title: The natural and unnatural history of the Mustard procedure: long-term outcome up to 40 years.
abstract: OBJECTIVE
  To describe long-term survival, clinical outcome and ventricular systolic function in a longitudinally followed cohort of patients after Mustard repair for transposition of the great arteries (TGA). There is serious concern about the long-term outcome after Mustard repair.
  
  
  RESULTS
  This longitudinal single-centre study consisted of 91 consecutive patients, who underwent Mustard repair before 1980, at age <15 years, and were evaluated in-hospital every 10 years. Survival status was obtained of 86 patients. Median follow-up was 35 (IQR 34-38) years. Cumulative survival was 84% after 10 years, 80% after 20 years, 77% after 30 years, and 68% after 39 years. Cumulative survival free of events (i.e. heart transplantation, arrhythmias, reintervention, and heart failure) was 19% after 39 years. Reinterventions were mainly required for baffle-related problems. Supraventricular and ventricular arrhythmias occurred in 28 and 6% of the patients, respectively. Pacemaker and/or ICD implantation was performed in 39%. Fifty survivors participated in the current in-hospital investigation including electrocardiography, 2D-echocardiography, cardiopulmonary-exercise testing, NT-proBNP measurement, Holter monitoring, and cardiac magnetic resonance. Right ventricular systolic function was impaired in all but one patient at last follow-up, and 14% developed heart failure in the last decade. NT-proBNP levels [median 31.6 (IQR 22.3-53.2) pmol/L] were elevated in 92% of the patients. Early postoperative arrhythmias were a predictor for late arrhythmias [HR 3.8 (95% CI 1.5-9.5)], and development of heart failure [HR 8.1 (95% CI 2.2-30.7)]. Also older age at operation was a predictor for heart failure [HR 1.26 (95% CI 1.0-1.6)].
  
  
  CONCLUSIONS
  Long-term survival after Mustard repair is clearly diminished and morbidity is substantial. Early postoperative arrhythmias are a predictor for heart failure and late arrhythmias.
authors.affiliation: Department of Cardiology, Erasmus MC, PO Box 2040, Rotterdam 3015 CE, The Netherlands j.cuypers@erasmusmc.nl.
authors.forename: Judith A A E
authors.initials: JA
authors.lastname: Cuypers
authors.affiliation: Department of Cardiology, Erasmus MC, PO Box 2040, Rotterdam 3015 CE, The Netherlands.
authors.forename: Jannet A
authors.initials: JA
authors.lastname: Eindhoven
authors.affiliation: Department of Cardiology, Erasmus MC, PO Box 2040, Rotterdam 3015 CE, The Netherlands.
authors.forename: Maarten A
authors.initials: MA
authors.lastname: Slager
authors.affiliation: Department of Cardiology, Erasmus MC, PO Box 2040, Rotterdam 3015 CE, The Netherlands.
authors.forename: Petra
authors.initials: P
authors.lastname: Opić
authors.affiliation: Department of Child and Adolescent Psychiatry and Psychology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: Elisabeth M W J
authors.initials: EM
authors.lastname: Utens
authors.affiliation: Department of Pediatrics, Division of Cardiology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: Willem A
authors.initials: WA
authors.lastname: Helbing
authors.affiliation: Department of Cardiology, Erasmus MC, PO Box 2040, Rotterdam 3015 CE, The Netherlands.
authors.forename: Maarten
authors.initials: M
authors.lastname: Witsenburg
authors.affiliation: Department of Cardiology, Erasmus MC, PO Box 2040, Rotterdam 3015 CE, The Netherlands.
authors.forename: Annemien E
authors.initials: AE
authors.lastname: van den Bosch
authors.affiliation: Department of Radiology, Erasmus MC, Rotterdam, The Netherlands.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Ouhlous
authors.affiliation: Department of Cardiology, Erasmus MC, PO Box 2040, Rotterdam 3015 CE, The Netherlands.
authors.forename: Ron T
authors.initials: RT
authors.lastname: van Domburg
authors.affiliation: Department of Biostatistics, Erasmus MC, Rotterdam, The Netherlands.
authors.forename: Dimitris
authors.initials: D
authors.lastname: Rizopoulos
authors.affiliation: Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Folkert J
authors.initials: FJ
authors.lastname: Meijboom
authors.affiliation: Department of Cardio-thoracic Surgery, Erasmus MC, Rotterdam, The Netherlands.
authors.forename: Ad J J C
authors.initials: AJ
authors.lastname: Bogers
authors.affiliation: Department of Cardiology, Erasmus MC, PO Box 2040, Rotterdam 3015 CE, The Netherlands.
authors.forename: Jolien W
authors.initials: JW
authors.lastname: Roos-Hesselink
mesh_terms: D000328:Adult; D001145:Arrhythmias, Cardiac / Q000209:etiology; D015415:Biomarkers / Q000378:metabolism; D006348:Cardiac Surgical Procedures / Q000379:methods* / Q000401:mortality; D002675:Child, Preschool; D004452:Echocardiography; D004562:Electrocardiography; D015716:Electrocardiography, Ambulatory; D004812:Epidemiologic Methods; D005080:Exercise Test / Q000379:methods; D005260:Female; D006333:Heart Failure / Q000209:etiology; D006801:Humans; D007223:Infant; D018810:Magnetic Resonance Angiography; D008297:Male; D020097:Natriuretic Peptide, Brain / Q000378:metabolism; D010446:Peptide Fragments / Q000378:metabolism; D011183:Postoperative Complications / Q000209:etiology; D012086:Reoperation; D014188:Transposition of Great Vessels / Q000401:mortality / Q000601:surgery*; D018497:Ventricular Dysfunction, Right / Q000209:etiology
chemical_list: D015415:Biomarkers; D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain
keywords: Arrhythmias; Mustard repair; RV dysfunction; Survival; Transposition of the great arteries