title: Pulmonary outflow obstruction protects against heart failure in adults with congenitally corrected transposition of the great arteries.
abstract: BACKGROUND
  Pulmonary outflow tract obstruction (POTO) reduces systemic atrioventricular valve (SAVV) regurgitation severity in congenitally corrected transposition of the great arteries (ccTGA). Therefore, pulmonary artery banding is proposed as a palliative intervention. We aimed to investigate the effect of native or surgically induced POTO on event-free survival, defined as the composite of all-cause mortality, heart transplantation, or congestive heart failure (CHF).
  
  
  RESULTS
  Patients with ccTGA (n=62; median age 27.5 (IQR 18.4-39.4) years; 39% with POTO) were selected from the Adult Congenital Heart Disease database of a tertiary hospital. At first visit, SAVV regurgitation ≥ 3/4, systemic RV dysfunction ≥ moderate, and CHF were present in 26%, 26%, and 15% of patients, respectively. Over a mean follow-up time of 10.1 ± 6.1 years, all-cause mortality, rate of heart transplantation, and CHF were 18%, 8% and 40%, respectively. SAVV regurgitation (HR: 1.99; 95% CI: 1.01-3.92; P=0.048) and systemic RV dysfunction severity (HR: 1.89; 95% CI: 1.05-3.37; P=0.033) were associated with the composite endpoint, independently of age at baseline, POTO, Ebstein-like malformation, and systemic RV dilatation. Patients with POTO had lower risk for developing SAVV regurgitation ≥ 3/4 (HR: 0.18; 95% CI: 0.05-0.58; P=0.004) and moderate systemic RV dysfunction (HR: 0.34; 95% CI: 0.15-0.78; P=0.011). When POTO was present, the mean progression-free interval for the composite endpoint increased from 11.2 to 18.1 years (P=0.035).
  
  
  CONCLUSIONS
  POTO is associated with an improved event-free survival in adults with ccTGA.
authors.affiliation: Division of Cardiology, Department of Cardiovascular Sciences, KU Leuven - University of Leuven, B-3000 Leuven, Belgium; Department of Cardiology, University Hospitals Leuven, B-3000 Leuven, Belgium.
authors.forename: Frederik
authors.initials: F
authors.lastname: Helsen
authors.affiliation: Division of Cardiology, Department of Cardiovascular Sciences, KU Leuven - University of Leuven, B-3000 Leuven, Belgium; Department of Cardiology, University Hospitals Leuven, B-3000 Leuven, Belgium.
authors.forename: Pieter
authors.initials: P
authors.lastname: De Meester
authors.affiliation: Department of Cardiology, University Hospitals Leuven, B-3000 Leuven, Belgium.
authors.forename: Jan
authors.initials: J
authors.lastname: Van Keer
authors.affiliation: Division of Cardiology, Department of Cardiovascular Sciences, KU Leuven - University of Leuven, B-3000 Leuven, Belgium; Department of Cardiology, University Hospitals Leuven, B-3000 Leuven, Belgium.
authors.forename: Charlien
authors.initials: C
authors.lastname: Gabriels
authors.affiliation: Department of Cardiology, University Hospitals Leuven, B-3000 Leuven, Belgium.
authors.forename: Alexander
authors.initials: A
authors.lastname: Van De Bruaene
authors.affiliation: Division of Experimental Cardiac Surgery, Department of Cardiovascular Sciences, KU Leuven - University of Leuven, B-3000 Leuven, Belgium; Department of Cardiac Surgery, University Hospitals Leuven, B-3000 Leuven, Belgium.
authors.forename: Paul
authors.initials: P
authors.lastname: Herijgers
authors.affiliation: Department of Cardiac Surgery, University Hospitals Leuven, B-3000 Leuven, Belgium; Division of Clinical Cardiac Surgery, Department of Cardiovascular Sciences, KU Leuven - University of Leuven, B-3000 Leuven, Belgium.
authors.forename: Filip
authors.initials: F
authors.lastname: Rega
authors.affiliation: Department of Cardiac Surgery, University Hospitals Leuven, B-3000 Leuven, Belgium; Division of Clinical Cardiac Surgery, Department of Cardiovascular Sciences, KU Leuven - University of Leuven, B-3000 Leuven, Belgium.
authors.forename: Bart
authors.initials: B
authors.lastname: Meyns
authors.affiliation: Division of Cardiovascular Developmental Biology, Department of Cardiovascular Sciences, KU Leuven - University of Leuven, B-3000 Leuven, Belgium; Department of Paediatrics, University Hospitals Leuven, B-3000 Leuven, Belgium.
authors.forename: Marc
authors.initials: M
authors.lastname: Gewillig
authors.affiliation: Department of Cardiology, University Hospitals Leuven, B-3000 Leuven, Belgium.
authors.forename: Els
authors.initials: E
authors.lastname: Troost
authors.affiliation: Division of Cardiology, Department of Cardiovascular Sciences, KU Leuven - University of Leuven, B-3000 Leuven, Belgium; Department of Cardiology, University Hospitals Leuven, B-3000 Leuven, Belgium. Electronic address: werner.budts@uzleuven.be.
authors.forename: Werner
authors.initials: W
authors.lastname: Budts
mesh_terms: D000293:Adolescent; D000328:Adult; D016208:Databases, Factual; D018572:Disease-Free Survival; D005260:Female; D006333:Heart Failure / Q000517:prevention & control*; D016027:Heart Transplantation / Q000706:statistics & numerical data; D006801:Humans; D008297:Male; D011651:Pulmonary Artery / Q000601:surgery*; D012189:Retrospective Studies; D015996:Survival Rate; D014188:Transposition of Great Vessels / Q000150:complications* / Q000401:mortality; D014262:Tricuspid Valve Insufficiency / Q000601:surgery*; D014694:Ventricular Outflow Obstruction / Q000209:etiology*; D055815:Young Adult
keywords: Congenital heart defects; Heart failure; Survival; Transposition of great vessels; Tricuspid valve insufficiency