Left superior vena cava in pediatric cardiology associated with extra-cardiac anomalies

Int J Cardiol. 2008 Jan 24;123(3):302-6. doi: 10.1016/j.ijcard.2006.12.020. Epub 2007 Mar 27.


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.

MeSH terms

  • Abnormalities, Multiple / diagnosis
  • Abnormalities, Multiple / diagnostic imaging*
  • Abnormalities, Multiple / epidemiology*
  • Adolescent
  • Age Distribution
  • Child
  • Child, Preschool
  • Cohort Studies
  • Comorbidity
  • Echocardiography, Transesophageal
  • Female
  • Heart Defects, Congenital / diagnosis
  • Heart Defects, Congenital / diagnostic imaging*
  • Heart Defects, Congenital / epidemiology*
  • Humans
  • Incidence
  • Infant
  • Infant, Newborn
  • Male
  • Netherlands / epidemiology
  • Odds Ratio
  • Probability
  • Retrospective Studies
  • Risk Assessment
  • Sex Distribution
  • Survival Rate
  • Vena Cava, Superior / abnormalities*