Left superior vena cava in pediatric cardiology associated with extra-cardiac anomalies.
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.
Postema
Pieter G
PG
Department of Pediatric Cardiology, VU University Medical Center, De Boelelaan 1117, 1081 HV Amsterdam, The Netherlands.
Rammeloo
Lukas A J
LA
van Litsenburg
Raphaele
R
Rothuis
Ellen G M
EG
Hruda
Jaroslav
J
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*