Abstract
BACKGROUND:
The goal of this study is to describe in-hospital and long-term mortality following single-stage repair of truncus arteriosus communis (TAC) and explore factors associated with these outcomes.
METHODS:
This is a cohort study of consecutive patients undergoing single-stage TAC repair between 1982 and 2011 reported to the Pediatric Cardiac Care Consortium registry. In-hospital mortality was obtained for the entire cohort from registry records. Long-term mortality was obtained for patients with available identifiers via matching with the National Death Index through 2020. Kaplan-Meier survival estimates were created for up to 30 years post-discharge. Cox regression models estimated hazard ratios for the associations with potential risk factors.
RESULTS:
647 patients (51% male) underwent single-stage TAC repair at a median age of 18 days. 53% had type I TAC, 13% had interrupted aortic arch and 10% underwent concomitant truncal valve surgery. Of these, 486 (75%) survived to hospital discharge. Post-discharge, 215 patients had identifiers for tracking long-term outcomes; 30-year survival was 78%. Concomitant truncal valve surgery at index procedure was associated with increased in-hospital and 30-year mortality. Concomitant interrupted aortic arch repair was not associated with increased risk for in-hospital or 30-year mortality.
CONCLUSIONS:
Concomitant truncal valve surgery but not interrupted aortic arch was associated with higher in-hospital and long-term mortality. Careful consideration of the need and timing for truncal valve intervention may improve TAC outcomes.
Truncus Arteriosus (TAC) presents with anatomic variations that can influence management and outcomes. These include varying origins of the pulmonary arteries, interrupted aortic arch (IAA), coronary artery abnormalities (CAA), truncal valve (TrV) disease and presence of DiGeorge syndrome (DGS).(1,2) Single-stage repair of TAC involves closure of the ventricular septal defect, disengagement of the pulmonary artery/arteries (PA) and anastomosing it/them to the right ventricle (RV). If IAA is present, aortic reconstruction is required. TrV intervention may be needed to address hemodynamically significant TrV disease.
TAC and IAA surgeries are considered high risk procedures and are individually assigned as STAT category 4 and together as 5.(3) Reports following single-stage TAC repair with and without concomitant IAA or TrV surgery are limited by smaller sample size or single-center experiences that may not be generalizable.(4–13) Therefore, we used the Pediatric Cardiac Care Consortium (PCCC), a US-based multicenter registry, to describe in-hospital and long-term survival following single-stage repair of TAC and associated defects and to identify potentially modifiable risk factors associated with outcomes.
METHODS
This study was approved by the Institutional Review Boards of Children’s Mercy Hospital, Emory University School of Medicine, and University of Georgia. This is a retrospective study of all consecutive patients who underwent single-stage TAC repair at 42 centers reporting to the PCCC from 1982 to 2011 and included patients with TAC Types I-III based on Collett-Edwards classification.(1) Patients with Type IV TAC, “hemi-truncus”, staged/palliative repairs, initial TAC repair at a center outside the PCCC, or those with missing operative notes were excluded.
Data collected from clinical records at the time of TAC repair included sex, age, weight, presence of DGS, CAA, type of TAC, IAA, TrV morphology, TrV hemodynamic severity, and concomitant TrV surgery. Patients with TAC repair only were compared to those with concomitant surgeries for IAA and TrV. Patients with both IAA and TrV were included in the TrV surgery group in unadjusted analyses as the additional intracardiac surgery was thought to be the discriminating event. However, in multivariable analyses, both procedures were accounted for. Surgical era was classified as early (1982–1989), middle (1990–1999) and late (2000–2011) determined from the index surgery date. Complications during index surgery hospitalization requiring a procedural intervention included extracorporeal membrane oxygenation (ECMO), mediastinal exploration, pacemaker implantation, dialysis, thoracic duct ligation, tracheostomy, and additional cardiac surgical/transcatheter interventions.
Body surface area (BSA) was estimated from weight at surgery [(weight in kg x 4) + 7]/[90 + weight in kg].(14) The size of the RV-PA conduit relative to the expected size of the native main pulmonary artery was calculated as a z-score.(15,16) RV-PA conduit/BSA z-score was categorized based on previously reported meaningful cut-off points.(15,17) In multivariable analysis, a cubic spline was used to evaluate the non-linear association of RV-PA conduit/BSA z-scores. Center characteristics included total annual surgical volume, proportion of “high-risk” operations and in-hospital mortality for high-risk procedures. These were estimated from all cardiac surgeries performed in the centers six months before and after the index procedure (Supplementary Methods).
Outcomes
In-hospital survival following single stage repair of TAC and associated defects was ascertained from registry records. The PCCC has been linked to the National Death Index (NDI) through December 31, 2020 for patients with adequate identifiers enrolled between 1982 (the year of PCCC initiation) and April 15, 2003 (the date of stricter implementation of Health Insurance Portability and Accountability Act [HIPAA] which prohibited the use of identifiers for those subsequently enrolled).(18) Therefore, survival to hospital discharge after index TAC repair is reported for all patients, whereas long-term survival are only available for patients enrolled prior to April 15, 2003. Data provided by the NDI include date and underlying/contributing causes of death ascertained from the death certificate.(19)
Our secondary goals were to identify potentially modifiable risk factors for mortality. We also assessed the need for reintervention within 5 years of the index TAC repair. Events occurring after hospital discharge from the initial TAC repair were available if the patient returned to a PCCC center for subsequent care. The reintervention query was limited to five years because within this timeframe it is likely patients will return to the same center. Patients from 39 centers were included in this analysis. Reinterventions included RV-PA conduit replacement, pseudoaneurysm resection, TrV surgery, residual ventricular septal defect repair, and PA or aortic balloon angioplasty/stent/surgery.
Statistical Methods
Categorical variables are displayed as counts and percentages and compared utilizing chi-square or Fisher’s exact tests. Continuous variables are reported as medians with 25th-75th interquartile range (IQR) and compared by Wilcoxon rank sum or Kruskal-Wallis test for comparing two or three groups respectively. Adjusted risk ratios (aRR) were estimated to identify factors related to in-hospital mortality.
Survival after hospital discharge was defined as time from hospital discharge to death or December 31, 2020, whichever came first. Kaplan-Meier curves were estimated up to 30 years and compared using log-rank and Wilcoxon tests. Cox proportional hazards models were used to estimate adjusted hazard ratios (aHRs). Factors related to unadjusted post discharge mortality (p-value <0.2 on the log-rank or Wilcoxon test) were included in the model (Supplementary Methods). Causes of death were compared within and post 1 year of discharge, between sexes, and by age at surgery dichotomized at 90 days. The cumulative incidence of reoperations was estimated considering death as a competing event. The Fine and Gray method was used to estimate sub distribution hazard ratio of reintervention by types of TAC. Clustering within centers was accounted for using generalized estimating equations. Additionally, models were repeated including center-level characteristics to assess their impact on outcomes and repeated in those with simple TAC to ensure the observed effects were not influenced by a subset of patients with unique challenges. Complete case analysis was used to address missingness. Statistical analyses were performed using SAS version 9.4 (Cary, NC). Statistical significance was assessed at 0.05 level.
RESULTS
A total of 647 patients underwent single-stage TAC repair between 1982 and 2011 at a median age of 18 days (IQR 8–55). The most common TAC was Type 1 (53%), about half were male, (Table 1) and 60% were treated in a large volume center (Supplementary Table 1). Among the 647 patients, 330 had identifiers for NDI linkage, 78 could not be linked due to missing identifiers, and 239 were enrolled in the post-HIPAA period and therefore not eligible for NDI matching (Supplementary Figure 1). There were no differences between those with missing identifiers when compared to patients with available characteristics from the same era. However, patients enrolled after 2003 were younger at surgery, with higher proportion diagnosed with DGS, and had smaller RV-PA conduit/BSA z-scores (Supplementary Table 2).
Table 1:
Patient characteristics by underlying pathology and types of index TAC repair
| Characteristics¶ | TAC All N=647 | TAC Simple N=508 (%) | TAC/IAA§ N=75 (%) | TAC/TrV Surgery N=64 (%) |
|---|---|---|---|---|
| Age at Surgery, Days | ||||
| Median (IQR) | 18 (8–55) | 26 (10–67)a,b | 7 (5–12)a,c | 11 (6–31)b,c |
| ≤28 days | 382 (59) | 265 (52)a,b | 71 (95)a,c | 46 (72)b,c |
| 29 – 90 days | 185 (29) | 168 (33) | 3 (4) | 14 (22) |
| >90 days | 80 (12) | 75 (15) | 1 (1) | 4 (6) |
| Male | 327 (50) | 265 (52) | 36 (48) | 26 (41) |
| Surgical Era | ||||
| 1982 – 1989 | 78 (12) | 65 (13) | 9 (12) | 4 (6) |
| 1990 – 1999 | 215 (33) | 171 (34) | 25 (33) | 19 (30) |
| 2000 – 2011 | 354 (55) | 272 (54) | 41 (55) | 41 (64) |
| Surgical Weight | ||||
| Median (IQR) | 3.3 (2.8–3.8) | 3.3 (2.8–3.9)a | 3.0 (2.7–3.5)a,b | 3.2 (2.8–3.7)b |
| <2.5 kg | 63 (10) | 47 (9) | 12 (16) | 4 (6) |
| DGS | 166 (26) | 124 (24) | 22 (29) | 20 (31) |
| TAC Type | ||||
| Type I | 340 (53) | 265 (52) | 38 (51) | 37 (58) |
| Non–Type I | 307 (47) | 243 (48) | 37 (49) | 27 (42) |
| CAA | 101 (16) | 71 (14)a | 14 (19) | 16 (25)a |
| TrV Regurgitation/Stenosis | ||||
| None/Mild | 550 (85) | 479 (94)a | 69 (92)b | 2 (3)a,b |
| Moderate/Severed | 97(15) | 29 (6)a | 6 (8)b | 62 (97)a,b |
| TrV Morphology | ||||
| No Abnormality/Tricuspid | 295 (46) | 255 (50)a | 39 (52)b | 1 (2)a,b |
| Dysplastic/Abnormal Leafletse | 352 (54) | 253 (50)a | 36 (48)b | 63 (98)a,b |
| RV-PA Conduit/BSA Z-score | ||||
| Median (IQR) | 2.1 (1.2–2.7) | 2.0 (1.2–2.6)a | 2.3 (1.5–2.8)a | 2.1 (1.5–2.8) |
| CPB Time (Minutes) | ||||
| Median (IQR) | 137 (106–171) | 133 (105–161)a | 161 (103–191) | 173 (129–231)a |
| LOS (Days) | ||||
| Median (IQR) | 16 (10–28) | 15 (10–25)a,b | 26 (16–44)a | 21 (14–48)b |
| In-Hospital Death | 161 (25) | 109 (21)a | 23 (31) | 29 (45)a |
indicates p-value <0.05 for differences in each characteristic between the two groups signified
6 have stenosis
Includes truncal valves with 2, 4, 5 or 6 leaflets.
Missing observations: Surgical weight: 3; CAA: 3; RV-PA Conduit/BSA Z-score: 33; CPB time: 300
10 patients had IAA and TrV surgery
Concomitant IAA surgery was performed in 85 patients, concomitant TrV surgery in 64, and 10 patients underwent both (Figure 1). Patients with concomitant IAA or TrV surgery were younger at time of repair and the latter had higher incidence of CAA compared with those who underwent simple TAC repair (Table 1).
Figure 1.
In-hospital mortality compared by type of surgeries.
The most common IAA was type B (73%). End-to-end anastomosis with a patch was the most common repair technique followed by direct end-to-end anastomosis in 34% and 26% subjects, respectively. Most patients (69%) who underwent TrV surgery had a quadricuspid valve anatomy with the majority described to have hemodynamically significant TrV regurgitation. Tricuspidalization of the TrV was the most common repair technique in 30%, followed by aortic homograft placement in 19%.
There were 43 patients with moderate-severe or severe TrV regurgitation, 36 of these underwent concomitant TrV surgery and 7 did not (18/36 and 6/7 died in-hospital respectively). In those with moderate only TrV regurgitation, 26/54 received concomitant TrV surgery but with no impact on in-hospital mortality (Supplementary Table 3).
About a third of the cohort experienced a complication during index hospitalization with need for ECMO in 8% and additional cardiac surgery in 4% of patients (Table 2). Overall, all-cause in-hospital mortality was 25%. Mortality decreased from 35% in the 1980s to 10% in the 2000s (p<0.001). In hospital mortality was higher in early era, weight at surgery < 2.5 kg, non-type 1 TAC, concomitant TrV surgery, in those with complications, larger RV/PA conduit and small volume centers (Figure 1, Table 3, and Supplementary Table 4).
Table 2:
In-hospital complications requiring intervention after index TAC repair
| Total N=647 (%) | TAC (Simple) N=508 (%) | TAC/IAA N=75 (%) | TAC/TrV Surgery N=64 (%) | |
|---|---|---|---|---|
| Any Complication a | 134 (21) | 95 (19) | 21 (28) | 18 (28) |
| ECMO | 49 (8) | 33 (6) | 9 (12) | 7 (11) |
| Mediastinal Exploration | 19 (3) | 17 (3) | 1 (1) | 1 (2) |
| Pacemaker | 10 (2) | 6 (1)c | 0 (0)d | 4 (6)c,d |
| Renal Failure | 25 (4) | 16 (3) | 4 (5) | 5 (8) |
| Thoracic Duct Ligation | 4 (<1) | 4 (1) | 0 (0) | 0 (0) |
| Tracheostomy | 10 (2) | 6 (1) | 3 (4) | 1 (2) |
| Otherb | 7 (1) | 5 (1) | 0 (0) | 2 (3) |
| Subsequent Cardiac Procedures During Index Surgery Hospitalization | ||||
| Surgical | 27 (4) | 16 (3)c | 6 (8) | 5 (8)c |
| Transcatheter | 11 (2) | 7 (1) | 4 (5) | 0 (0) |
Patients can have ≥1 specific complication
Includes peritoneal drain, bowel resection, diaphragm plication, pericardiocentesis
p-value <0.05 for chi-squared test comparing two groups signified
Table 3:
Association of patient characteristics with in-hospital mortality (n=647)
| Characteristics | In-Hospital Death N=161 (%) | Survived to Hospital Discharge N=486 (%) | aRR (95% CI)¥ |
|---|---|---|---|
| Age at Surgery | |||
| ≤28 days | 96 (25) | 286 (75) | ref |
| 29 – 90 days | 53 (29) | 132 (71) | 1.0 (0.8–1.4) |
| >90 days | 12 (15) | 68 (85) | 0.5 (0.3–0.9) |
| Sex | |||
| Male | 77 (24) | 250 (76) | ref |
| Female | 84 (26) | 236 (74) | 1.1 (0.8 – 1.4) |
| Surgical Era | |||
| 1982 – 1989 | 40 (51)a | 38 (49)a | 4.5 (3.1–6.4) |
| 1990 – 1999 | 71 (33)a | 144 (67)a | 2.3 (1.7–3.2) |
| 2000 – 2011 | 50 (14)a | 304 (86)a | ref |
| Surgical Weight | |||
| <2.5 kg | 24 (38)a | 39 (62)a | 1.7 (1.2–2.5) |
| ≥2.5 kg | 136 (23)a | 445 (77)a | ref |
| DGS | |||
| Yes | 35 (21) | 131 (79) | 0.9 (0.6–1.2) |
| No | 126 (26) | 355 (74) | ref |
| TAC Type | |||
| Type I | 74 (22) | 266 (78) | ref |
| Non–Type I | 87 (28) | 220 (72) | 1.3 (1.0–1.7) |
| CAA | |||
| Yes | 29 (29) | 72 (71) | 1.1 (0.8–1.5) |
| No | 131 (24) | 413 (76) | ref |
| IAA | |||
| Yes | 27 (32) | 58 (68) | 1.0 (0.7–1.4) |
| No | 134 (24) | 428 (76) | ref |
| TrV Surgery b | |||
| Yes | 29 (45)a | 35 (55)a | 2.2 (1.6–3.0) |
| No | 132 (23)a | 451 (77)a | ref |
| RV-PA Conduit/BSA Z-score | |||
| ≤1 | 24 (21)a | 89 (79)a | 1.1 (0.7–1.7) |
| >1 – 2 | 37 (21)a | 143 (79)a | ref |
| >2 – 2.5 | 26 (20) | 103 (80)a | 1.0 (0.7–1.6) |
| >2.5 | 66 (35) | 125 (65) | 1.4 (1.0–2.0) |
| Intervention for Complication c | |||
| Yes | 68 (51)a | 66 (49)a | 2.6 (2.0–3.4) |
| No | 93 (18)a | 420 (82)a | ref |
Unadjusted comparison of in-hospital mortality by characteristics, p-value <0.05
Trv Surgery – Repair in 52, Replacement in 12, 39% mortality among repair and 75% mortality among replacement group (p=0.02)
Includes surgical and catheter-based interventions as mentioned in Table 2.
bolded aRR indicates statistically significant 95% CI
Long-term Survival
Of the 330 patients matched to NDI, 215 (65%) survived to hospital discharge and were included in long-term analysis. Over a median follow-up of 21.8 years (IQR 18.9–26.0), there were 42 deaths. The 30-year survival conditioned upon being alive at hospital discharge was 78% (95% CI: 71%−84%). Those who had concomitant IAA surgery had a 30-year survival of 90% (95% CI: 66–97%), while lower survival was noted for those who underwent concomitant TrV surgery, 49% (95% CI: 12–79%) (Supplementary Table 5, Figure 2). Half of late deaths occurred within a year following hospital discharge with the 30 year-survival conditioning surviving the 1st year post TAC repair reaching 87% (95% CI: 80 – 92%). Weight at surgery <2.5 Kg, surgery at >90 days vs <28 days, female sex and TrV surgery at index procedure predicted increased 30-year mortality (Figure 3). There were no sex-specific unique characteristics (Supplementary Tables 6 and 7). After inclusion of center volume in the model, non-type 1 TAC trended towards statistical significance (aHR=2.1 [1.0–4.3]) and the effect of female sex attenuated (aHR=1.8 [0.9–3.6]) (Supplementary Figure 2). Most of the contributory and underlying causes of late deaths were related to cardiovascular conditions and no meaningful differences were noted within or post one year of hospital discharge.
Figure 2.
A: Post discharge Kaplan-Meier survival plot with 95% CI following single-staged TAC repair, all types. B: Post discharge Kaplan-Meier survival plot with 95% CI following single-stage repair of TAC only, TAC with IAA and TAC with TrV surgeries
Figure 3.
Predictors of Late Mortality. X axis is on the natural log scale
Reintervention
The cumulative incidence of any reintervention was 50% within 5 years after hospital discharge (n=108) (Supplementary Figure 3). RV-PA conduit reintervention was most common, followed by PA reintervention and some underwent multiple reinterventions (Supplementary Table 8). Younger age at index surgery and presence of CAA were associated with shorter time to reintervention (Supplementary Table 9). The RV-PA conduit/BSA z-score at index surgery was related to time to reintervention in a nonlinear pattern (Supplementary Figure 4). No differences were observed in the sensitivity analysis restricted to those with simple TAC.
COMMENT
TAC is a rare congenital heart disease, categorized as “high-risk” in the current era despite relative simplicity of sub-procedure components. Our study, which to our knowledge includes the largest multicenter cohort after single-stage TAC repair is congruent with this categorization and provides meaningful estimates for survival and factors influencing it. In-hospital mortality remains high but has decreased across eras. There is a continued steep decline in survival within the first year of life following hospital discharge from index surgery and closer surveillance during this time may improve survival. Reassuringly, TAC with IAA as a single-stage procedure had outcomes similar to TAC alone in this larger sample size. However, concomitant TrV surgery was associated with higher in-hospital and long-term mortality suggesting that careful consideration of the need and timing for TrV intervention at index operation may offer an opportunity to improve outcomes.
In-hospital mortality following single-stage TAC repair has decreased likely due to a combination of earlier identification/intervention before congestive heart failure and pulmonary hypertension worsen and improved operative techniques and perioperative management. Nevertheless, in-hospital complications were significant, occurring in about a third of patients and contributed to in-hospital mortality. Among patients discharged alive, we estimated 30-year survival to be 78% for all types of TAC with few events occurring past the 20-year landmark. In concordance with the high STAT score, our in-hospital mortality is comparable to other contemporaneous reports.(6–8,10,11,13,20)
Concomitant TrV surgery increased the risk for in-hospital mortality. This may be due to the complexity of intervening on neonatal valves, additional cardiopulmonary bypass time in a volume/pressure loaded left ventricle and/or coronary artery compromise. Commonly reported indications for concomitant TrV surgery include hemodynamically significant TrV regurgitation, and TrV dysplasia.(10,12,21) Russell et al(22) reported a threefold increase in-hospital mortality in those undergoing concomitant TrV surgeries, whereas other reports have not.(12,13) Consistent with other studies,(21,22) concomitant TrV surgery at index operation also impacted late survival in our cohort suggesting an adverse cumulative burden of evolving TrV disease. However, in contrast, Naimo et al(12) reported no adverse late outcomes among patients undergoing concomitant TrV surgery during single-stage TAC repair, but this may be limited by single center and small sample size. Intervening on TrV is driven by clinical factors, and practitioners would agree that severe TrV regurgitation should be addressed at time of TAC repair. However, there is no consensus for moderate TrV regurgitation. Our results suggest intervening on moderate TrV disease may not improve outcomes. As TrV regurgitation often diminishes after the volume load is reduced, deferring intervening on TrV regurgitation to a later time, may be preferable as improved maturity of the valve tissue and better choices for valve replacement may improve outcomes.(23)
Regarding concomitant IAA surgery, previous reports are conflicting.(8,9,22) A previous multi-institutional study on the TAC-IAA outcomes reported by the Congenital Heart Surgeons Society(9), comprised of 50 patients showed an early mortality of 47%. However, our results agree with other studies(7,8,10), showing no differential in-hospital or long-term mortality in this group and provides continued reassurance that combining IAA surgery with TAC repair is justified.
In concordance with other studies, we found that TAC patients often require RV outflow tract reintervention.(24, 25) We found a trend of a RV-PA conduit/BSA ≥2.5 SD being related to greater risk of in-hospital death similar to other reports but this association attenuated with adjustment of center characteristics. Conduit relative to body size was also related to time-to-reintervention in our study. Smaller conduits lead to earlier reinterventions, while larger conduits may be able to delay reintervention.(11,20,25)
There are several limitations to this study. First, the retrospective and registry-based nature of the dataset limits the use of variables to those available within the registry. That includes details of operative techniques and post-surgical care as well as variables (degree of TrV regurgitation) that were assigned by centers leading to possible misclassification. In addition, the lack of information regarding residual functionality of the TrV after an attempted repair limits our ability to adjust the post-surgical outcomes. Availability of reintervention data limited to the first five years after initial repair may underestimate our assessment of reinterventions. Given the multifactorial nature of mortality, our ability to identify an underlying pathophysiological reason leading to mortality was limited to that listed on the death certificate and details surrounding any specific event leading to death were not available. Incomplete linkage to NDI precluded the assessment of long-term outcomes in the more recent era. Although technical innovation for repair of TAC has not evolved much over decades; diagnostic evaluation, surgical techniques, and medical care continuously improve which may change survival for current patients relative to even the most recent surgeries in our cohort.
In conclusion, despite prevailing high operative risk, this large, multi-institutional cohort of TAC patients with single-stage repair, demonstrates a 78% 30-year post-discharge survival and up to 87% conditioning surviving the first-year post repair. Concomitant IAA surgery did not affect in-hospital nor long-term survival. TrV surgery at index procedure was the only potentially modifiable factor increasing in-hospital and post-hospital discharge risk for death. Careful patient selection and timing consideration for TrV intervention along with the adoption of current improved surgical valve restoration techniques may offer opportunities to improve outcomes of patients with TAC and TrV disease.
Supplementary Material
Acknowledgments
The authors thank the PCCC centers, program directors, and data coordinators.
Funding:
NHLBI (R01 HL122392), Department of Defense (PR180683), Sarah Morrison Medical Student Research Grant, University of Missouri Kansas City School of Medicine.
ABBREVIATIONS
- aHR
Adjusted hazard ratio
- aRR
Adjusted risk ratio
- BSA
Body surface area
- CAA
Coronary artery abnormality
- CI
Confidence interval
- DGS
DiGeorge Syndrome
- ECMO
Extra corporeal membrane oxygenation
- HIPAA
Health insurance portability and accountability act
- IAA
Interrupted aortic arch
- IQR
Interquartile range
- NDI
National death index
- PA
Pulmonary artery
- PCCC
Pediatric cardiac care consortium
- RV
Right ventricle
- SD
Standard deviation
- TAC
Truncus arteriosus communis
- TrV
Truncal valve
Footnotes
Declaration of interests
The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
Conflict of interest: No conflicts to declare.
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REFERENCES
- 1.Collett RW, Edwards JE. Persistent truncus arteriosus; a classification according to anatomic types. Surg Clin North Am. 1949;29(4):1245–70. [DOI] [PubMed] [Google Scholar]
- 2.Alsoufi B, McCracken C, Shashidharan S, Deshpande S, Kanter K, Kogon B. The Impact of 22q11.2 Deletion Syndrome on Surgical Repair Outcomes of Conotruncal Cardiac Anomalies. Ann Thorac Surg. 2017;104(5):1597–604. [DOI] [PubMed] [Google Scholar]
- 3.Kumar SR, Gaynor JW, Jones LA, et al. The Society of Thoracic Surgeons Congenital Heart Surgery Database: 2022 Update on Outcomes and Quality. Ann Thorac Surg. 2023;115(4):807–819. [DOI] [PubMed] [Google Scholar]
- 4.Asagai S, Inai K, Shinohara T, et al. Long-term Outcomes after Truncus Arteriosus Repair: A Single-center Experience for More than 40 Years. Congenit Heart Dis. 2016;11(6):672–7. [DOI] [PubMed] [Google Scholar]
- 5.Bohuta L, Hussein A, Fricke TA, et al. Surgical repair of truncus arteriosus associated with interrupted aortic arch: long-term outcomes. Ann Thorac Surg. 2011;91(5):1473–7. [DOI] [PubMed] [Google Scholar]
- 6.Buckley JR, Amula V, Sassalos P, et al. Multicenter Analysis of Early Childhood Outcomes After Repair of Truncus Arteriosus. Ann Thorac Surg. 2019;107(2):553–9. [DOI] [PubMed] [Google Scholar]
- 7.Ivanov Y, Mykychak Y, Fedevych O, Motrechko O, Kurkevych A, Yemets I. Single-centre 20-year experience with repair of truncus arteriosus. Interact Cardiovasc Thorac Surg. 2019. Jul 1;29(1):93–100. [DOI] [PubMed] [Google Scholar]
- 8.Naimo PS, Konstantinov IE. Surgery for Truncus Arteriosus: Contemporary Practice. Ann Thorac Surg. 2021. May;111(5):1442. [DOI] [PubMed] [Google Scholar]
- 9.Konstantinov IE, Karamlou T, Blackstone EH, et al. Truncus arteriosus associated with interrupted aortic arch in 50 neonates: a Congenital Heart Surgeons Society study. Ann Thorac Surg. 2006;81(1):214–22. [DOI] [PubMed] [Google Scholar]
- 10.Naimo PS, Bell D, Fricke TA, et al. Truncus arteriosus repair: A 40-year multicenter perspective. J Thorac Cardiovasc Surg. 2020;S0022–5223(20). [DOI] [PubMed] [Google Scholar]
- 11.Mastropietro CW, Amula V, Sassalos P, et al. Characteristics and operative outcomes for children undergoing repair of truncus arteriosus: A contemporary multicenter analysis. J Thorac Cardiovasc Surg. 2019;157(6):2386–98. [DOI] [PubMed] [Google Scholar]
- 12.Naimo PS, Fricke TA, d’Udekem Y, et al. Impact of truncal valve surgery on the outcomes of the truncus arteriosus repair. Eur J Cardiothorac Surg. 2018;54(3):524–31. [DOI] [PubMed] [Google Scholar]
- 13.Bakar AM, Costello JM, Sassalos P, et al. Multicenter Analysis of Truncal Valve Management and Outcomes in Children with Truncus Arteriosus. Pediatr Cardiol. 2020;41(7):1473–83. [DOI] [PubMed] [Google Scholar]
- 14.Costeff H A simple empirical formula for calculating approximate surface area in children. Arch Dis Child. 1966;41(220):681–3. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 15.Bonilla-Ramirez C, Ibarra C, Binsalamah ZM, et al. Right Ventricle to Pulmonary Artery Conduit Size Is Associated with Conduit and Pulmonary Artery Reinterventions After Truncus Arteriosus Repair. Semin Thorac Cardiovasc Surg. 2021. [DOI] [PubMed] [Google Scholar]
- 16.Pettersen MD, Du W, Skeens ME, Humes RA. Regression equations for calculation of z scores of cardiac structures in a large cohort of healthy infants, children, and adolescents: an echocardiographic study. J Am Soc Echocardiogr. 2008;21(8):922–34. [DOI] [PubMed] [Google Scholar]
- 17.Karamlou T, Blackstone EH, Hawkins JA, et al. Can pulmonary conduit dysfunction and failure be reduced in infants and children less than age 2 years at initial implantation? J Thorac Cardiovasc Surg. 2006;132(4):829–38. [DOI] [PubMed] [Google Scholar]
- 18.Spector LG, Menk JS, Knight JH, et al. Trends in Long-Term Mortality After Congenital Heart Surgery. J Am Coll Cardiol. 2018;71(21):2434–46. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 19.McCracken C, Spector LG, Menk JS, et al. Mortality Following Pediatric Congenital Heart Surgery: An Analysis of the Causes of Death Derived From the National Death Index. J Am Heart Assoc. 2018;7(22):e010624. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 20.Rajasinghe HA, McElhinney DB, Reddy VM, Mora BN, Hanley FL. Long-term follow-up of truncus arteriosus repaired in infancy: a twenty-year experience. J Thorac Cardiovasc Surg. 1997;113(5):869–78. [DOI] [PubMed] [Google Scholar]
- 21.Gellis L, Binney G, Alshawabkeh L, et al. Long-Term Fate of the Truncal Valve. J Am Heart Assoc. 2020;9(22):e019104. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 22.Russell HM, Pasquali SK, Jacobs JP, et al. Outcomes of repair of common arterial trunk with truncal valve surgery: a review of the society of thoracic surgeons congenital heart surgery database. Ann Thorac Surg. 2012;93(1):164–9. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 23.Javier Delmo EM, Hetzer R. Mitral valve surgery in infants and children. Transl Pediatr. 2020;9(2):187–90. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 24.McElhinney DB, Rajasinghe HA, Mora BN, Reddy VM, Silverman NH, Hanley FL. Reinterventions after repair of common arterial trunk in neonates and young infants. J Am Coll Cardiol. 2000;35(5):1317–22. [DOI] [PubMed] [Google Scholar]
- 25.Poynter JA, Eghtesady P, McCrindle BW, et al. Association of pulmonary conduit type and size with durability in infants and young children. Ann Thorac Surg. 2013;96(5):1695–701. [DOI] [PubMed] [Google Scholar]
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