Original Article
Persistent left superior vena cava: Experience of a tertiary
health-care center
Serdar Kula,
1
Ayhan Cevik,
1
Cihat Sanli,
2
Ayhan Pektas,
1
Fatma Sedef Tunaoglu,
1
Ayse Deniz Oguz
1
and Rana Olgunturk
1
1
Gazi University Faculty of Medicine, Department of Pediatric Cardiology, Besevler, Ankara and
2
Kırıkkale University
Faculty of Medicine, Department of Pediatric Cardiology, Kırıkkale, Turkey
Abstract Background: The aim of this study was to assess the prevalence of persistent left superior vena cava (PLSVC) in
patients with all types of congenital heart defects and to determine the congenital heart anomalies accompanying
PLSVC.
Methods: The present study is based on a retrospective review of 1205 children who consecutively underwent cardiac
catheterization from 2000 to 2007. In order to determine the existence of PLSVC, all the subjects routinely underwent
superior vena cava (SVC) injection during angiography at the catheter laboratory of the study center.
Results: The prevalence of PLSVC was computed to be 6.1% for the present study population. Transthoracic echocar-
diography was able to detect PLSVC in 32 children (2.6%) whereas angiography diagnosed PLSVC in 74 children
(6.1%). The mean age of the patients with PLSVC was 40.09 1 50.21 months. A communication between the right and
left SVC was determined in 27% of the children who were diagnosed with PLSVC after angiography was performed (20
out of 74). A statistically significant association was present between PLSVC and other congenital cardiac anomalies,
including ventricular septal defect (n = 42, 56.8%), atrial septal defect (n = 31, 41.9%), pulmonary stenosis (n = 19,
25.7%), atrioventricular septal defect (n = 10, 13.5%), patent ductus arteriosus (n = 6, 8.1%) and cor triatriatum (n = 3,
4.1%).
Conclusion: Transthoracic echocardiography usually visualizes dilated coronary sinus in association with PLSVC.
However, SVC injection should be performed in patients undergoing angiography so that morbidity and mortality related
with persistent left superior vena cava can be avoided during cardiovascular surgery.
Key words child, congenital heart disease, persistent left superior vena cava.
First described in 1738, persistent left superior vena cava
(PLSVC) is one of the most commonly encountered systemic
venous anomalies. This vascular anomaly has an incidence of
0.3–0.5% within the general population.
1,2
Since PLSVC is
usually asymptomatic, its diagnosis can be delayed until associ-
ated complications occur while central venous interventions are
performed.
3
As a part of normal embryological development, the left ante-
rior cardinal vein collapses and eventually degenerates. When
this process is interrupted, the left anterior cardinal vein becomes
patent and PLSVC happens.
4
A number of cardiac and extra-
cardiac malformations can accompany PLSVC and, thus, this
venous anomaly may present with various clinical symptoms and
signs.
5,6
In at least 67% of the patients, there is patency of right
superior vena cava which refers to “bilateral superior vena
cavae.”
1,6
Early diagnosis of PLSVC is important in conditions requir-
ing central venous access, cardiac catheterization, pacemaker
implantation and cardiovascular surgery.
4
Angiography is the
gold standard for visualizing the anatomy and the accompanying
malformations in patients with PLSVC. Moreover, direct X-ray,
computed tomography, magnetic resonance imaging and tran-
sthoracic echocardiography may aid in the diagnosis of this vas-
cular anomaly.
6
Transthoracic echocardiography has a sensitivity
of 68–96% for the diagnosis of PLSVC and this diagnostic sen-
sitivity increases in case contrast echocardiography or transe-
sophageal echocardiography is also utilized.
1–8
The present study reviews the experience of a tertiary health-
care center of children diagnosed with PLSVC over a 10-year
period of time.
Methods
The present study is based on the retrospective review of the
medical records of 1205 children who consecutively underwent
cardiac catheterization because of congenital heart disease at the
department of pediatric cardiology in Gazi University Medical
Faculty Hospital, from 2000 to 2007. The present study was
approved by the local medical ethics committee which decided
Correspondence: Serdar Kula, MD, Gazi University Faculty of Medi-
cine, Department of Pediatric Cardiology, Besevler, Ankara 06500,
Turkey. Email: kula@gazi.edu.tr
Received 8 March 2011; revised 7 June 2011; accepted 30 June
2011.
Pediatrics International (2011) 53, 1066–1069 doi: 10.1111/j.1442-200X.2011.03443.x
© 2011 The Authors
Pediatrics International © 2011 Japan Pediatric Society