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

详细信息    查看全文

• 作者：Pieter G. Postema ; Lukas A.J. Rammeloo ; Raphaele van Litsenburg ; Ellen G.M. Rothuis ; Jaroslav Hruda
• 关键词：Congenital ; hereditary ; and neonatal diseases and abnormalities ; Echocardiography ; Heart defects ; congenital ; Pediatrics ; Veins
• 刊名：International Journal of Cardiology
• 出版年：2008
• 出版时间：24 January 2008
• 年：2008
• 卷：123
• 期：3
• 页码：302-306
• 全文大小：112 K

文摘

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.