A contralateral persistent superior vena cava (PSVC) can occur in a normal child or in association with congenital heart defects (CHDs). Its prevalence has been demonstrated in relatively small cohorts. We aim to assess the frequency of a PSVC in a large cohort of children with and without CHDs. To estimate its significance, we have searched for a PSVC in all children referred for echocardiography in our institution during a 16.5-year period. A group of 17,219 children comprised 8,140 children with a structural heart anomaly and 9,079 children with a structurally normal heart. Association between a PSVC and specific classes of CHD were looked for. A total of 288 children (1.7%) had a PSVC; 0.56% (51 of 9,079) in the normal heart group and 2.9% (237 of 8,140) in the congenital heart anomalies group. Odds ratio for having heart anomaly in the presence of PSVC was 5.2 (95% confidence interval 3.7 to 7.0). A PSVC was above all associated with atrioventricular septal defects, conotruncal malformations, and left-sided defects. The odds ratio of having PSVC in the aforementioned malformations compared with the normal heart group was 23.8, 13.6, and 11.0, respectively. In conclusion, although present in normal subjects, PSVC was more often associated with congenital heart and other anomalies, especially with atrioventricular septal defects, conotruncal malformations, and left-sided defects.
A contralateral persistent superior vena cava (PSVC) is the most common variant of the thoracic venous system. In subjects with a normal heart, its prevalence is estimated as 0.3%. However, true incidence is unknown because a PSVC usually remains clinically silent until it is detected incidentally during cardiovascular imaging, pacemaker implantation, or surgery. Preoperative knowledge of a PSVC may avoid needless complications, especially for patients undergoing cardiopulmonary bypass surgery and electrophysiologic procedures. Although a PSVC may not have direct hemodynamic significance, it is frequently associated with congenital heart defects (CHDs) or cardiac arrhythmia. A PSVC can be reliably diagnosed at prenatal screening echocardiography and has been proposed as a marker indicating the need for thorough examination to rule out the presence of other cardiac and noncardiac embryopathies. Yet, the reported frequency of a PSVC in patients with CHD also varies widely, from 1.3% to 11%, because of the small study cohorts and selection bias in studies reporting prevalence ( Table 1 ). We determined the prevalence of a PSVC in a large study population including patients with a wide variety of congenital heart anomalies and in subjects with structurally normal hearts.
| Reference | Year | PSVC in CHD (%) | PSVC in Normal (%) | Method of Detection | Population |
|---|---|---|---|---|---|
| Abott | 1936 | 2.6 | Autopsy | CHD (26/1,000) | |
| Campbell and Deuchar | 1954 | 3 | Cath; autopsy; surgery | CHD (46/1,500) | |
| Fraser et al | 1961 | 3.8 | 0 | Cath (27); surgery (1); autopsy (2) | CHD or RHD (869); CHD (30/786); RHD (0/83) |
| Cha and Khoury | 1972 | — | — | Cath | CHD or RHD (12/275) (10 CHD, 2 normal, 4.4% overall) |
| Bjerregaard and Laursen | 1980 | 1.3 | — | Cath or autopsy | CHD (49/3,671) |
| Huhta et al | 1982 | 5 | — | Cath | CHD (40/800) |
| Buirski et al | 1986 | 11 | — | Cath | CHD (56/519) |
| Nsah et al | 1991 | 9 | — | Autopsy | CHD (104/1,203) |
| Leibowitz et al | 1992 | — | 0.1 | Cath | Emergency cath (3/4,000) |
| Macedo et al | 1996 | 9 | — | Fetopsy | Overall (9/99) |
| Parikh et al | 1996 | 4.4 | 1 | Cath in CHD and acquired HD | Overall (36/882); CHD (35/780); RHD (1/102) |
| Biffi et al | 2001 | — | 0.3 | PM or CD implantation | Overall (4/1,254) |
| Morgan et al | 2002 | — | 3 | PM or CD implantation | Overall (10/300) |
| Gonzalez-Juanatey et al | 2004 | — | — | PSVC on TTE, TEE, MRI | Overall (10/9,075); CHD (3/10) |
| Pasquini et al | 2005 | 2.7 | 0.3 | Fetal echocardiography | CHD (12/448); normal (4/1,230) |
| Galindo et al | 2007 | 9 | 0.2 | Fetal ultrasound | CHD (44/504); normal (10/5,233) |
| Postema et al | 2008 | 4.8 | 0.5 | TTE | CHD (89/1,825); (13/2,601) |
Methods
We conducted a retrospective review of our pediatric echocardiography database to identify all patients who were referred to the Department of Pediatric Cardiology of the Hadassah-Hebrew University Medical Center for echocardiographic assessment from January 1993 to June 2009. All imaging and laboratory evaluations were clinically indicated. The search for a PSVC either directly or by way of the presence of a dilated coronary sinus is an integral part of the routine pediatric echocardiographic study. Our Institutional Review Board waived the requirement for informed consent.
During the period included in this study, 17,415 children underwent 38,440 full echocardiographic studies in our echocardiography laboratory. From this large population, 196 children were excluded because of insufficient or missing data. The remaining 17,219 children were divided into 2 groups.
Group 1 (the CHD group) included 8,140 children (47.3%) with a congenital cardiac malformation. Most patients with cardiac anomalies had been referred for assessment immediately after birth and usually before the age of 12 months.
Group 2 (the normal heart group) included the remaining 9,079 children (52.7%) who had no structural heart defects on echocardiography. Studies were performed in the normal heart group because of functional murmur (4,376, 48.2%), prechemotherapy (863, 9.5%), suspected rheumatic heart disease (590, 6.5%), chest pain (445, 4.9%), palpitations or tachycardia (409, 4.5%), syndromes and extracardiac anomalies (381, 4.2%), cyanotic events (291, 3.2%), fever workup (236, 2.6%), syncope (227, 2.5%), fetal echogenic foci (154, 1.7%), cerebrovascular event (100, 1.1%), family history of CHD (82, 0.9%), and other reasons (925, 10.2%).
Archived echocardiographic data and computer-based patient records were reviewed for all patients included in the study. The presence or absence of a PSVC, other structural cardiac anomalies, and extracardiac anomalies and syndromes were recorded.
The diagnosis of a PSVC included persistent left superior vena cava in situs solitus and persistent right superior vena cava in situs inversus of viscera and atria. Cases with single left-sided superior vena cava in situs inversus were excluded.
Congenital heart anomalies included left-sided obstructive lesions (LOLs), including bicuspid aortic valve (with any degree of obstruction or insufficiency); right-sided obstructive lesions; shunt lesions, excluding a patent foramen ovale; and complex lesions, including single ventricle, atrioventricular septal defects (AVSDs), conotruncal malformations (CTMs), and combined and heterotaxy lesions. Shunt lesions (atrial septal defect, ventricular septal defect, or patent ductus arteriosus) were regarded separately only when they were not part of a more complex intracardiac lesion. For example, atrial septal defect and additional ventricular septal defect in tetralogy of Fallot were not counted as simple shunt lesions.
Three groups of heart lesions were classified: (1) CTMs, which included tetralogy of Fallot, double outlet right ventricle, interrupted aortic arch, aortopulmonary window, absent pulmonary valve, transposition of the great arteries, pulmonary atresia, and truncus arteriosus; (2) LOLs, including cor triatriatum, supramitral ring, mitral stenosis, discrete subaortic membrane, bicuspid aortic valve (with any degree of obstruction or insufficiency), aortic stenosis, and coarctation of the aorta; and (3) AVSDs, including primum atrial septal defect, cleft mitral valve, and intermediate or complete forms of canal lesions. The frequency of a PSVC in patients with various CHD, and in those with normal heart anatomy, was calculated and compared.
All patients underwent a thorough transthoracic echocardiographic study performed in a standard fashion, according to the accepted guidelines. The superior vena cava was evaluated from the subcostal short- and long-axis views. A PSVC was demonstrated by 2-dimensional echocardiography and color Doppler interrogation from the subcostal and suprasternal short- and long-axis views. A PSVC usually drains into a dilated coronary sinus ; therefore, coronary sinus size was assessed from the subcostal, apical 4-chamber, and parasternal views. We defined PSVC only when directly demonstrated and did not suffice with the indirect finding of dilated coronary sinus. A systematic search for a communicating vein between the venae cavae was performed.
Echocardiographic examinations for the patients included in this study were performed and interpreted by 1 of 4 well-trained pediatric cardiologists (AJJTR, AN, ZP, and SG). Pediatric echocardiographies were performed with 1 of 5 systems (SS 380 Powervision with 5-MHz transducer at 7.5-MHz imaging frequency, Toshiba Medical Systems, Tokyo, Japan; Sonos 1500 with a 7.5- or 5-MHz transducer at 7.5-MHz imaging frequency, Hewlett-Packard, Andover, Massachusetts; Sequoia 512 with the 5- and 7-MHz transducers, Acuson Computed Sonography, Mountain View, California; and Vivid FiVe and Vivid 7 Dimension with 3.5- to 7-MHz phased array transducers, GE Medical, Milwaukee, Wisconsin).
The prevalence of a PSVC in the group of patients with any congenital cardiac malformations and in the subgroups of specific malformations was compared with prevalence in the group of children with structurally normal hearts using the chi-square or Fisher’s exact test when sample size was small. Odds ratios for having a congenital heart disease after finding a PSVC were calculated. A p value of ≤0.05 was considered statistically significant.
Results
A total of 288 patients had a PSVC ( Table 2 ). The odds ratio for having a PSVC in the presence of a congenital heart malformation was 5.2 (95% confidence interval 3.7 to 7.0).
| Cardiac Structure | Total | PSVC, % | Odds Ratio | p | Test | |
|---|---|---|---|---|---|---|
| Compared With Other CHD Group ∗ | Compared With Normal Heart Group | |||||
| Normal | 9,079 | 0.6 (51/9,079) | ||||
| All CHDs | 8,140 | 2.9 (237/8,140) | 5.2 | <0.0001 † | Pearson chi-square | |
| Atrioventricular septal defects | 224 | 13.4 (30/224) | 9.1 | 23.8 | <0.0001 † | Fisher’s exact test |
| CTMs | 814 | 7.6 (62/814) | 5.2 | 13.6 | <0.0001 † | Pearson chi-square |
| LOLs | 860 | 6.2 (53/860) | 4.2 | 11.0 | <0.0001 † | Pearson chi-square |
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