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.
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: Wai Lun Alan Fung; Eva W C Chow; Gary D Webb; Michael A Gatzoulis; Anne S Bassett Journal: Int J Cardiol Date: 2008-01-11 Impact factor: 4.164
Authors: Nihal M Batouty; Donia M Sobh; Basma Gadelhak; Hoda M Sobh; Walaa Mahmoud; Ahmed M Tawfik Journal: Radiol Med Date: 2019-12-10 Impact factor: 3.469