title: Cardiovascular magnetic resonance parameters associated with early transplant-free survival in children with small left hearts following conversion from a univentricular to biventricular circulation.
abstract: BACKGROUND
  We sought to identify cardiovascular magnetic resonance (CMR) parameters associated with successful univentricular to biventricular conversion in patients with small left hearts.
  
  
  METHODS
  Patients with small left heart structures and a univentricular circulation who underwent CMR prior to biventricular conversion were retrospectively identified and divided into 2 anatomic groups: 1) borderline hypoplastic left heart structures (BHLHS), and 2) right-dominant atrioventricular canal (RDAVC). The primary outcome variable was transplant-free survival with a biventricular circulation.
  
  
  RESULTS
  In the BHLHS group (n = 22), 16 patients (73%) survived with a biventricular circulation over a median follow-up of 40 months (4-84). Survival was associated with a larger CMR left ventricular (LV) end-diastolic volume (EDV) (p = 0.001), higher LV-to-right ventricle (RV) stroke volume ratio (p < 0.001), and higher mitral-to-tricuspid inflow ratio (p = 0.04). For predicting biventricular survival, the addition of CMR threshold values to echocardiographic LV EDV improved sensitivity from 75% to 93% while maintaining specificity at 100%. In the RDAVC group (n = 10), 9 patients (90%) survived with a biventricular circulation over a median follow-up of 29 months (3-51). The minimum CMR values were a LV EDV of 22 ml/m² and a LV-to-RV stroke volume ratio of 0.19.
  
  
  CONCLUSIONS
  In BHLHS patients, a larger LV EDV, LV-to-RV stroke volume ratio, and mitral-to-tricuspid inflow ratio were associated with successful biventricular conversion. The addition of CMR parameters to echocardiographic measurements improved the sensitivity for predicting successful conversion. In RDAVC patients, the high success rate precluded discriminant analysis, but a range of CMR parameters permitting biventricular conversion were identified.
authors.affiliation: Department of Cardiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02115, USA. puja.banka@cardio.chboston.org.|Department of Pediatrics, Harvard Medical School, Boston, MA, USA. puja.banka@cardio.chboston.org.
authors.forename: Puja
authors.initials: P
authors.lastname: Banka
authors.affiliation: Department of Cardiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02115, USA. barbara.schaetzle@hotmail.com.|Department of Pediatrics, Harvard Medical School, Boston, MA, USA. barbara.schaetzle@hotmail.com.|Current address: Kantonsspital Winterthur, Winterthur, Switzerland. barbara.schaetzle@hotmail.com.
authors.forename: Barbara
authors.initials: B
authors.lastname: Schaetzle
authors.affiliation: Department of Cardiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02115, USA. rukmini.komarlu@cardio.chboston.org.|Department of Pediatrics, Harvard Medical School, Boston, MA, USA. rukmini.komarlu@cardio.chboston.org.|Current address: Department of Pediatric Cardiology, Cleveland Clinic, Cleveland, OH, USA. rukmini.komarlu@cardio.chboston.org.
authors.forename: Rukmini
authors.initials: R
authors.lastname: Komarlu
authors.affiliation: Department of Cardiac Surgery, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02115, USA. sitaram.emani@cardio.chboston.org.|Department of Surgery, Harvard Medical School, Boston, MA, USA. sitaram.emani@cardio.chboston.org.
authors.forename: Sitaram
authors.initials: S
authors.lastname: Emani
authors.affiliation: Department of Cardiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02115, USA. tal.geva@cardio.chboston.org.|Department of Pediatrics, Harvard Medical School, Boston, MA, USA. tal.geva@cardio.chboston.org.
authors.forename: Tal
authors.initials: T
authors.lastname: Geva
authors.affiliation: Department of Cardiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02115, USA. andrew.powell@cardio.chboston.org.|Department of Pediatrics, Harvard Medical School, Boston, MA, USA. andrew.powell@cardio.chboston.org.
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Powell
mesh_terms: D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D003287:Contrast Media; D018572:Disease-Free Survival; D004694:Endocardial Cushion Defects / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D006343:Heart Septal Defects; D016027:Heart Transplantation; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D008943:Mitral Valve / Q000503:physiopathology; D011237:Predictive Value of Tests; D020127:Recovery of Function; D012189:Retrospective Studies; D012307:Risk Factors; D013318:Stroke Volume; D013997:Time Factors; D016896:Treatment Outcome; D014261:Tricuspid Valve / Q000503:physiopathology; D016277:Ventricular Function, Left; D016278:Ventricular Function, Right
chemical_list: D003287:Contrast Media