title: Left superior vena cava in pediatric cardiology associated with extra-cardiac anomalies.
abstract: BACKGROUND
  In case reports and small series, the coexistence of a persistent left superior vena cava (LSVC) and extra-cardiac anomalies has been noted. However, an association between LSVC and extra-cardiac anomalies has not been documented. We investigated the association between LSVC and extra-cardiac anomalies in patients referred to our tertiary pediatric cardiology department between 1998 and 2005.
  
  
  METHODS
  Trans-thoracic echocardiograms were performed on 4426 consecutive patients. Cardiac and extra-cardiac anomalies were registered prospectively in a computerized database. In a retrospective observational design, characteristics of patients with LSVC were collected.
  
  
  RESULTS
  In 4426 patients, 1825 (41%) were diagnosed with congenital heart disease (CHD) and 295 patients (7%) with extra-cardiac anomalies. LSVC was present in 102 patients, of which 89 (87%) with CHD (OR 10.2, 95% CI 5.7 to 18.3, p<0.001) and 61 (60%) with extra-cardiac anomalies (OR 26.0, 95% CI 17.1 to 39.5, p<0.001). Confirmed syndromes were present in 43 LSVC patients (42%), including VACTERL association (vertebral defects, anal atresia, cardiac malformations, tracheo-esophageal fistula with esophageal atresia, radial and renal dysplasia, and limb anomalies, 9%), trisomy 21 (7%), 22q11 (6%) and CHARGE association (coloboma, heart defects, atresia of choanae, retardation, genital and ear anomalies, 5%). In 17 LSVC patients (17%) with multiple anomalies in different organ systems, a syndrome diagnosis was not confirmed.
  
  
  CONCLUSIONS
  The LSVC appears to be indicative for both cardiac and extra-cardiac anomalies (e.g. septal defects, tetralogy of Fallot, VACTERL and CHARGE association). Disorders in the development of the secondary heart field may be causal to this combination of anomalies.
authors: Department of Pediatric Cardiology, VU University Medical Center, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands.
authors: Pieter G
authors: PG
authors: Postema
authors: Lukas A J
authors: LA
authors: Rammeloo
authors: Raphaele
authors: R
authors: van Litsenburg
authors: Ellen G M
authors: EG
authors: Rothuis
authors: Jaroslav
authors: J
authors: Hruda
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D000293:Adolescent; D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D015897:Comorbidity; D017548:Echocardiography, Transesophageal; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009426:Netherlands / Q000453:epidemiology; D016017:Odds Ratio; D011336:Probability; D012189:Retrospective Studies; D018570:Risk Assessment; D017678:Sex Distribution; D015996:Survival Rate; D014683:Vena Cava, Superior / Q000002:abnormalities*