for children with this lesion will be reported by our research group elsewhere).
Data Collection and Definitions
A comprehensive list of variables and definitions is included in Table E2. Right ventricle-to-pulmonary artery (RV-PA) conduit diameter was indexed to body surface area (BSA) to adjust the absolute values for differences in body size across subjects. The primary outcome of interest was the occurrence of MACE defined as intraoperative or postoperative cardiopulmonary resuscitation (CPR), ECMO, or operative mortality. This composite outcome measure has been used elsewhere in the cardiac surgical literature.
Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery: an analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.
Data are represented as medians with 25th and 75th percentiles for continuous variables and absolute counts with percentages for categorical variables unless otherwise noted. To determine risk factors associated with MACE after repair of truncus arteriosus, we performed a bivariate analysis using Wilcoxon rank sum test, χ2 test, and Fisher exact test as appropriate for individual variables. All variables with P values <.2 on bivariate analyses were considered for inclusion in our multivariate logistic regression model. The multivariable model was also analyzed as a mixed model with center as a random effect. We treated the effect of center on the model as a random (as opposed to fixed) variable because the centers involved in the study represent a random sample of the population of all congenital cardiac centers, with considerable variation in center volume, preoperative and postoperative care models, and geographic location. Linearity in the logit was examined for continuous variables before model-building; variables with evidence of nonlinearity were converted to categorical variables using receiver operative characteristic analysis to identify optimal cut points. This approach (as opposed to transformation of the continuous data) was chosen to facilitate clinical interpretation of the data analysis. Variables with P values < .05 after multivariable analysis were identified as independent risk factors for MACE after surgical repair of truncus arteriosus. All statistical analyses were performed using STATA version 14 (StataCorp LP, College Station, Tex) and SAS version 9.4 (SAS Institute Inc, Cary, NC).
Results
We retrospectively enrolled 216 children who underwent surgical repair of truncus arteriosus (Van Praagh type A1-A3) between 2009 and 2016 at 15 institutions. Characteristics of the entire cohort are summarized in Table 1, Table 2, Table 3. Prenatal diagnosis occurred in 63% of patients, whereas 21% were not diagnosed before discharge from the nursery, with 14 patients (6%) being diagnosed outside of the neonatal period (>30 days). The distribution of age at diagnosis is depicted in Figure E1. The proportion of patients diagnosed prenatally and after discharge from the nursery did not vary significantly over time (Figure E2) and did not vary significantly across centers (P = .13 for both relationships). However, 17 patients (8%) in the study were discharged to home after their diagnosis was confirmed, with a plan of medical management and elective surgery at a later date. Twelve of these patients (71%) were readmitted before their scheduled surgery for acute respiratory failure or congestive heart failure. Moreover, 3 of the children developed necrotizing enterocolitis (1 of whom underwent surgical intervention for perforation) and 2 suffered cardiac arrests (1 of whom received preoperative ECMO support) before their surgical repair.
Table 1Comparison of patients with and without MACE after repair of truncus arteriosus (2009-2016); demographic information and baseline patient characteristics
n = 207 patients; excludes 8 patients who underwent direct anastomoses of main pulmonary artery segment and 1 patient who underwent placement of systemic-to-pulmonary artery shunt.
n = 207 patients; excludes 8 patients who underwent direct anastomoses of main pulmonary artery segment and 1 patient who underwent placement of systemic-to-pulmonary artery shunt.
51 (45.6, 56.4)
49.9 (44.9, 55.5)
52.9 (49, 57.3)
.01
RV-PA conduit size >50 mm/m2
112 (52)
79 (46)
33 (75)
<.01
Truncal valve repaired
34 (16)
25 (15)
9 (21)
.34
Truncal valve replaced
6 (3)
3 (2)
3 (7)
.10
Pulmonary artery banding
3 (1)
3 (2)
0 (0)
1.00
Contegra conduits are from Medtronic Inc (Minneapolis, Minn).
MACE, Major adverse cardiac events; RV-PA, right ventricle-to-pulmonary artery.
∗ Continuous variables are represented as median (25th, 75th percentile); categorical data are represented as absolute counts (%).
† n = 207 patients; excludes 8 patients who underwent direct anastomoses of main pulmonary artery segment and 1 patient who underwent placement of systemic-to-pulmonary artery shunt.
Operatively, 191 patients (88%) received 1 of 3 types of RV-PA conduits: pulmonary allograft, aortic allograft, or Contegra (Medtronic Inc, Minneapolis, Minn) bovine jugular vein conduit. Of the remaining 25 patients, 13 patients received femoral vein allografts, 2 patients received GORE-TEX (W.L. Gore & Associates Inc, Flagstaff, Ariz) conduits, 1 patient received a conduit created from anterior pericardium, 8 patients underwent direct anastomosis of their main pulmonary artery segment to their right ventricle (with or without patch augmentation), and 1 patient with a coexisting unbalanced atrioventricular septal defect had a systemic-to-pulmonary artery shunt placed. For the 209 patients who received RV-PA conduits, the range of the absolute values for the RV-PA conduit diameters used was relatively narrow, with all but 3 of these patients receiving a conduit with a diameter between 8 mm and 14 mm. When indexed to BSA, this range of RV-PA conduit diameters was 29 mm/m2 to 78 mm/m2.
For the entire patient population, median postoperative duration of mechanical ventilation was 5 days (range, 1-148), median hospital length of stay was 23 days (range, 6-282), and operative mortality was 6.9% (n = 15). MACE occurred in 44 patients (20%). The proportions who suffered each type of MACE or other complications are provided in Table E3, and variation in MACE and operative mortality across institutions is provided in Figure 1. Of note, operative mortality in the 22 patients who received ECMO was 36% (n = 8). Bivariate analysis of demographic data, underlying comorbidities, and preoperative and operative variables is included in Table 1, Table 2, Table 3. Preoperatively, patients who experienced MACE were significantly more likely to have been diagnosed after discharge from the nursery and more likely to have developed shock, although patients who were diagnosed after discharge from the nursery were not more likely to develop shock preoperatively—4 of 45 children (9%)—compared with 17 of 171 children (10%) diagnosed prenatally or before nursery discharge (P = 1.00).
Figure 1Variation across centers in the number of patients who underwent repair of truncus arteriosus and the occurrence of major adverse cardiovascular events. Each bar contains the number of patients who suffered operative mortality (blue portion), number who suffered cardiac arrest or received extracorporeal membrane oxygenation but survived (red portion), and number who did not suffer major adverse cardiovascular events (green portion of bars). Centers are arranged in order of increasing surgical volume of patients who underwent truncus arteriosus repair during the study period.
We also found that patients with MACE had longer duration of cardiopulmonary bypass (CPB) and larger diameter RV-PA conduits (indexed to BSA), whereas aortic cross-clamping duration, use of deep hypothermic circulatory arrest, and lowest targeted temperature during surgery were not statistically different between patients with and without MACE. Using receiver operative characteristic analysis, we identified CPB duration greater than 150 minutes and RV-PA conduit diameter greater than 50 mm/m2 as optimal cut-points for prediction of MACE. The relationships between MACE, RV-PA conduit diameter, and BSA are illustrated in Figure 2, where the significant increase in the MACE in patients with RV-PA conduit diameter greater than 50 mm/m2 can be visually appreciated. In contrast, the occurrence of MACE was relatively evenly distributed across different body sizes. Moreover, we noted a significantly higher proportion of MACE in patients who received Contegra conduits, although conduit diameter was significantly larger in the 55 patients who received Contegra conduits, median 54 mm/m2 (range, 48-57), compared with the 136 patients who received pulmonary or aortic allografts, median 50 mm/m2 (range, 44-54; P < .01).
Figure 2Major adverse cardiovascular events (MACE) in relation to body surface area and the diameter of surgically-placed right ventricle-to-pulmonary artery (RV-PA) conduit in patients who underwent repair of truncus arteriosus. Patients who experienced MACE (n = 44, red circles) were significantly more likely to have RV-PA conduits >50 mm/m2 in diameter (dotted reference line) than patients who did not experience MACE. Specifically, 33 of 44 patients (75%) who suffered MACE had RV-PA conduits >50 mm/m2, compared with 79 of 172 patients (46%) who did suffer MACE (P < .01). Importantly, though smaller patients were more likely to have larger diameter RV-PA conduits, no significant relationship between the occurrence of MACE and body size was observed.
The results of our mixed effects logistic regression analysis are provided in Table 4, including odds ratios (ORs) and 95% confidence intervals (CIs) for the fixed effects adjusted for center. We identified diagnosis after discharge from the nursery, CPB duration >150 minutes, and RV-PA conduit diameter >50 mm/m2 as factors independently associated with MACE after repair of truncus arteriosus in a model that included preoperative shock. The relationship between the CPB duration and RV-PA conduit diameter with postoperative complications is further explored in Figure 3. Operative mortality, CPR, delayed sternal closure, and postoperative tachyarrhythmias were significantly more common in patients with larger diameter RV-PA conduits (Figure 3, A). In contrast, operative mortality, ECMO, and inhaled nitric oxide use were significantly more frequent in patients with prolonged CPB duration (Figure 3, B).
Table 4Multivariable logistic regression analysis for predictors of operative mortality after repair of truncus arteriosus (analyzed as a mixed model with a random effect of center)
Figure 3A, Patients with right ventricle-to-pulmonary artery (RV-PA) conduits >50 mm/m2 in diameter were significantly more likely to suffer operative mortality and receive cardiopulmonary resuscitation ([CPR] ie, major cardiac adverse events [MACE]). Patients with RV-PA conduits >50 mm/m2 were also more likely to experience tachyarrhythmias or undergo delayed sternal closure. In contrast, use of extracorporeal membrane oxygenation (ECMO) or inhaled nitric oxide (iNO) were not significantly more likely to occur in patients with conduit diameters >50 mm/m2. B, Patients with cardiopulmonary bypass (CPB) duration >150 minutes were more likely to suffer operative mortality and receive ECMO (ie, MACE), and more likely to be administered iNO. The occurrence of CPR, tachyarrhythmias, and delayed sternal closure, however, were not statistically more likely in patients with CPB durations >150 minutes. *Statistically significant, P < .05.
We notably did not find an increased occurrence rate of MACE patients who underwent concomitant surgical intervention on their truncal valve (Table 2). It should be noted that of the 6 patients who underwent truncal valve replacement at the time of the surgery, MACE occurred in the 3 patients who first underwent an attempt at truncal valve repair and then underwent a second course of CPB and truncal valve replacement during the same operation (CPB durations: 333, 336, and 356 minutes), whereas no MACE occurred in the 3 patients who underwent truncal valve replacement without a previous attempt at truncal valve repair (CPB durations: 217, 291, and 323 minutes). Accordingly, to better examine whether the association of MACE and prolonged CPB duration was more related to the duration of CPB itself or the need for concomitant truncal valve intervention that contributed to the prolonged duration of CPB, we performed a sensitivity analysis excluding the 37 patients who underwent truncal valve interventions. In this mixed effects logistic regression analysis, which included adjustments for preoperative shock as a fixed effect and center as a random effect, failure to diagnose truncus arteriosus before nursery discharge (OR, 2.8; 95% CI, 1.1-7.4), CPB duration >150 minutes (OR, 3.0; 95% CI, 1.1-7.7), and RV-PA conduit diameter >50 mm/m2 (OR, 3.3; 95% CI, 1.3-8.2) remained independently associated with MACE. A summary of these results can also be viewed in Video 1.
Discussion
In this contemporary multicenter data set, we found that 1 in 5 patients who underwent surgical repair of truncus arteriosus suffered MACE. Furthermore, although operative mortality was lower than in previous reports from the STS-CHSD, especially in patients requiring ECMO,
the occurrence of MACE did not decrease appreciably during the study period and complications were frequent relative to other neonatal operations. For example, 58% of patients in our study underwent delayed sternal closure (planned or unplanned), in contrast to 21% of all neonates reported to the STS-CHSD between 2013 and 2016.
In other words, although operative mortality for patients who undergo surgery for congenital heart disease might be improving, patients with truncus arteriosus remain at considerable risk for complications and adverse cardiac events including death after surgery.
Fortunately, we identified 3 independent and potentially modifiable risk factors for MACE after repair of truncus arteriosus: failure to diagnose truncus arteriosus before discharge from the nursery, CPB duration >150 minutes, and RV-PA conduit diameter >50 mm/m2. Interestingly, in our multivariable analysis, diagnosis after discharge from the nursery (ie, late diagnosis) was associated with MACE independent of preoperative shock. On the basis of this analysis, we speculate that many patients who were diagnosed late presented in an advanced state of compensated congestive heart failure that had not yet progressed to meet the STS-CHSD definition of shock, and neonates need not progress to a late stage of shock before their burden of illness will influence their postoperative course. Early diagnosis before the development of shock with lactic acidosis should therefore be the aim of current neonatal screening protocols.
In 2009, the American Academy of Pediatrics and American Heart Association published a joint statement on pulse oximetry screening as a means to detecting undiagnosed congenital heart disease before nursery discharge.
Section on Cardiology and Cardiac Surgery Executive Committee Endorsement of Health and Human Services recommendation for pulse oximetry screening for critical congenital heart disease.
In 2017, data from the National Center for Health Statistics showed a significant decrease in infant cardiac deaths between 2007 and 2013 in states that had implemented statewide mandatory policies for newborn screening for critical congenital heart disease compared with states without these policies.
Data on the adherence to these practices at individual centers, however, are sparse. In one recent report from a center where the practice of routine pulse oximetry screening for congenital heart disease was adopted and then audited, compliance with their protocol was 88%, which is good but not optimal.
The association of late diagnosis with MACE after repair of truncus arteriosus in our study and the aforementioned data on the benefits of routine pulse oximetry screening for congenital heart disease support the further expansion of this practice and the establishment of infrastructure at the local level to ensure that these programs are operating appropriately.
It should also be noted that 17 patients were discharged to home after diagnosis, of whom 71% were readmitted with life-threatening illnesses before repair. This observation argues against sending these children home without surgical intervention. Indeed, the feasibility and practicality of performing the surgical repair of truncus arteriosus in the neonatal period have long been established and confirmed by many single center studies.
On the basis of the observed association between longer duration of CPB and use of ECMO or inhaled nitric oxide, we presume that longer durations of CPB in our patients could have predisposed them to myocardial depression and pulmonary hypertensive crises that necessitated these adjunctive therapies. Notably, the relationship between prolonged CPB duration and adverse outcomes persisted when operations with concomitant truncal valve procedures were excluded. We are not, however, advocating that the quality of repair be sacrificed in an effort to be more expeditious. Rather, awareness of the association between MACE and prolonged duration of CPB after repair of truncus arteriosus should emphasize the importance of minimizing exposure to CPB whenever possible.
Perhaps the most compelling and easily modifiable identified risk factor for MACE after repair of truncus arteriosus is larger diameter of the RV-PA conduit, specifically >50 mm/m2. (For reference, in Figure E3, absolute RV-PA conduit diameters that fall below the threshold of 50 mm/m2 for BSAs between 0.15 m2 and 0.25 m2 are provided.) In a single-center study of 83 patients, Tlaskal and colleagues also noted a trend toward increased mortality in patients with larger absolute RV-PA conduit diameters.
To our knowledge, our study represents the first to examine RV-PA conduit diameter indexed to BSA as an important variable to consider when planning surgical correction for these children. On the basis of our data, the pathophysiologic explanation for this finding cannot be clearly discerned. We speculate that larger diameter conduits could be associated with increased ventriculotomy size relative to the size of the neonatal myocardium, which could exacerbate restrictive right ventricular physiology or provide an arrhythmogenic substrate during the recovery period. Larger conduits could suffer from distortion or compression during sternal closure leading to conduit insufficiency, pulmonary artery distortion, or prolonged duration of open sternotomy, predisposing patients to infectious or respiratory complications. A combination of these possibilities could be responsible for the worse outcomes in patients with conduit diameters >50 mm/m2. Future studies on the relationship between conduit size and postoperative outcomes should be designed to more specifically investigate these or other potential pathophysiologic mechanisms.
Nearly all patients who undergo repair of truncus arteriosus will require replacement of their RV-PA conduit later in life and, consequently, many surgeons will err on the side of larger conduit diameters in an attempt to extend the period of time before conduit revision is needed. Indeed, several studies have associated smaller absolute conduit diameter with earlier need for reintervention,
In a seminal study from the Congenital Heart Surgery Society of 429 children with various congenital heart lesions who required RV-PA conduits, use of smaller conduits were associated with earlier reinterventions, leading the authors to conclude “insertion of the largest conduit possible (within the constraints of our data and patient size) would be expected to postpone explantation prompted by the somatic growth of the patient.”
On the basis of our data, use of larger conduits to promote long-term durability could come at a cost for some patients. None of the 8 patients who underwent direct anastomoses of the pulmonary artery suffered MACE, and studies have reported longer freedom from reoperation in patients who underwent direct anastomoses.
Repair of persistent truncus arteriosus without a conduit: sleeve resection of the pulmonary trunk from the aorta and direct right ventricle-pulmonary artery anastomosis.
Additionally, a recent study reported that modified repair of truncus arteriosus in which the branch pulmonary arteries are left in situ and septated from the truncal root might promote conduit longevity.
Further research focused on these modifications or other innovations could mitigate the impetus to use larger conduits at the initial repair of truncus arteriosus and, possibly, reduce the risk of MACE. Accordingly, additional research in children with congenital heart disease who receive RV-PA conduits should aim to determine a range of conduit diameters that could optimally balance the risks of short- and long-term morbidity and mortality.
This study has the limitations inherent to its retrospective design. For example, data on RV-PA conduit diameter were obtained retrospectively from operative reports and not confirmed by direct measurement via postoperative echocardiogram. If actual RV-PA conduit diameters differed from what was provided by the tissue record, the accuracy of our RV-PA conduit measurements would be affected. We also acknowledge that the components of our composite primary outcome measure differ in terms of severity (ie, mortality is a worse outcome than CPR or ECMO alone). However, etiologies for the need for CPR, need for ECMO, or operative mortality were likely similar in many patients (eg, myocardial depression, pulmonary hypertensive crises, arrhythmias, multiorgan dysfunction) and most clinicians would agree that any of the 3 included outcomes are undesirable. Notably, we did not include long-term outcomes in this study but rather maintained the focus on early outcomes. Last, patients for this study were identified in institutional surgical databases, which did not allow us to report data on patients who might have been diagnosed with truncus arteriosus but died before surgical intervention. The strength of this study is its collaborative multicenter design, which allowed us to obtain data on variables not entered into current databases such as the STS-CHSD as well as identify risk factors for MACE after repair of truncus arteriosus that are independent of center. Future analyses will focus on postdischarge outcomes including freedom from conduit intervention, truncal valve intervention, or death.
Conclusions
Operative mortality and other adverse cardiac events after repair of truncus arteriosus continue to be relatively common. In our contemporary multicenter study, operative mortality was 6.9% and MACE occurred in one-fifth of patients. We hope that future investigations will reveal that attention to the independent and potentially modifiable risk factors identified in this report—diagnosis after discharge from the nursery, duration of CPB >150 minutes, and RV-PA conduit diameter >50 mm/m2—will lead to improvements in the operative and early postoperative outcomes in these fragile children. We also suggest that neonatal repair becomes the preferred option for surgical timing at all centers, because patients who are discharged to home with medical management after diagnosis of truncus arteriosus are at considerable risk of preoperative morbidity.
Authors have nothing to disclose with regard to commercial support
The authors acknowledge and thank the following individuals who assisted with administrative tasks, data collection, or review of echocardiograms for this study:
•
Amy Moravec, BS, Ann & Robert H. Lurie Children's Hospital of Chicago
•
Kari Nelson, BA, MSN, CPNP, University of Wisconsin-American Family Children's Hospital
•
Josh Belfer, MD, Resident in Pediatrics, Cohen Children's Medical Center of New York
•
Eva Cheung, MD, Assistant Professor, Department of Pediatrics, Division of Cardiology, Columbia University College of Physicians & Surgeons, Morgan Stanley Children's Hospital of New York, New York, New York
•
Linda M. Lambert, MSN-cFNP, Administrative Director of Research, Heart Center–Primary Children's Hospital, University of Utah
•
Lisa Michelle Hansen, Clinical Research Coordinator, Heart Center–Primary Children's Hospital, University of Utah
Figure E1Histogram showing the variation in age at diagnosis of patients with truncus arteriosus included in the study. Most patients (n = 188; 87%) were prenatally diagnosed or diagnosed within the first week of life, whereas 14 patients (6%) were diagnosed outside the neonatal period (>30 days), which includes an outlier not represented in the histogram—a child who was adopted from outside the United States with a presumed diagnosis of tetralogy of Fallot but was found to have truncus arteriosus upon evaluation on day of life 1275 (3.5 years).
Figure E2Trends in the diagnosis of truncus arteriosus over time. The percentage of patients diagnosed with truncus arteriosus prenatally (blue bars), postnatally but before discharge from the nursery (red bars), and postnatally after discharge from the nursery (green bars). The variations in the percentage of patients diagnosed prenatally and patients diagnosed after discharge from the nursery over the duration of the study period were not statistically significant (P = .48 and P = .98, respectively).
Figure E3Absolute right ventricle-to-pulmonary artery (RV-PA) conduit diameters for patients with body surface areas between 0.15 and 0.25 mm2 (10th percentile to 90th percentile of study population) that will result in diameters indexed to body surface area up to 50 mm/m2. RV-PA conduit diameters above the blue bars at each body surface area might increase the risk of major adverse cardiovascular events after repair of truncus arteriosus.
Age, sex, race, ethnicity, and anthropometric data
•
Estimated gestational age at birth
•
Prematurity: less than 37 weeks' gestation
•
Presence of genetic anomalies or noncardiac anatomic anomalies
•
Age and setting of diagnosis (ie, prenatal diagnosis, diagnosis after birth but before discharge from the nursery, or diagnosis after discharge from the nursery)
Outcomes of repair of common arterial trunk with truncal valve surgery: a review of the Society of Thoracic Surgeons Congenital Heart Surgery Database.
Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery: an analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.
Repair of persistent truncus arteriosus without a conduit: sleeve resection of the pulmonary trunk from the aorta and direct right ventricle-pulmonary artery anastomosis.
Funding from the Department of Pediatrics at Indiana University School of Medicine was provided for this study through a Riley Children’s Foundation Grant (intramural) for administrative support.
Truncus arteriosus (TA) accounts for 1% to 4% of all cases of congenital heart disease.1 McGoon and colleagues2 reported on the first repair of TA with a valved homograft in 1967. Since then, improvements in cardiopulmonary bypass, surgical techniques, and postoperative management have all reduced early mortality.3
The article in this issue of the Journal by Mastropietro and colleagues1 investigates in-hospital adverse outcomes and their determinants among patients with truncus arteriosus undergoing complete primary repair at 15 selected institutions from 2009 to 2016. Among the 216 patients included, the in-hospital mortality was 6.9%, and the prevalence of major adverse cardiac events (MACE), defined as intraoperative or perioperative cardiopulmonary resuscitation, extracorporeal membrane oxygenation, or death, was 20%.
In the current issue of the Journal, Mastropietro and colleagues1 retrospectively reviewed the results of 216 children who had undergone surgical repair of truncus arteriosus at 15 institutions in the United states between 2009 and 2016. They focused on early outcomes and assessed risk factors associated with major adverse cardiac events (MACE), an outcome variable that combined in-hospital mortality with the requirement for postoperative cardiopulmonary resuscitation or extracorporeal membrane oxygenation support.
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