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: Pieter G Postema, Lukas A J Rammeloo, Raphaele van Litsenburg, Ellen G M Rothuis, Jaroslav Hruda
• Journal: International journal of cardiology (Int J Cardiol) Vol. 123 Issue 3 Pg. 302-6 (Jan 24 2008) ISSN: 1874-1754 [Electronic] Netherlands
• PMID: 17391785 (Publication Type: Journal Article)

Topics

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