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

doi:10.1016/j.ijcard.2006.12.020

International Journal of Cardiology

Volume 123, Issue 3, 24 January 2008, Pages 302-306

https://doi.org/10.1016/j.ijcard.2006.12.020 Get rights and content

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.

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.

Section snippets

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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Clinical significance of prenatally diagnosed persistent left superior vena cava
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Citation Excerpt :
This dilatation is due to blood flow from the left superior vena cava [6]. In postnatal series, it has been reported that PLSVC is associated with intracardiac and extracardiac anomalies and aneuploidy [7,8]. However, since only symptomatic patients were evaluated in this series, it was concluded that this relationship was more than actual.
The aim of our study was to investigate the persistent left superior vena cava (PLSVC) cases diagnosed in the prenatal period in our clinic and to compare the obstetric and genetic outcomes of isolated PLSVC cases with cases accompanied by other cardiac or extracardiac anomalies.
The cases diagnosed as PLSVC between January 2015 and January 2019 in our perinatology clinic were evaluated retrospectively. Patients were divided into two subgroups as isolated PLSVC and PLSVC accompanied by another anomaly. Furthermore, patients with extra anomalies were divided into three groups which are cardiac anomaly, extracardiac anomaly and those with both. The groups were compared in terms of genetic results and obstetric outcomes.
89 patients were included in our study. Cases with positive pregnancy outcomes were significantly higher in the isolated PLSVC group than with extra anomaly group (p < 0.001). No karyotype anomaly was observed in the isolated group. Pregnancy results were significantly worse (postpartum demise, termination of pregnancy, in utero demise) in with both cardiac and extracardiac anomalies group (p < 0.001). There was no significant difference between the groups in terms of karyotype results (p = 0.535).
The diagnosis of PLSVC has gained importance and it can be made easier due to the fact that anatomic imaging can be performed in more detail. The isolated PLSVC cases have a very good prognosis. Obstetric outcomes vary according to the accompanying anomaly.
Impact of concomitant complex cardiac anatomy in nonsyndromic patients with complete atrioventricular septal defect
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Citation Excerpt :
However, there may be a potential relationship between a persistent LSVC and abnormalities during development in the secondary heart field.15 Postema and colleagues16 demonstrated an association between a persistent LSVC and cardiac and extracardiac anomalies. This variable may be of importance because of the direct effect of the dilated coronary sinus, both as potential restriction of LAVV-annular growth and a deviation of inflow toward the right side of the heart.
We studied a cohort of patients with nonsyndromic complete atrioventricular septal defect with and without concomitant complex cardiac anatomy and compared the outcomes after surgical repair.
Between 1993 and 2018, 62 nonsyndromic patients underwent complete atrioventricular septal defect repair. Sixteen patients (26%) had complex complete atrioventricular septal defect with variables representing concomitant cardiac anatomic complexity: tetralogy of Fallot, double outlet right ventricle, total anomalous pulmonary venous return, concomitant aortic arch reconstruction, multiple ventricular septal defects, staged repair of coarctation of the aorta, and a persisting left superior vena cava. The mean follow-up was 12.7 ± 7.9 years. Baseline variables were retrospectively evaluated and analyzed using univariable logistic regression. Survival was studied using Kaplan–Meier estimates, and group comparisons were performed using the log-rank test. A competing-risk analysis estimated the risk of reoperation with death as the competing event. A Gray's test was used to test equality of the cumulative incidence curves between groups.
The perioperative mortality was 3.2% (2/62). Actuarial survival was 100% versus 66.7% ± 14.9% at 10 years in the noncomplex and complex groups, respectively (P < .01). There was no significant difference in the overall reoperation rate between the noncomplex group (7/46; 15%) and the complex group (4/16; 25%) (odds ratio, 1.86; 95% confidence interval, 0.46-7.45; P = .30). The competing-risk analysis demonstrated no significant difference in reoperation between the groups (P = .28).
Our data show that nonsyndromic patients without complex cardiac anatomy have a good long-term survival and an acceptable risk of reoperation similar to contemporary outcomes for patients with complete atrioventricular septal defect with trisomy 21. However, the corresponding group of nonsyndromic patients with concomitant complex cardiac lesions are still a high-risk population, especially regarding mortality.
Tricuspid Atresia with Persistent Left Superior Vena Cava and Pulmonary Arterial Hypertension. Case Report
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Persistent left superior vena cava with patent left innominate vein: Ideal anatomy for mini invasive thoracoscopic ligature
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Persistent left superior vena cava is the most common congenital anomaly of the thoracic venous system. In most cases, the left vena cava drains into the coronary sinus and remains asymptomatic. When draining in the left atrium, paradoxical embolization could be a severe complication of the right-to-left shunt and is an indication for surgery. We describe the thoracoscopic approach for ligation of a persistent left superior vena cava in a 9 years old girl with VACTERL association and a history of stroke. Bilateral superior vena cava connected by a bridging vein allowed simple ligature of the abnormal systemic venous return.
Prenatal diagnosis of persistent left superior vena cava, polyhydramnios and a small gastric bubble in a fetus with VACTERL association
2021, Taiwanese Journal of Obstetrics and Gynecology
We reported a fetus that presenting with persistent left superior vena cava (PLSVC), polyhydramnios, and a small gastric bubble during prenatal examination and identified VACTERL association after birth.
A 34-year-old woman underwent amniocentesis at 18 weeks of gestation because of advanced maternal age and the result was normal. Subsequently, an ultrasound revealed single umbilical artery (SUA) at 21 weeks of gestation. She received a detailed fetal anatomy survey that presented the same findings and PLSVC. A small visible gastric bubble was noted at that time, and the other organs were unremarkable. Polyhydramnios was identified at 30 weeks of gestation and amnioreduction was subsequently performed at 32 weeks of gestation. However, polyhydramnios was persisted despite amnioreduction and intrauterine growth restriction was also detected. A cesarean section was performed because of fetal distress at 36 + 2 weeks, and a 1832-g female baby was delivered. Pre-axial polydactyly at left thumb, SUA and esophageal atresia with distal tracheoesophageal fistula (TEF) were identified after birth. The neonate died at age of 4 days because of surgical complication following esophageal anastomosis.
Prenatal diagnosis of PLSVC associated with polyhydramnios and a small gastric bubble may indicate esophageal atresia with TEF, and further examination for associated syndromes such as VACTERL association is warranted.
Trans–coronary sinus puncture for catheter ablation and left atrial appendage closure device implantation in a patient with dextrocardia and persistent right superior vena cava
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Left superior vena cava in pediatric cardiology associated with extra-cardiac anomalies

Abstract

Background

Methods

Results

Conclusions

Introduction

Section snippets

Materials and methods

Results

Discussion

Limitations

Conclusions

Ann Thorac Surg

Clin Radiol

Chest

Ann Thorac Surg

Lancet

Am Heart J

J Pediatr Surg

Am J Cardiol

J Am Soc Echocardiogr

Am J Med

J Am Soc Echocardiogr

Dev Biol

Dev Cell

Dev Biol

Am J Cardiol

Three cases of the persistence of the left superior vena cava

Anat Rec

Persistent left superior vena cava and mitral stenosis

Z Gesamte Exp Med

Pathogenesis of persistent left superior vena cava with a coronary sinus connection

Pediatr Pathol

The left-sided superior vena cava

Br Heart J