title: Characteristics and outcomes of double outlet left ventricle.
abstract: BACKGROUND
  Double outlet left ventricle (DOLV) is a rare cardiac malformation in which both great arteries originate from the morphological left ventricle. There are limited data regarding the anatomical spectrum, characteristics and outcomes of DOLV.
  
  
  METHODS
  All patients with DOLV between 1960 and 2008 were identified. Patients' presentation, anatomy, surgical history, and outcomes were recorded. Kaplan-Meier estimates were used to describe time-related survival and freedom from surgical intervention.
  
  
  RESULTS
  There were 19 patients with DOLV during the study period, 14 of whom were male (74%). Median age at presentation was 2 days (birth--5.5 years). The most prominent associated cardiac morphologies (in addition to DOLV) were atrial septal defects (14, 74%), ventricular septal defects (13, 68%), right ventricular hypoplasia (12, 63%), and patent ductus arteriosus (15, 80%). At presentation, nine patients (47%) were cyanosed, nine (42%) had cardiomegaly, and all had some degree of right ventricular dysfunction. Of the 19 patients, seven underwent biventricular repairs, nine underwent univentricular palliations, one had only palliative care, and two were seen for consultation only. There were six deaths in this series, five were cardiac in nature (one due to genetic syndrome). Five-year survival after presentation for both biventricular and univentricular repairs was 70-75% (P= 0.80). Most surviving patients had residual cardiac abnormalities at last follow-up despite surgical repair.
  
  
  CONCLUSIONS
  DOLV is a rare cardiac malformation associated with high mortality and guarded outcomes from residual lesions.
authors.affiliation: Department of Pediatrics, Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Carolin
authors.initials: C
authors.lastname: Imai-Compton
authors.forename: Maryam
authors.initials: M
authors.lastname: Elmi
authors.forename: Cedric
authors.initials: C
authors.lastname: Manlhiot
authors.forename: Alejandro A
authors.initials: AA
authors.lastname: Floh
authors.forename: Fraser
authors.initials: F
authors.lastname: Golding
authors.forename: William G
authors.initials: WG
authors.lastname: Williams
authors.forename: Brian W
authors.initials: BW
authors.lastname: McCrindle
mesh_terms: D000015:Abnormalities, Multiple; D001011:Aorta / Q000002:abnormalities* / Q000601:surgery; D006348:Cardiac Surgical Procedures; D002648:Child; D002675:Child, Preschool; D005260:Female; D006330:Heart Defects, Congenital / Q000401:mortality / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D006352:Heart Ventricles / Q000002:abnormalities* / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D009864:Ontario; D010166:Palliative Care; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery; D012189:Retrospective Studies; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome