1979, British Journal of Radiology, 52, 855-861
NOVEMBER 1979
The left atrial notch: a sign of persistent left superior vena cava draining to the right atrium By J. P. Owen, M.B., B.S., D.M.R.D., F.R.C.R. Department of Diagnostic Radiology, University of Newcastle upon Tyne and W. Urquhart, M.B., Ch.B., D.M.R.D., F.R.C.R.* Late Newcastle General Hospital and the Regional Cardiothoracic Centre, Freeman Hospital, Newcastle upon Tyne {Received May 1979) ABSTRACT
A notch in the inferior border of the left atrium has been observed in patients with a persistent left superior vena cava draining via the coronary sinus into the right atrium. It is suggested that the notch is due to the dilated coronary sinus. Persistence of the left superior vena cava (LSVC) is encountered in a number of children with congenital heart disease and its pre-operative diagnosis may be important to the cardiac surgeon. For example, in patients undergoing open-heart surgery by extracorporeal by-pass an unsuspected LSVC could result in a troublesome inflow of blood into the right atrium. The presence of a LSVC and left innominate *This paper is based on an original observation by Dr. Urquhart though he did not live to see the final form of the publication. His wisdom, knowledge and expertise are greatly missed.
would allow a right SVC to be used in the repair of a sinus venosus atrial septal defect and the larger SVC could be used when a cavo-pulmonary shunt is to be created. This paper presents another sign for suspecting a left superior vena cava (LSVC) at cardiac catheterization. MATERIALS AND RESULTS
Table I records the angiographic findings in four patients in all of whom a notch was present on the inferior border of the left atrium in association with a left persistent superior vena cava draining via the coronary sinus into the right atrium. A possible embryological and anatomical mechanism for production of the atrial notch is postulated in Fig. 1.
TABLE I ANGIOGRAPHIC FINDINGS
Case
Sex, age
Findings
Catheter passed to LSVC
Notch present on inferior border of left atrium
1
F 3.5 months Figs. 2A, 2B
Pulmonary valve stenois Pulmonary infundibular stenosis VSD Right sided aorta Left superior vena cava draining to coronary sinus
Yes
Yes
2
M 1 year 10 months Figs. 3A, 3B
Right ventricular hypertrophy Infundibular pulmonary stenosis Pulmonary valve stenosis VSD Left superior vena cava draining to coronary sinus
Yes
Yes
3
M 8 weeks Figs. 4A, 4B
Transportation of great vessels Hypoplastic stenosed mitral valve VSD Left superior vena cava draining to coronary sinus
Yes
Yes
4
F 10 years Fig. 5
Mitral regurgitation Left superior vena cava draining to coronary sinus
Yes
Yes
855
VOL.
52, No. 623 J. P. Owen and W. Urquhart
ac
v pc B.
A
a sp sv dc
4 weeks — common atrium u - septum primum V ac — sinus venosus pc - duct of Cuvier
-
dv L sub isa
umbilical vein vitelline vein anterior cardinal vein posterior cardinal vein
5J weeks - ductus venosus - Liver — subcardinal vein — intersubcardinal anastomosis
ss az h.az IVC
sup ivc h
— supracardinal veins — inferior vena cava - left innominate vein
superior vena cava left innominate vein septum secuncfum azygos vein h.az — hemi-azygos vein SVC
li
FIG.
1.
856
NOVEMBER 1979
The left atrial notch: a sign of persistent left superior vena cava draining to the right atrium
Isvc cor
cor
E.
8 weeks Normal anatomy lm ov cor
F.
Persistent left superior vena cava draining into coronary sinus. Isvc - left superior vena cava cor - coronary sinus
ligament of Marshall oblique vein coronary sinus
FIG. 1. The normal development of the venous system in the human embryo and a postulated mechanism for the production of the left atrial notch.
The angio-graphic appearances are illustrated in Figs. 2-5. DISCUSSION
It is suggested (Caffey, 1973) that a left superior vena cava (LSVC) should be defined as a structure which receives the hemiazygos vein and has a communication with the coronary sinus or a communication with the left atrium when there is no coronary sinus. In the absence of these features the vein is best described as a vertical vein. McManus (1941), cited by Gardner and Oram (1953), classifies the various types of LSVC into: 1. Persistent superior vena cava (SVC) with right SVC (bilateral SVC). (a) Persistent LSVC connected to the coronary sinus and either with a cross anastomosis (left innominate vein) or without this. (b) Persistent LSVC draining the pulmonary veins. 2. Persistent LSVC without right SVC. A persistent LSVC has been recorded once in 350 autopsies (Keith et ah, 1967) but the incidence in congenital heart disease varies between 3% (Campbell and Deuchar, 1954) and 4.3% (Fraser et al, 1961).
FIG. 2A. Case 1. Cine. Catheter in a persistent left superior vena cava. Contrast enters right atrium.
857
VOL.
52, No. 623 J. P. Owen and W. Urquhart
FIG. 3A. Case 2. Cine. Injection into left superior vena cava. The contrast opacities a communicating vein to the right superior vena cava. The LSVC drains to the right atrium.
FIG. 2B. Case 1. Two-plane angiogram. Late stages of a selective right ventricular angiogram showing the left atrium with a distmctive notch on the inferior border.
FIG. 3B. Case 2.
858
NOVEMBER 1979
The left atrial notch: a sign of persistent left superior vena cava draining to the right atrium
shadow of LSVC
\ mitral valve notch FIG. 3B. Case 2. Two-plane angiogram. Late stages following right ventricular angiogram. The left atrium, left ventricle and aorta opacify. A distinct notch is seen in the inferior border of the left atrium, the mediastinal shadow is widened on the left due to the persistent left superior vena cava.
TIG. -tA. Case J. Cine. Injection into left superior vena cava which drains into the right atrium.
FIG. 4B. Case 3. Cine. Injection into left atrium demonstrating a notch on the inferior border of the left atrium and a hypoplastic stenosed mitral valve.
859
VOL.
52, No. 623 J. P. Owen and W. Urquhart LSVC may occur either as an isolated anomaly or in association with other congenital malformations such as total anomalous pulmonary venous drainage, transposition of the great arteries, ventricular septal defects and persistent ductus arteriosus. Wood (1968) records it in 5% of atrial septal defects, 22% of atrio-ventricular canal defects, and 20% of cases with Fallots tetrology. It has also been found in association with tricuspid atresia (Campbell and Deuchar, 1954), pulmonary stenosis or atresia (Campbell and Deuchar, 1954; Fraser et al., 1961) and asplenia (Randall et al., 1973; Snellen and Dekker, 1963). A persistent LSVC can sometimes be identified on plain chest films as a soft tissue density in the upper left paramediastinum, though often this cannot be distinguished in young infants from a thymic shadow. At cardiac catheterization the catheter may pass into the anomalous vein and then a direct angiographic demonstration is possible (Figs. 2A, 3A, 4A). Indirect angiography by contrast injections into the left subclavian or left innominate vein is also of value. This paper presents another sign for suspecting the presence of a left superior vena cava. It is postulated that a notch on the inferior border of the left atrium is caused by a dilated coronary sinus receiving blood directly from a persistent left superior vena cava. In the experience of one of us (W. U.) it is a constant finding in cases with a LSVC when the left atrium is normal in size, but it is less marked when there is total anomalous pulmonary venous drainage and the left atrium is small. We have not seen this angiographic change in any other clinical context nor have we seen previous reports of it in the literature. ACKNOWLEDGMENTS
I would like to thank Mr. Lidington of the Department of Graphics at the University of Newcastle upon Tyne for the line diagrams; Dr. P. M. Hacking for his constructive comments; The Department of Photography at the University of Newcastle upon Tyne for the photographic prints and Miss Sheila Tate, Mrs. A. Hall and Mrs. M. Shepherd for valued secretarial help.
left atrial appendage
REFERENCES CAFFEY, J., 1973. In Paediatric X-ray Diagnosis, 6th edn. I pp. 504-508 (Year Book Medical Publications).
mitral valve
CAMPBELL, M. and DEUCHAR, D. C , 1954. The left-sided
superior vena cava. British Heart Journal, 16, 423—439. FRASER, R. S., DVORKIN, J., ROSSALL, R. E. and EIDEM, R.,
notch FIG. 5. Case 4. Two-plane angiogram. Selective left ventricular angiogram showing mitral regurgitation, a notch on the inferior border of the left atrium and widening of the left of the mediastinum due to the persistent left superior vena cava.
1961. Left superior vena cava. American Journal of Medicine, 31, 711-716. GARDNER, F. and ORAM, S., 1953. Persistent left superior vena cava draining into the pulmonary veins. British Heart Journal, 75,305-318. KEITH, J. D., ROWE, R. D. and VLAD, P., 1967. In Heart
Disease in Infancy and Childhood, 2nd edn. pp. 493-496. (The Macmillan Company), MCMANUS, J. F. A., 1941. (Cited by Gardner and Oram) Canadian Medical Association Journal, 45, 261.
860
NOVEMBER
1979
The left atrial notch: a sign of persistent left superior vena cava draining to the right atrium 1973. monary venous drainage in relation to left superior vena The spleen and congenital heart disease. American cava and coronary sinus. American Heart Journal, 66, 184Journal of Roentgenology, Radium Therapy and Nuclear 196. Medicine', 119, 551 -559. WOOD, P., 1968. Diseases of Heart and Circulation (Eyre and Spottiswoode) pp. 423, 436 and 518. SNELLEN, H. A. and DEKKER, K. 1963. Anomalous pul-
RANDALL, P. A., MOLLER, J. M. and AMPLATZ, K.,
Book reviews Atlas of Pediatric Nuclear Medicine. By P. O. Alderson, D. L. Gilday, H. N. Wagner, Jr, 1978, pp. xv+296 (YB Medical Publishers, London), £30-50. ISBN 0-8016-0107-X At a time when nuclear medicine is beginning to become established in children's hospitals in this country, it is salutary to realize that this atlas is based largely on the experience of Dr. Gilday from the Hospital for Sick Children in Toronto, acquired over the past eight years. The arrival of an up-to-date text is to be welcomed as there has been no reference work on the subject published since 1974. The layout of the book is more comprehensive than many atlases; after a short introduction, the chapters are arranged by systems, each of which begins with a detailed description of the method employed to obtain diagnostic images. This advice will be invaluable to the radiologist beginning these procedures, and will avoid the false positive errors, for instance in the diagnosis of Meckel's diverticulum, which are so damaging to the reputation of a new diagnostic facility. The chapters are then presented in the fashionable "problem-orientated" approach with good clinical detail and excellent illustrations of the appropriate X-rays and scans. Also included in the text are tables and references which are well chosen and up to 1978. But this book is not only for the radiologist doing paediatric scanning; paediatricians would be well advised to browse through and rethink more traditional and invasive methods of investigation, and the nuclear medicine specialist will find information about unusual childhood conditions ranging from the bone scan appearance in Engelmann's disease to the diagnosis of transient myocardial ischaemia in the newborn diagnosed by 201thallium scintigraphy. This atlas is recommended for your department bookshelf.
Diagnostic radiology in clinical medicine. By Rosalind H. Troupin, 2nd ed. 1978 pp. xiii + 172. (Year Book Medical Publishers, Chicago) £9-25. ISBN0-8151-8851-X. This small paperback aims to present diagnostic radiology including nuclear medicine, ultrasound and CT scanning, to clinical medical students. There are ten chapters in 1 54 pages on such topics as chest films, bone films, gastrointestinal examinations, special imaging techniques, etc. and an epilogue (which consists of some answers to questions posed in the text and a bibliography surprisingly detailed for medical students). There are plenty of small, clear illustrations with some helpful diagrams and correlations of the appearances of the same lesion {e.g. a renal cyst) shown by differing imaging techniques. There are brief outlines of suggested method of examination of films, followed by very concise summaries of some radiological signs and the appearances in various clinical situations. The material selected ranges from the simple (e.g. pneumothorax, pneumonia etc.) to the more exotic (e.g. bleeding from a colonic diverticulum demonstrated by arteriography). There are plenty of Americanisms and an interesting list of "typical" changes combining technical and professional fees, for radiographic examinations in the USA. In such a concise book much must be and is omitted. The selection of the material is obviously a matter of personal preference of the author. My general impression of the book presents a clear interesting review of radiology, integrating its various branches well. The presentation is, naturally and rightly, aimed at American students who may well find the cost is justified by the clear writing, good illustrations and comprehensive cover. Time will tell whether British students will react as favourably.
S. T. MELLER.
861
DAVID H. TRAPNELL.
NOVEMBER 1979
The left atrial notch: a sign of persistent left superior vena cava draining to the right atrium By J. P. Owen, M.B., B.S., D.M.R.D., F.R.C.R. Department of Diagnostic Radiology, University of Newcastle upon Tyne and W. Urquhart, M.B., Ch.B., D.M.R.D., F.R.C.R.* Late Newcastle General Hospital and the Regional Cardiothoracic Centre, Freeman Hospital, Newcastle upon Tyne {Received May 1979) ABSTRACT
A notch in the inferior border of the left atrium has been observed in patients with a persistent left superior vena cava draining via the coronary sinus into the right atrium. It is suggested that the notch is due to the dilated coronary sinus. Persistence of the left superior vena cava (LSVC) is encountered in a number of children with congenital heart disease and its pre-operative diagnosis may be important to the cardiac surgeon. For example, in patients undergoing open-heart surgery by extracorporeal by-pass an unsuspected LSVC could result in a troublesome inflow of blood into the right atrium. The presence of a LSVC and left innominate *This paper is based on an original observation by Dr. Urquhart though he did not live to see the final form of the publication. His wisdom, knowledge and expertise are greatly missed.
would allow a right SVC to be used in the repair of a sinus venosus atrial septal defect and the larger SVC could be used when a cavo-pulmonary shunt is to be created. This paper presents another sign for suspecting a left superior vena cava (LSVC) at cardiac catheterization. MATERIALS AND RESULTS
Table I records the angiographic findings in four patients in all of whom a notch was present on the inferior border of the left atrium in association with a left persistent superior vena cava draining via the coronary sinus into the right atrium. A possible embryological and anatomical mechanism for production of the atrial notch is postulated in Fig. 1.
TABLE I ANGIOGRAPHIC FINDINGS
Case
Sex, age
Findings
Catheter passed to LSVC
Notch present on inferior border of left atrium
1
F 3.5 months Figs. 2A, 2B
Pulmonary valve stenois Pulmonary infundibular stenosis VSD Right sided aorta Left superior vena cava draining to coronary sinus
Yes
Yes
2
M 1 year 10 months Figs. 3A, 3B
Right ventricular hypertrophy Infundibular pulmonary stenosis Pulmonary valve stenosis VSD Left superior vena cava draining to coronary sinus
Yes
Yes
3
M 8 weeks Figs. 4A, 4B
Transportation of great vessels Hypoplastic stenosed mitral valve VSD Left superior vena cava draining to coronary sinus
Yes
Yes
4
F 10 years Fig. 5
Mitral regurgitation Left superior vena cava draining to coronary sinus
Yes
Yes
855
VOL.
52, No. 623 J. P. Owen and W. Urquhart
ac
v pc B.
A
a sp sv dc
4 weeks — common atrium u - septum primum V ac — sinus venosus pc - duct of Cuvier
-
dv L sub isa
umbilical vein vitelline vein anterior cardinal vein posterior cardinal vein
5J weeks - ductus venosus - Liver — subcardinal vein — intersubcardinal anastomosis
ss az h.az IVC
sup ivc h
— supracardinal veins — inferior vena cava - left innominate vein
superior vena cava left innominate vein septum secuncfum azygos vein h.az — hemi-azygos vein SVC
li
FIG.
1.
856
NOVEMBER 1979
The left atrial notch: a sign of persistent left superior vena cava draining to the right atrium
Isvc cor
cor
E.
8 weeks Normal anatomy lm ov cor
F.
Persistent left superior vena cava draining into coronary sinus. Isvc - left superior vena cava cor - coronary sinus
ligament of Marshall oblique vein coronary sinus
FIG. 1. The normal development of the venous system in the human embryo and a postulated mechanism for the production of the left atrial notch.
The angio-graphic appearances are illustrated in Figs. 2-5. DISCUSSION
It is suggested (Caffey, 1973) that a left superior vena cava (LSVC) should be defined as a structure which receives the hemiazygos vein and has a communication with the coronary sinus or a communication with the left atrium when there is no coronary sinus. In the absence of these features the vein is best described as a vertical vein. McManus (1941), cited by Gardner and Oram (1953), classifies the various types of LSVC into: 1. Persistent superior vena cava (SVC) with right SVC (bilateral SVC). (a) Persistent LSVC connected to the coronary sinus and either with a cross anastomosis (left innominate vein) or without this. (b) Persistent LSVC draining the pulmonary veins. 2. Persistent LSVC without right SVC. A persistent LSVC has been recorded once in 350 autopsies (Keith et ah, 1967) but the incidence in congenital heart disease varies between 3% (Campbell and Deuchar, 1954) and 4.3% (Fraser et al, 1961).
FIG. 2A. Case 1. Cine. Catheter in a persistent left superior vena cava. Contrast enters right atrium.
857
VOL.
52, No. 623 J. P. Owen and W. Urquhart
FIG. 3A. Case 2. Cine. Injection into left superior vena cava. The contrast opacities a communicating vein to the right superior vena cava. The LSVC drains to the right atrium.
FIG. 2B. Case 1. Two-plane angiogram. Late stages of a selective right ventricular angiogram showing the left atrium with a distmctive notch on the inferior border.
FIG. 3B. Case 2.
858
NOVEMBER 1979
The left atrial notch: a sign of persistent left superior vena cava draining to the right atrium
shadow of LSVC
\ mitral valve notch FIG. 3B. Case 2. Two-plane angiogram. Late stages following right ventricular angiogram. The left atrium, left ventricle and aorta opacify. A distinct notch is seen in the inferior border of the left atrium, the mediastinal shadow is widened on the left due to the persistent left superior vena cava.
TIG. -tA. Case J. Cine. Injection into left superior vena cava which drains into the right atrium.
FIG. 4B. Case 3. Cine. Injection into left atrium demonstrating a notch on the inferior border of the left atrium and a hypoplastic stenosed mitral valve.
859
VOL.
52, No. 623 J. P. Owen and W. Urquhart LSVC may occur either as an isolated anomaly or in association with other congenital malformations such as total anomalous pulmonary venous drainage, transposition of the great arteries, ventricular septal defects and persistent ductus arteriosus. Wood (1968) records it in 5% of atrial septal defects, 22% of atrio-ventricular canal defects, and 20% of cases with Fallots tetrology. It has also been found in association with tricuspid atresia (Campbell and Deuchar, 1954), pulmonary stenosis or atresia (Campbell and Deuchar, 1954; Fraser et al., 1961) and asplenia (Randall et al., 1973; Snellen and Dekker, 1963). A persistent LSVC can sometimes be identified on plain chest films as a soft tissue density in the upper left paramediastinum, though often this cannot be distinguished in young infants from a thymic shadow. At cardiac catheterization the catheter may pass into the anomalous vein and then a direct angiographic demonstration is possible (Figs. 2A, 3A, 4A). Indirect angiography by contrast injections into the left subclavian or left innominate vein is also of value. This paper presents another sign for suspecting the presence of a left superior vena cava. It is postulated that a notch on the inferior border of the left atrium is caused by a dilated coronary sinus receiving blood directly from a persistent left superior vena cava. In the experience of one of us (W. U.) it is a constant finding in cases with a LSVC when the left atrium is normal in size, but it is less marked when there is total anomalous pulmonary venous drainage and the left atrium is small. We have not seen this angiographic change in any other clinical context nor have we seen previous reports of it in the literature. ACKNOWLEDGMENTS
I would like to thank Mr. Lidington of the Department of Graphics at the University of Newcastle upon Tyne for the line diagrams; Dr. P. M. Hacking for his constructive comments; The Department of Photography at the University of Newcastle upon Tyne for the photographic prints and Miss Sheila Tate, Mrs. A. Hall and Mrs. M. Shepherd for valued secretarial help.
left atrial appendage
REFERENCES CAFFEY, J., 1973. In Paediatric X-ray Diagnosis, 6th edn. I pp. 504-508 (Year Book Medical Publications).
mitral valve
CAMPBELL, M. and DEUCHAR, D. C , 1954. The left-sided
superior vena cava. British Heart Journal, 16, 423—439. FRASER, R. S., DVORKIN, J., ROSSALL, R. E. and EIDEM, R.,
notch FIG. 5. Case 4. Two-plane angiogram. Selective left ventricular angiogram showing mitral regurgitation, a notch on the inferior border of the left atrium and widening of the left of the mediastinum due to the persistent left superior vena cava.
1961. Left superior vena cava. American Journal of Medicine, 31, 711-716. GARDNER, F. and ORAM, S., 1953. Persistent left superior vena cava draining into the pulmonary veins. British Heart Journal, 75,305-318. KEITH, J. D., ROWE, R. D. and VLAD, P., 1967. In Heart
Disease in Infancy and Childhood, 2nd edn. pp. 493-496. (The Macmillan Company), MCMANUS, J. F. A., 1941. (Cited by Gardner and Oram) Canadian Medical Association Journal, 45, 261.
860
NOVEMBER
1979
The left atrial notch: a sign of persistent left superior vena cava draining to the right atrium 1973. monary venous drainage in relation to left superior vena The spleen and congenital heart disease. American cava and coronary sinus. American Heart Journal, 66, 184Journal of Roentgenology, Radium Therapy and Nuclear 196. Medicine', 119, 551 -559. WOOD, P., 1968. Diseases of Heart and Circulation (Eyre and Spottiswoode) pp. 423, 436 and 518. SNELLEN, H. A. and DEKKER, K. 1963. Anomalous pul-
RANDALL, P. A., MOLLER, J. M. and AMPLATZ, K.,
Book reviews Atlas of Pediatric Nuclear Medicine. By P. O. Alderson, D. L. Gilday, H. N. Wagner, Jr, 1978, pp. xv+296 (YB Medical Publishers, London), £30-50. ISBN 0-8016-0107-X At a time when nuclear medicine is beginning to become established in children's hospitals in this country, it is salutary to realize that this atlas is based largely on the experience of Dr. Gilday from the Hospital for Sick Children in Toronto, acquired over the past eight years. The arrival of an up-to-date text is to be welcomed as there has been no reference work on the subject published since 1974. The layout of the book is more comprehensive than many atlases; after a short introduction, the chapters are arranged by systems, each of which begins with a detailed description of the method employed to obtain diagnostic images. This advice will be invaluable to the radiologist beginning these procedures, and will avoid the false positive errors, for instance in the diagnosis of Meckel's diverticulum, which are so damaging to the reputation of a new diagnostic facility. The chapters are then presented in the fashionable "problem-orientated" approach with good clinical detail and excellent illustrations of the appropriate X-rays and scans. Also included in the text are tables and references which are well chosen and up to 1978. But this book is not only for the radiologist doing paediatric scanning; paediatricians would be well advised to browse through and rethink more traditional and invasive methods of investigation, and the nuclear medicine specialist will find information about unusual childhood conditions ranging from the bone scan appearance in Engelmann's disease to the diagnosis of transient myocardial ischaemia in the newborn diagnosed by 201thallium scintigraphy. This atlas is recommended for your department bookshelf.
Diagnostic radiology in clinical medicine. By Rosalind H. Troupin, 2nd ed. 1978 pp. xiii + 172. (Year Book Medical Publishers, Chicago) £9-25. ISBN0-8151-8851-X. This small paperback aims to present diagnostic radiology including nuclear medicine, ultrasound and CT scanning, to clinical medical students. There are ten chapters in 1 54 pages on such topics as chest films, bone films, gastrointestinal examinations, special imaging techniques, etc. and an epilogue (which consists of some answers to questions posed in the text and a bibliography surprisingly detailed for medical students). There are plenty of small, clear illustrations with some helpful diagrams and correlations of the appearances of the same lesion {e.g. a renal cyst) shown by differing imaging techniques. There are brief outlines of suggested method of examination of films, followed by very concise summaries of some radiological signs and the appearances in various clinical situations. The material selected ranges from the simple (e.g. pneumothorax, pneumonia etc.) to the more exotic (e.g. bleeding from a colonic diverticulum demonstrated by arteriography). There are plenty of Americanisms and an interesting list of "typical" changes combining technical and professional fees, for radiographic examinations in the USA. In such a concise book much must be and is omitted. The selection of the material is obviously a matter of personal preference of the author. My general impression of the book presents a clear interesting review of radiology, integrating its various branches well. The presentation is, naturally and rightly, aimed at American students who may well find the cost is justified by the clear writing, good illustrations and comprehensive cover. Time will tell whether British students will react as favourably.
S. T. MELLER.
861
DAVID H. TRAPNELL.
