Characteristics and operative outcomes for children undergoing repair of truncus arteriosus: A contemporary multicenter analysis Christopher W. Mastropietro,

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Characteristics and operative outcomes for children undergoing repair of truncus arteriosus: A contemporary multicenter analysis Christopher W. Mastropietro, MD, FCCM, Venu Amula, MD, Peter Sassalos, MD, Jason R. Buckley, MD, Arthur J. Smerling, MD, Ilias Iliopoulos, MD, Christine M. Riley, CPNP-AC, Aimee Jennings, PNP-AC/PC, Katherine Cashen, DO, Sukumar Suguna Narasimhulu, MD, MPH, Keshava Murty Narayana Gowda, MBBS, Adnan M. Bakar, MD, Michael Wilhelm, MD, Aditya Badheka, MD, Elizabeth A.S. Moser, MS, John M. Costello, MD, MPH The Journal of Thoracic and Cardiovascular Surgery Volume 157, Issue 6, Pages 2386-2398.e4 (June 2019) DOI: 10.1016/j.jtcvs.2018.12.115 Copyright © 2019 The American Association for Thoracic Surgery Terms and Conditions

Figure 1 Variation 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. The Journal of Thoracic and Cardiovascular Surgery 2019 157, 2386-2398.e4DOI: (10.1016/j.jtcvs.2018.12.115) Copyright © 2019 The American Association for Thoracic Surgery Terms and Conditions

Figure 2 Major 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 Journal of Thoracic and Cardiovascular Surgery 2019 157, 2386-2398.e4DOI: (10.1016/j.jtcvs.2018.12.115) Copyright © 2019 The American Association for Thoracic Surgery Terms and Conditions

Figure 3 A, 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. The Journal of Thoracic and Cardiovascular Surgery 2019 157, 2386-2398.e4DOI: (10.1016/j.jtcvs.2018.12.115) Copyright © 2019 The American Association for Thoracic Surgery Terms and Conditions

Figure E1 Histogram 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). The Journal of Thoracic and Cardiovascular Surgery 2019 157, 2386-2398.e4DOI: (10.1016/j.jtcvs.2018.12.115) Copyright © 2019 The American Association for Thoracic Surgery Terms and Conditions

Figure E2 Trends 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). The Journal of Thoracic and Cardiovascular Surgery 2019 157, 2386-2398.e4DOI: (10.1016/j.jtcvs.2018.12.115) Copyright © 2019 The American Association for Thoracic Surgery Terms and Conditions

Figure E3 Absolute 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. The Journal of Thoracic and Cardiovascular Surgery 2019 157, 2386-2398.e4DOI: (10.1016/j.jtcvs.2018.12.115) Copyright © 2019 The American Association for Thoracic Surgery Terms and Conditions

Modifiable risk factors for major adverse cardiac events after truncus arteriosus repair. The Journal of Thoracic and Cardiovascular Surgery 2019 157, 2386-2398.e4DOI: (10.1016/j.jtcvs.2018.12.115) Copyright © 2019 The American Association for Thoracic Surgery Terms and Conditions

The Journal of Thoracic and Cardiovascular Surgery 2019 157, 2386-2398 The Journal of Thoracic and Cardiovascular Surgery 2019 157, 2386-2398.e4DOI: (10.1016/j.jtcvs.2018.12.115) Copyright © 2019 The American Association for Thoracic Surgery Terms and Conditions