title: Outcomes of Truncus Arteriosus Repair in Children: 35 Years of Experience From a Single Institution.
abstract: We evaluated the long-term outcomes following repair of truncus arteriosus (TA) from a single institution. We conducted a retrospective review of children (n = 171) who underwent TA repair between 1979 and 2014. Early mortality rate was 11.7% (20/171). There were 19 late deaths. Most deaths (74%, 29/39) occurred within the first year following surgery. The 1-year mortality rate in 1979-2004 was 18% (25/136) and decreased to 11% (4/35) in 2005-2014. The overall survival rate was 73.6% at 30 years. Multivariate analysis identified postoperative extracorporeal membrane oxygenation (P = 0.003), operative weight <2.5kg (P = 0.012), prior surgical intervention (P = 0.018), and coronary artery anomaly (P = 0.037) as risk factors for early mortality. A Cox regression model identified DiGeorge syndrome (P = 0.008) as a risk factor for late mortality. Freedom from right ventricular outflow tract reoperation was 4.6% at 20 years. Concomitant truncal valve (TV) repair or replacement was undertaken in 20 patients. Additionally, 14 patients underwent late TV repair or replacement. The overall survival rate in patients who underwent TV operation was 76.9% at 20 years. A total of 19 patients had concomitant interrupted aortic arch with a survival rate of 89.5% at 20 years. Median follow-up was 19 years (mean = 17 years, range: 1-34 years). All patients were in New York Heart Association Class I/II at last follow-up. Following repair of TA, patients had good long-term functional status but had high reoperation rates. Repair of interrupted aortic arch and TV were not risk factors for mortality. Postoperative extracorporeal membrane oxygenation, operative weight <2.5kg, prior surgical intervention, and coronary artery anomaly were risk factors for early death. DiGeorge syndrome was associated with late death, most commonly from infection.
authors.affiliation: Department of Cardiac Surgery, Royal Children׳s Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia.
authors.forename: Phillip S
authors.initials: PS
authors.lastname: Naimo
authors.affiliation: Department of Cardiac Surgery, Royal Children׳s Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia.
authors.forename: Tyson A
authors.initials: TA
authors.lastname: Fricke
authors.affiliation: Department of Cardiac Surgery, Royal Children׳s Hospital, Melbourne, Australia.
authors.forename: Matthew S
authors.initials: MS
authors.lastname: Yong
authors.affiliation: Department of Cardiac Surgery, Royal Children׳s Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia; Murdoch Children׳s Research Institute, Melbourne, Australia.
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
authors.affiliation: Department of Cardiology, Women׳s and Children׳s Hospital, Adelaide, Australia.
authors.forename: Andrew
authors.initials: A
authors.lastname: Kelly
authors.affiliation: Department of Cardiology, The Prince Charles Hospital, University of Queensland, Brisbane, Australia.
authors.forename: Dorothy J
authors.initials: DJ
authors.lastname: Radford
authors.affiliation: Department of Cardiology, Princess Margaret Hospital for Children, Perth, Australia.
authors.forename: Andrew
authors.initials: A
authors.lastname: Bullock
authors.affiliation: Department of Cardiac Surgery, Royal Children׳s Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia; Murdoch Children׳s Research Institute, Melbourne, Australia.
authors.forename: Robert G
authors.initials: RG
authors.lastname: Weintraub
authors.affiliation: Department of Cardiac Surgery, Royal Children׳s Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia; Murdoch Children׳s Research Institute, Melbourne, Australia.
authors.forename: Christian P
authors.initials: CP
authors.lastname: Brizard
authors.affiliation: Department of Cardiac Surgery, Royal Children׳s Hospital, Melbourne, Australia; University of Melbourne, Melbourne, Australia; Murdoch Children׳s Research Institute, Melbourne, Australia. Electronic address: igor.konstantinov@rch.org.au.
authors.forename: Igor E
authors.initials: IE
authors.lastname: Konstantinov
mesh_terms: D001724:Birth Weight; D001835:Body Weight; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D003330:Coronary Vessel Anomalies / Q000401:mortality; D004062:DiGeorge Syndrome / Q000401:mortality; D018572:Disease-Free Survival; D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D016015:Logistic Models; D008297:Male; D015999:Multivariate Analysis; D016017:Odds Ratio; D011183:Postoperative Complications / Q000209:etiology / Q000401:mortality / Q000601:surgery; D016016:Proportional Hazards Models; D012086:Reoperation; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D014338:Truncus Arteriosus / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery*; D014739:Victoria
keywords: common arterial trunk; congenital heart disease; conotruncal; surgery; truncus arteriosus