Left superior vena cava in pediatric cardiology associated with extra-cardiac anomalies
Introduction
The persistent left superior vena cava (LSVC) was first recognized by Le Cat in 1738 [1], [2]. This intriguing vein has an estimated prevalence in the general population of 0.1–0.3% and is the most common venous anomaly in humans [3], [4]. In congenital heart disease (CHD) its prevalence is higher, between 3 and 11% [4], [5], [6]. An isolated LSVC is seldom associated with symptoms and may remain undiagnosed [5], [7]. Nevertheless, there are frequent reports on complications, especially when central venous access is required [7], [8], [9], [10], [11], [12], [13], [14], [15], [16].
In case reports and small series, the coexistence of LSVC and extra-cardiac anomalies has been noted [2], [17], [18], [19]. However, an association between LSVC and extra-cardiac anomalies has not been documented in large clinical series. We reviewed the data in our department over a seven year period in order to evaluate the presence of an association between LSVC and extra-cardiac anomalies.
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Materials and methods
Trans-thoracic echocardiograms were performed on 4426 consecutive pediatric patients (45% female), referred to our tertiary pediatric cardiology department between September 1998 and September 2005. The age ranged from birth to 18 years (mean age 2.9 ± 4.3 years). Echocardiograms were carried out using a Vingmed System FiVe echocardiograph (GE Medical Systems, Milwaukee, WI, USA).
Screening for a LSVC has been part of our echocardiography protocol since 1998. From the suprasternal notch approach,
Results
In 4426 patients, 1825 were diagnosed with CHD (41%) and in 295 patients extra-cardiac anomalies were found (7%). LSVC was present in 102 patients (2%, 49% female, age 0.7 ± 2.0 years). Patients with LSVC were considerably younger than patients without LSVC but there was no difference in sex (Table 1). All but one LSVC drained through the coronary sinus into the right atrium, one LSVC drained directly into the left atrium.
Among the 102 LSVC patients, 89 were diagnosed with CHD (87%). The most
Discussion
In the present study, we observed that a LSVC is strongly associated with both cardiac and extra-cardiac anomalies (odds ratios 10.2 and 26.0 respectively). The most common extra-cardiac anomaly was esophageal atresia, diagnosed in 11% of LSVC patients, of which 6 were diagnosed with VACTERL. Conversely, one recent study demonstrated an 8/89, 9.9%, incidence of LSVC in esophageal atresia (number of patients with VACTERL not reported) [21]. In our LSVC patients, we frequently observed
Limitations
The principal limitation of the present study is its retrospective nature. However, our findings during echocardiography were entered prospectively into a computerized database. We did not assess sensitivity and specificity for the diagnosis of LSVC. Assessing sensitivity and specificity would require catheterization, CT or MRI for all consecutive patients. Furthermore, we do not provide new data on the prevalence of LSVC in the general or CHD population. LSVC patients without any associated
Conclusions
A LSVC is more commonly encountered in congenital heart disease than in the general population (e.g. in combination with septal defects or tetralogy of Fallot). The persistence of this vein also appears to be indicative for extra-cardiac anomalies in patients referred to a tertiary pediatric cardiology department, such as in VACTERL or CHARGE association and various genetic syndromes such as trisomy 21 or 22q11. Disorders in the development of the secondary heart field may be causal to this
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