Clinical Radtology (1986) 37, 131-138 0009-9260/86/419131502.00 © 1986 Royal College of Radiologists

Superior Vena Caval Abnormalities" Their Occurrence Rate, Associated Cardiac Abnormalities and Angiographic Classification in a Paediatric Population With Congenital Heart Disease GRAHAM BUIRSKI, S. C. JORDAN*, H. S. JOFFE* and P. WILDE

Department of Radiodiagnosis and Imaging Research Unit, and * Department of Cardiology, Bristol Royal Infirmary, Bristol

Angiography of the superior systemic veins was per- formed on 510 patients with congenital heart disease. An analysis of these angiograms was carried out and a simple angiographic classification of superior vena caval ano- malies is presented. Eleven per cent of cases had complete bilateral superior vena cavae, a frequency of occurrence higher than that previously reported. An analysis of associated congenital heart lesions revealed a higher than average association of bilateral superior vena cavae with atrioventricular septal defects and double-outlet right ventricle, but this was seen rarely in cases of transposi- tion of the great arteries. Bilateral superior vena cavae also occurred very frequently (72 %) in patients with situs abnormalities. Femoral vein catheterisation is likely to facilitate diagnosis and appropriate radiological signs are discussed.

Due to the increasing operative treatment of congenital heart disease and the use of cardiopulmonary bypass, the pre-operative diagnosis of a persistent left superior vena cava (SVC) is now of some importance (Gensini et al., 1959; De Leval et al., 1975). Failure to detect its presence may result in excessive return of blood through the right atrium during cardiopulmonary bypass, inade- quate venous return to the pump and the formation of residual intracardiac shunts (De Leval et al., 1975). This is especially important in patients with congenital defects such as tetralogy of Fallot, pulmonary atresia and truncus arteriosus where post-operative systemic venous pressure is often above left atrial pressure (Huhta et al., 1982). Accidental ligation of the vessel may result in localised venous hypertension (De Leval et al., 1975).

The incidence of persistent left SVC in the normal population is estimated at 0.5% (Steinberg et al., 1953) but in patients with congenital heart disease the inci- dence is 1.3-5% (Fraser et al., 1961; Bjerregard and Laursen, 1980; Huhta et al., 1982). These figures have been obtained from studies at autopsy, echocardiogra- phy and angiocardiography. It has been suggested by Campbell and Deuchar (1954) that persistent left SVC m congenital heart disease may occur considerably more often than previously described. Some authors have suggested that as many as 10% of cases with congenital heart disease have this abnormality (Feind and Hauch, 1953; Sipila et al., 1955).

Routine systemic venous injections during cardiac catheterisation have been performed at this hospital

regularly for some years and it was felt that persistent left SVC occurred more often than previously reported. A retrospective study was, therefore, undertaken to confirm this. Anomalies of the inferior vena cava (IVC), azygos and hepatic venous drainage were identified where possible but are not included in this review.

No discussion of the embryological development of persistent left SVC is presented as this has been well reviewed by other authors (Cambell and Deuchar, 1954; Winter, 1954; Gensini et al., 1959).

METHODS

All paediatric cardiac catheterisations at this hospital between January 1978 and May 1983 were included.

All the angiocardiograms were reviewed and in each case the angiographic diagnosis of congenital heart dis- ease was made as accurately as possible. Normal and post-operative angiocardiograms were excluded, but patients' pre-operative cardiac studies were included if they fell within the review period. Care was taken to ensure that each patient was included only once if they had multiple investigations.

The tip of the catheter was placed, whenever possible, in the left brachiocephalic vein and hand injections of contrast medium were performed and recorded by cineradiography in the anterior projection.

All cases of persistent left SVC were analysed with reference to their haemodynamic importance and radio- graphic appearances. A 'haemodynamically significant' vessel was defined as one which accepted a significant flow of venous blood from either the pulmonary or systemic circulations.

Atrial situs was assessed in all cases with abnormal SVC. This was done by observing bronchial situs from the cineangiograms where possible and, if necessary, from plain chest radiographs. Measurement of the ratio between the right and left main bronchi were compared as described by Partridge (1979) who found this to be the best indicator of atrial situs.

RESULTS

During the review period 552 new patients with con- genital heart disease underwent cardiac catheterisation. Of these, 510 patients (92%) had systemic venous injec- tions recorded by cineangiography. In all but two

132 CLINICAL RADIOLOGY

patients the venous catheter was introduced via the femoral vein.

The main congenital heart defects and their overall occurrence are listed in Appendix 1. Diagnoses were allocated on the basis of cardiac connections and impor- tant cardiac malformations. Abnormalities of position and situs were usually not included in the main diagnos- tic table but have been discussed separately. The case occurrence rate of haemodynamically significant persis- tent left SVC in each condition is shown in the rank order in Table 1.

Superior vena caval abnormalities were seen in 66 patients. A persistent left SVC (complete or partial) in addition to a right SVC was seen in 61 cases and could be angiographically separated into five groups (Fig. 1). Complete bilateral SVC (Groups 1, 2, 3 and 5) was found in 56 of these cases (11%). Haemodynamically significant SVC anomalies (Groups 1-4) were present in 53 cases (10.4%). Four patients were found to have an isolated left SVC with otherwise normal systemic venous drainage. A duplicated left brachiocephalic vein was found in one patient and has not been included in any further analysis.

The distribution of bronchial situs in each patient with abnormal systemic venous drainage is shown in Table 2.

Table 1 - Distribution of 53 cases of bilateral SVC (Groups 1-4) in relation to congenital heart disease*

Main diagnosis Total No, of cases Case No. of with significant occurrence cases persistent left rate (%)

SVC (Groups 1-4)

Situs inversus (normal 1 1 connections)

Double-outlet right ventricle 8 5 Total anomalous pulmonary 10 6

venous drainage Common atrioventricular 41 8

septal defects Truncus arteriosus 6 1 Secundum atrial septal defect 20 3 Coarctation of aorta 65 8 Pulmonary atresia 34 4 Tetralogy of Fallot 42 4 Ventricular septal defect 65 6 Hypoplastic left heart 29 2 'Single ventricle' 33 2 Pulmonary stenosis 42 2 Transposition of the 54 1

great arteries

100.0

62.5 60.0

19.5

16.7 15.0 12.3 11.8 9.5 9.2 6.9 6.1 4.8 1.9

*This is a simplified version of Appendix 1.

Table 2 - Distribution of bronchial situs in 65 patients with abnormal systemic venous drainage

Bronchial Systemic venous anomaly (by Groups) SitUS

Group Group Group Group Group Isolated 1 2 3 4 5 left

SVC

Situs solitus 19 15 1 4 8 - Situs inversus 1 1 - - - 1 Situs ambiguus

Left 3 3 - 1 - 1 isomerism

Right 1 4 - - - 2 isomerism

Group 1: Bilateral SVC Without Intercommunicating Vein

Group 1 comprised 24 patients, 12 male and 12 female, with an age range of 1 day to 15 years. The right SVC drained the right arm and right head and neck (Fig. 2a). The left SVC accepted venous blood from a similar drainage area on the left (Fig. 2b) and was, therefore, of haemodynamic significance. There was no intercom- municating vessel between the two superior vena cavae. In 80% of the patients the calibre of the two vessels was the same while in 10% either the right or left SVC was larger. In four patients the left SVC entered the left atrium. The remainder drained into the right atrium via the coronary sinus.

Group 2: Bilateral SVC With Intercommunicating Vein

Group 2 comprised 23 patients, with a male to female ratio of 1:1.2 and an age range of 1 day to 11 years (Fig. 3a). The persistent left SVC was considered to have the same haemodynamic significance as in Group 1. The calibre of the two vessels was the same in 68% of patients. In 22% of patients the right SVC was dominant while in 10% the left SVC was dominant. The intercom- municating vein was usually single and haemo- dynamically insignificant, but in two patients it was large, causing shunting to either the left or the right- sided vessel, the redundant SVC being of a smaller calibre. In one patient the intercommunicating vein anastomosed with the inferior thyroidal veins (Fig. 3b) and in another it consisted of a plexus interlinking the two vena cavae. One persistent left SVC drained into the left atrium; the others into the coronary sinus.

(a) (b) (c) (d) (e)

Fig. 1 - Angiographic classification of bilateral SVC (61 cases). (a) Group 1 - bilateral SVC without intercommunicating vein (24 cases). (b) Group 2 - bilateral SVC with intercommunicating vein (23 cases). (c) Group 3 - bilateral SVC with supracardiac total anomalous pulmonary venous drainage (TAPVD) (1 case). (d) Group 4 - Supracardiac TAPVD (5 cases). (e) Rudimentary persistent left SVC (8 cases).

S U P E R I O R V E N A C A V A L A B N O R M A L I T I E S 133

(.) (b) Fig. 2 - (a) Angiogram with the catheter running from the inferior vena cava to the right-sided SVC and contrast medium injection recorded in the anterior view. Thereisn•evidence•fthe•eftbrachi••epha•icvein(n•turbu•entin••wandn•reflux•fc•ntrastmedium) Aninferior thyroidalvein is demonstrated. (b) Angiogram with the catheter running from the inferior vena cava to the left-sided superior vena cava via the coronary sinus and contrast medium injection recorded in the anterior view. Reflux of contrast medium down the hemiazygos system is seen but there is no evidence of intercommunication with the right SVC.

(a)

Fig. 3 - Angiograms with the catheter running from the inferior vena cava to the right-sided SVC and contrast medium injection recorded in the anterior view. (a) There is an intercommunicating vein with passage of contrast medium that outlines the left-sided superior vena cava. (b) A bilateral SVC with an intercommunicating vein is shown. Note the communication with the inferior thyroid vein. (b)

Group 3: Bilateral SVC With Supracardiac Anomalous Pulmonary Venous Drainage

T h e r e was o n e p a t i e n t in G r o u p 3. T h e a n o m a l o u s p u l m o n a r y ve in s d r a i n e d in to a c o m p l e t e p e r s i s t e n t lef t S V C w h i c h c o m m u n i c a t e d w i t h b o t h t h e c o r o n a r y s inus and a n o r m a l le f t b r a c h i o c e p h a l i c ve in .

13

Group 4: Supracardiac Total Anomalous Pulmonary Venous Drainage (Partial Persistent Left SVC With Ano- malous Pulmonary Venous Drainage)

T h e r e w e r e f ive p a t i e n t s in G r o u p 4 (Fig. 4a). I n this w e l l - r e c o g n i s e d c o n d i t i o n , t he a s c e n d i n g l e f t - s i ded v e i n f r o m t h e le f t h i l u m to t h e lef t b r a c h i o c e p h a l i c v e i n

134 C L I N I C A L R A D I O L O G Y

(a) (b)

Fig. 4 - (a) Angiogram with the catheter running from the inferior vena cava to the ascending portion of the left SVC via the normal right SVC and the left brachiocephalic vein. Contrast medium injection is recorded in the anterior view. The cine study showed pulmonary venous blood joining this vessel and flowing superiorly to the left brachiocephalic vein (b) Late phase of a pulmonary arteriogram in the anterior view with the catheter running from the right subclavian vein to the main pulmonary artery via the normal SVC and right-sided cardiac chambers. A left-sided vein is seen ascending towards the left brachiocephalic vein.

r ep re sen t s the u p p e r pa r t of a pe r s i s t en t left SVC. T h e p u l m o n a r y venous a n a t o m y in these cases is bes t d e m o n s t r a t e d by the fo l low- th rough of a p u l m o n a r y a r t e r i o g r a m , pa r t i cu l a r ly if select ive left and r ight in jec- t ions are m a d e (Fig. 4b) , a l though the u p p e r pa r t of the pe r s i s t en t left S V C can be shown in the usual way by di rec t in jec t ion .

Group 5: Rudimentary Persistent Left SVC

G r o u p 5 c o n t a i n e d e ight pa t ien t s (Fig. 5). In these pa t ien t s a lmos t all the venous b l o o d of the left s ide of the head and neck and left a rm d ra ined into a n o r m a l left b r ach iocepha l i c ve in and thence into the r ight SVC, bu t a very smal l c o m m u n i c a t i n g vessel in the pos i t ion of the pe r s i s t en t left S V C was d e m o n s t r a t e d by the con t ras t m e d i u m in jec t ion . Con t r a s t m e d i u m was seen to f low towards the h e a r t and en t e r into the c o r o n a r y sinus in seven pa t ien t s . In one pa t i en t the ca the t e r was passed r e t r o g r a d e l y in to the b rach iocepha l i c vein f rom the cor- ona ry sinus, fo l lowing the same course as desc r ibed above .

Other Superior Systemic Venous Anomalies

A b n o r m a l venous a n a t o m y was inconclus ive ly d e m o n s t r a t e d in a fu r the r 14 pa t ien ts . In e ight , con t ras t m e d i u m e n t e r e d a smal l vessel in the pos i t ion of a rudi- m e n t a r y pe r s i s t en t lef t S V C and was seen to f low re t ro - g rade ly t owards the hea r t . No c o m m u n i c a t i o n wi th the c o r o n a r y sinus was d e m o n s t r a t e d bu t the d i r ec t ion of

Fig. 5 - Angiogram with the catheter running from the IVC to the left brachxocephalic vein via the normal right SVC. Contrast medium injection recorded in the anterior view. A small, left-sided vessel drains down to the coronary sinus. This was seen clearly on the cine study but it has been marked additionally for clarity in this illustration (arrows).

SUPERIOR VENA CAVAL ABNORMALITIES 135

flow and the anatomical position of the vessel suggested that this may be a rudimentary left SVC (Group 5).

No evidence of an intercommunicating vessel or of a normal brachiocephalic vein was demonstrated by contrast medium injection or catheterisation in six patients. Only a single right SVC was found and it was, therefore, presumed that a second SVC had been pre- sent, thus allowing venous drainage from the left arm and head to occur.

Situs abnormalities

Abnormalities of bronchial (and, thus, presumed atrial situs) were seen in 18 (28%) of 65 patients with systemic venous abnormalities (Table 2). Within this group of abnormal situs, 72% of patients had complete bilateral SVC, the remainder having an isolated left SVC. The associated congenital heart defects in patients with isomerism and complete bilateral SVC are shown in Table 3.

The overall case abnormality rate of SVC anomalies in the remaining 492 patients with situs solitus was 9.5 %.

Table 3 - Congenital heart defects in 11 patients with isomerism and bilateral persistent left SVC

SVC Group 1 SVC Group 2

Left Coarctatioaa Coarctation isomerism Single ventricle Atrioventricular septal

defect Ventricular septal defect Ventricular septal defect

Right Double-outlet R ventricle Double-outlet R ventricle isomerism Single ventricle

Pulmonary atresia T~ansposition +

pulmonary atresia

Associated Congenital Heart Defects (Table 1 and Appendix 1)

There was an important association of bilateral SVC with atrioventricular septal defects (19.5%) but the superior vena caval anomalies were found only in those with both atrial and large ventricular septal components of the defect (i.e. those with complete atrioventricular septal defect, of whom 26% had persistent left SVC). An even higher incidence was seen in patients with a double-outlet right ventricle, where five (62.5%) of eight patients had persistent left SVC (including two with situs abnormalities). Bilateral SVC was present in the solitary case of situs inversus with no other cardiac abnormality.

In pulmonary stenosis there was a lower than average incidence (4.8 %) and only one case (1.9 %) of significant bilateral SVC was diagnosed in the 54 patients with transposition of the great arteries. The incidence of persistent left SVC in the remaining conditions lay between 5% and 17%.

DISCUSSION

In all previous reports the angiographic diagnosis of persistent left SVC has been obtained predominantly

from catheterisation of the right or left arm (Campbell and Deuchar, 1954; Gensini et al., 1959; Fraser et al., 1961). Catheterisation from the left arm is more likely to demonstrate persistent left SVC but the complication rate is higher (Fraser et al., 1961) and if the right atrium° is reached via the persistent left SVC and coronary sinus it usually proves very difficult to catheterise the right ventricle and pulmonary artery (Gensini et al., 1959). If a right arm route is used then a left-sided SVC is easy to overlook.

The use of femoral vein catheterisation (usually per- cutaneous) allows ready access to the normal systemic veins as well as to the coronary sinus and a persistent left SVC if present. In addition, this approach usually allows satisfactory catheter positions to be achieved in the other cardiac chambers. The ease with which the femoral catheter can be positioned in the systemic veins has enabled injections of contrast medium to be made routinely in nearly all cases.

The high occurrence rate of left SVC found in this series is comparable with that estimated by Sipila et al. (1955) and is higher than that reported in any other study, including the comprehensive echocardiographic study recently reported by Huhta et al. (1982).

Different authors have described anatomical (Winter, 1954), embryological (Fraser et al., 1961) and haemo- dynamic (Gensini et al., 1959) classifications of persis- tent left SVC but, as the diagnosis is often made at angiography, an angiographic classification has been presented.

Groups 1, 2 and 5 in our classification have all been anatomically recognised as early as 1916 by McCotter and varying incidences of these have been quoted by subsequent authors. It is difficult to make useful com- parisons of the incidence of each type as the selection of cases and modes of examination have varied widely in the previous studies. It is clear, however, that our Groups i and 2 have been recognised by all as being the dominant groups. The diagnosis of an intercommunica- tion between left and right SVC is important since liga- tion of the left SVC may be contraindicated unless a suitable communication exists (Huhta et al., 1982). Although non-invasive techniques such as echocar- diography can now demonstrate significant abnor- malities of systemic venous drainage, the accuracy in demonstrating an intercommunicating vessel is only 72% (Huhta et al., 1982). We, therefore, believe that angiography still provides the most accurate method of diagnosis.

Attempts to correlate the persistent left SVC abnor- malities with different congenital heart lesions have not yielded consistent results in the literature. In our series, the overall pattern of congenital heart disease investig- ated (Appendix 1) is roughly in keeping with that expected in a large centre (Keith et al., 1978). In many conditions, the occurrence of persistent left SVC is simi- lar to that in the group as a whole (e.g. ventricular septal defect, coarctation, tetralogy of Fallot), but there are noteworthy exceptions. A much higher incidence of persistent left SVC was found in total atrioventricular septal defects (26%) and, particularly, in double outlet right ventricle (62.5%). The first of these associations has been previously recognised (De Leval et al., 1975). Although more complex cardiac disease occurred in patients with right isomerism (Table 3), only two patients in this group had double-outlet right ventricle.

136 CLINICAL RADIOLOGY

In addition, no patient with left isomerism had a com- plete atrioventricular canal defect. Therefore, the high occurrence rate of bilateral SVC in these two defects cannot be ascribed to the presence of isomerism and, thus, probably represents a truly high occurrence rate. It is of interest that a very low case occurrence rate of persistent left SVC was encountered in cases of trans- position of the great arteries, only one case of haemo- dynamically significant persistent left SVC being n o t e d in 54 cases and this was in a case with situs abnormality. We found no cases of bilateral SVC in the 31 patients with uncomplicated D-loop transposition of the great arteries. The condition has been shown to occur in trans- position by others (De Leval etal . , 1975; Bjerregard and Laursen, 1980) but our results indicate an unusually low incidence. In 30 cases of persistent left SVC reported by Fraser et al. (1961), none was associated with transposi- tion of the great arteries; De Leval et al. (1975) also noted a low incidence.

The presence of various situs abnormalities appears to be associated with a high incidence of SVC anomalies. In our series, 18 patients had situs abnormalities of various types (Table 2) and, of these, 13 (72%) had bilateral SVC. This compares well with the incidence of 66% in situs abnormalities quoted by Huhta et al. (1982). The frequency of bilateral SVC in patients with isomerism found in our series is also similar to that reported by Huhta et al. (1982). In their series they found that an intercommunication vessel (Group 2) occurred with the same frequency as Group 1 persistent left SVC in patients with left isomerism. Only one patient, however, with Group 2 persistent left SVC was s e e n with right isomerism. This is in contrast to our series, where four of five patients with right isomerism were classified as Group 2 persistent left SVC.

In our cases from Groups 1, 2 and 5, 7% of left SVC drained into the left atrium. The reported frequency of this anomaly is 4% (Winter, 1954). It is clinically impor- tant to recognise the anomaly as it results in systemic arterial desaturation of varying degrees. During angio- graphy in the anterior view, contrast medium from the persistent left SVC becomes turbulent and diluted shortly after reaching the left heart border, whereas usually the contrast medium is well contained within the coronary sinus passing obliquely into the right atrium.

The lack of reflux of contrast medium into the left brachiocephalic vein during injections into the right SVC in patients with persistent left SVC was first noted by Peel et al. (1956) and was seen in all of our patients in Groups 1 and 2. Other signs which were found useful in confirming the presence of abnormal venous anatomy w e r e :

(1) inability to pass the femoral vein catheter into a left brachiocephalic vein from the SVC,

(2) characteristic 'sigmoid' appearance of the catheter as it passed upwards from the IVC to coronary sinus and left SVC (Fig. 6) and

(3) the absence of non-opaque blood mixing with contrast medium in the right SVC at the expected site of confluence of the left brachiocephalic vein.

Gensini et al. (1959) found that in patients with bilateral SVC and an intercommunicating vein, the right SVC was reduced in calibre. In our Group 2 cases the calibre of the right SVC was often normal but did vary greatly, depending on the size of the anastomotic vessel.

Fig. 6 - Anglogram showing the characteristic sigmoid appearance of a catheter passed into the left superior venzl cava from the normal right- sided inferior vena cava via the coronary sinus: anterior view.

In the majority of cases, the right and left SVC were of equal size.

Although two-dimensional echocardiography has now been shown to be a reliable non-invasive way of detecting significant superior vena caval abnormalities (Huhta et al., 1982), it does require considerable opera- tor experience.

CONCLUSION

A large angiographic series of superior vena caval anomalies in a paediatric population with congenital heart disease is presented and a simple classification is outlined. The overall occurrence rate of SVC anomalies is approximately 12.9%. Complete bilateral persistent left superior vena cava occurred in 11.0%, with haemo- dynamically significant bilateral SVC (Groups 1-4) occurring in 10.4%.

Persistent left SVC is especially frequent in associa- tion with common atrioventricular septal defects, dou- ble-outlet right ventricle and situs abnormalities but rare with transposition of the great arteries.

It is recommended that transfemoral catheterisation is used and injections of contrast medium performed with the catheter in the left brachiocephalic vein if SVC anomalies are to be detected.

Acknowledgements. We are indebted to Miss J. Hugh and Miss N. Eberle for typing the manuscript.

REFERENCES

Bjerregard, P. & Laursen, H. B. (1980). Persistent left superior vena cava: incidence, associated congenital heart defects and frontal plane P-wave axis in a paediatric population with congenital heart disease. Acta Paediatrica Scandinavica, 69, 105-108.

Campbell, M. & Deuchar, D. C. (1954). The left-sided superior vena cava. British Heart Journal, 16, 423-439.

SUPERIOR VENA CAVAL ABNORMALITIES 137

De Leval, S. R., Ritter, D. G,, McGoon, D. C. & Danielson, G. K. (1975). Anomalous systemic venous connection: surgical con- siderations. Mayo Clinic Proceedings, 50, 599-610.

Feind, H. R. & Hauch, H. J. (1953). Uber drei Falle von Doppelter oberer Hohlvene. Zeitschriftfiir Kreislaufforschung, 42, 53-61.

Fraser, R. S., Dvorkin, J., Rossal, R. E. & Eidem, R. (1961). Left superior vena cava: a review of associated congenital heart lesions. Catheterization data and roentgenologic findings. American Jour- nal of Medicine, 31, 711-716.

Gensini, G. G., Caldini, P., Casaccio, F. & Blount, S. G. (1959). Persistent left superior vena cava. American Journal of Cardiol- ogy, 4, 677-685.

Huhta, J. C., Smallhorn, J. F., Macartney, F. J., Anderson, R. H. & De Leval, M. (1982). Cross-sectional echocardiographic diagnosis of systemic venous return. British Heart Journal, 48, 388-403.

Keith, J. D., Rowe, R. D. & Vlad, P. (1978). Prevalence, incidence

and epidemiology. Heart Disease in Infancy and Childhood, Chap. 1, pp. 3-13. Macmillan, London.

McCotter, R. E. (1916). Three cases of the persistence of the left superior vena cava. Anatomical Record, 10, 371-383.

Partridge, J. (1979). The radiologlcal evaluation of atrial situs. Clinical Radiology, 30, 95-103.

Peel, A. A. F., Semple, T., Kelly, J. C. & Blum, K. (1956). Ano- malous venous drainage with death from cardiac catheterization. Scottish Medical Journal, 2, 83-84.

Sipila, W., Hakkila, J., Heikel, P. E. & Kyllonen, K. E. J. (1955). Persistent left superior vena cava. Annals Medicine Internae Fen- niae, 44, 251-261.

Steinberg, L., Dubilier, W. & Lukas, D. (1953). Persistence of left superior vena cava. Disease of the Chest, 24, 479-488.

Winter, F. S. (1954). Persistent left superior vena cava: survey of world literature. Angiology, 5, 90-132.

A p p e n d i x 1 - Inc idence of congeni ta l hear t d isease and bi lateral SVC in 552 patients

Overall % No. of No. of % incidence No. of inctdence cases with cases of of bdateral cases in study systemic bilateral SVC SVC in each

population venous (Groups 1-4) condition angiogram (rudimentary in (rudimentary m

parentheses, parentheses) Group 5)

Comments

Ventricular septal defect

Alone

With aortic regurgitation

Coarctation of the aorta

Alone With VSD

Transposition of the great arteries (a) D-loop (b) D-loop+VSD (c) D-loop+coarctation (d) D-loop+complex or multiple lesions (e) L-loop (corrected) (0 L-Loop (+VSD or complex)

Pulmonary stenosis Valvular and/or

infundibular With VSD (not tetralogy of

Fallot)

Tetralogy of Fallot

Patent ductus arteriosus

Common atrioventricular septal defect Partial (primum ASD)

Total (primum ASD+VSD)

Pulmonary atresia With VSD Intact ventricular septum

'Single ventricle' group Tricuspid atresia

Others

Hypoplastic left heart syndrome

13.2

70* 62 6* (1)

3 3 -

47 19

36 7 4 9

1 4

36

10

44*

43

11

31

27 11

20 14

33

12.0

11.1

3

8.3

8.0

7.8

7.6

6.9

6.2

6.0

46 19

31 7 3 9

34

8

42

40

10

31

23 11

19 14

29

6 (1) 2

- (1)

2

4*

3 (1) 1

f (1) 1

2 (3)

9.2 (1.5)

12.3 (1.5)

1.9 (1.9)

4.8

9.5

19.5

11.8 (2.9)

6.1 (3.0)

6.9 (10.3)

Single or multiple: all sites and sizes

Includes those with associated PDA

Includes cases with interrupted aortic arch

One case associated with cor triatriatum

One primum ASD patient with Down's syndrome

Seven total AVC patients with Down's syndrome

One patient had coarctation

Complex combinations include TGA, common- inlet and double-inlet ventricle

Includes aortic and mitral stenosis or atresia

138 CLINICAL R A D I O L O G Y

A p p e n d i x 1 cont. - Inc idence of congenital heart disease and b i l a t e ra l SVC in 552 patients

Overall % No. of No. of % Incidence Comments No. of incidence cases with cases of of bilateral cases in study systemic bilateral SVC SVC in each

population venous ( Grouops 1-4) condition angiogram (rudimentary in (rudimentary in

parentheses, parentheses) GroUp 5)

S e c u n d u m a t r ia l s ep ta l defec t 21 3.8 20 3 15.0

To ta l a n o m a l o u s P V D 2.0 60.0 (a) Sup raca rd i ac 6 6 6 T h e s e p a t i e n t s are in

G r o u p s 3 and 4 (b) R e t r o c a r d i a c 4 3 - P u l m o n a r y veins to r igh t

SVC in one pa t i en t (c) In f raca rd iac 1 1 -

D o u b l e - o u t l e t r igh t ven t r i c l e 10 2.0 8 5 62.5

Truncus a r t e r iosus 8 1.4 6 1 16.7

Par t i a l a n o m a l o u s P V D 4 0.7 4 - -

C o r o n a r y a n o m a l i e s 3 0.5 3 - -

C o n g e n i t a l mi t r a l r e g u r g i t a t i o n 3 0.5 3 - -

C a r d i o m y o p a t h y 3 0.5 2 - -

A o r t o - p u l m o n a r y w i n d o w 2 0.4 2 - -

H y p o p l a s t i c r ight ven t r i c le 2 0.4 2 - -

E b s t e i n ' s a n o m a l y 1 0.2 1 - -

D o u b l e aor t i c a rch 1 0.2 1 - -

Cor t r i a t r i a tum 1 0.2 1 - - S e c o n d case u n d e r T G A

Situs inversus 1 0.2 1 1 100

Congen i t a l l y absen t p u l m o n a r y 1 0.2 1 - - va lve

Tota l s 552 510 53 (8) 10.4 (1.6)

*A case wi th s i tus inversus . P D A = p a t e n t duc tus a r te r iosus ; V S D = v e n t r i c u l a r sep ta l defect ; A S D = a t r i o v e n t r i c u l a r sep ta l defec t ; A V C = a t r i o v e n t r i c u l a r sep ta l defect ;

T G A = t r a n s p o s i t i o n of the g rea t a r te r ies ; P V D = p u l m o n a r y venous d ra inage .