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A new surgical approach for correction of partial anomalous pulmonary venous drainage into the superior vena cava
A new surgical approach for correction of partial anomalous pulmonary venous drainage into the superior vena cava Nine children with partial anomalous pulmonary venous drainage into the superior vena cava were operated upon. The technique consisted essentially of partitioning and enlargement of the superior vena cava. The partitioning was done in all but one patient, with a longitudinal suture starting above the highest pulmonary vein directing the pulmonary venous flow through the enlarged atrial septal defect into the left atrium. The anterior cavo-auricular tunnel was enlarged with a right atrial appendage-superior vena cava angioplasty. Follow-up studies were done between 1 and 3 years after surgery. The hemodynamic data were normal in 7 patients. In 8 children, the superior vena cava was unobstructed and its diameter was normal as demonstrated by cavograms. In all patients, the angiographic evaluation of the pulmonary venous return was normal. These results are encouraging and indicate that this new approach is superior to those which have previously been reported.
Claude Chartrand, M.D., F.R.C.S.(C)* (by invitation), Maurice Payot, M.D. (by invitation), Andre Davignon, M.D., F.R.C.P.(C) (by invitation), Ronald Guerin, M.D., F.R.C.P.(C) (by invitation), and Paul Stanley, M.D. (by invitation), Montreal, Quebec, Canada Sponsored by Jacques Bruneau, M.D., F.R.C.S.(C), Montreal, Quebec, Canada
V-corrective techniques for partial anomalous pulmonary venous drainage into the superior vena cava have not been adequate up to the present time. Apart from mortality and immediate postoperative morbidity rates, a high rate of occlusion of the superior vena cava or of the pulmonary veins in addition to residual shunts was reported in follow-up studies. 1-4 Recently, our group5 presented a long-term evaluation of children treated for this anomaly by different surgical methods, and the incidence of late complications has been similar to that of other authors. These results prompted us to search for a better approach. The purpose of this paper is to describe a new surgical technique for correction of partial anomalous pulmonary venous drainage into the superior vena cava and to present the results of late follow-up evaluation. From the Section of Cardiovascular Surgery, Department of Surgery, Hopital Sainte-Justine, Universite de Montreal, 3175 Ch. Sainte-Catherine, Montreal H3T 1C5, Quebec, Canada. Read at the Fifty-fifth Annual Meeting of The American Association for Thoracic Surgery, New York, N. Y., April 14, 15, and 16, 1975. •Scholar of the Canadian Medical Research Council.
Clinical material Nine patients, aged 4 to 9 years (average 6 years, 2 months), had anomalous pulmonary veins entering the superior vena cava. One to three veins from the right superior or middle lobes drained into the superior vena cava. In 6 patients, a sinus venosus type of atrial septal defect was associated to the anomalous veins and in 2 an ostium secundum was present. Two children who had a left superior vena cava entering the coronary sinus presented with a small right superior vena cava. Surgical technique The operation is carried out through a median sternotomy. The anterior aspect of the vena cava is dissected free from the atrium to the azygos vein. The superior caval tourniquet is placed above the azygos vein, which has been ligated. The pulmonary venous return is inspected, and the anomalous veins are identified (Fig. 1). The caval catheters are introduced through the lateral wall of the right atrium in order to preserve the right atrial appendage. The tip of the superior caval catheter is positioned above the ligated azygos vein. Car29
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©
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© Fig. 1. Surgical technique. /, Caval catheters introduced through the lateral wall of the right atrium. Tip of the superior catheter positioned above the ligated azygos vein. Vertical incision extending from the apex of the right atrial appendage (point A) up the anterior aspect of the superior vena cava to a point above the highest anomalous pulmonary vein (point B). 2, Adequate exposure of the orifices of the anomalous veins and of the atrial septal defect. Atrial septum disinserted from the right inferior rim of the septal defect (point C) at a distance of 3 cm. in the direction of the inferior vena cava (point D). Approximating point C to point E will displace the free edge of the septum to the right. 3, Disinserted edge of the septum sutured to the right atrial wall from point £ to point D. Partitioning of the superior vena cava is begun above the entrance of the highest anomalous vein (point F) and carried out by impinging generously on the lateral walls of the superior vena cava at points far anterior to the orifices of the pulmonary veins as indicated by the dotted line. 4, Partitioning completed by continuous suture down to point E. Right atrial appendage-superior vena cava angioplasty carried out first by bringing in line the apex of the atrial appendage (point A) and the upper end of the incision on the superior vena cava (point B). 5, With point A approximated to point B, the auricular appendage is drawn upward and now overlies the incised superior vena cava. The anastomosis is completed by suturing the cut edges of the atrial appendage to those of the superior vena cava on both sides. RAA, Right atrial appendage, IVC, Inferior vena cava. SVC, Superior vena cava. AV, Azygos vein. PV, Pulmonary veins.
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Table I. Summary of hemodynamic data obtained in Case 5 (Patient M. T.) Site SVC (H) SVC (L) RA RV PA RPW LPW FA
Pressure (mm. Hg)
Oxygen sat.
4 4 5 22/0 18/8 11 11 105/70
73 75 75 75 75 97 97 97
(%)
L-R shunt
R-L shunt
0 0 0 0 0
_ -
-
0
Legend: Note the absence of pressure gradient across the angioplasty.the absence of shunts as assessed by oximetry and dilution curves, and the normal right pulmonary wedge pressure as compared to the left. Abbreviations: L-R shunt. Left-to-right shunt. R-Lshunt. Right-to-left shunt. SVC(H),Superior vena cava (high). SVC (L). Superior vena cava (low). RA. Right atrium. RV. Right ventricle. PA. Pulmonary artery. RPW. Right pulmonary wedge. LPW. Left pulmonary wedge. FA. Femoral artery.
diopulmonary bypass is instituted, and electrical fibrillation of the heart is induced to minimize chances of air embolism. A vertical incision extending from the apex of the right atrial appendage up the anterior aspect of the superior vena cava to a point cephalad to the highest anomalous pulmonary vein is carried out. The superior caval catheter is drawn upward, and the edges of the incision are retracted laterally with stay sutures. This gives excellent exposure of the orifices of the anomalous veins and of the atrial septal defect. The atrial septum is disinserted from the right inferior border of the septal defect for a distance of 3 cm. in the direction of the inferior vena cava. Bringing the inferior rim of the defect 2 cm. up on the right atrial wall moves the septum to the right. The disinserted edge of the septum is then sutured to the right atrial wall. This procedure not only widens the atrial septal defect but also orientates the partitioning of the superior vena cava. This partitioning is begun above the entrance of the highest anomalous pulmonary vein. It is carried out by impinging generously on the lateral walls of the superior vena cava at points far anterior to the orifices of the pulmonary veins. It is completed by continuous suture down to the inferior border of the septal defect after air has been expelled from the left cavities. The right pulmonary venous flow is now diverted to the left atrium through this wide posterior conduit and the enlarged atrial septal defect. The remaining portions of the vena cava are consequently reduced in width. Should they be sutured directly, the diameter of the superior vena cava would be reduced significantly causing obstruction. In order to enlarge the anterior cavo-auricular tunnel, a right atrial appendage-superior vena cava angioplasty is
Fig. 2. In Case 7, a 30 per cent stenosis of the superior vena cava is demonstrated angiographically. At the operation, this vein associated with a left superior vena cava measured only 6 mm. performed. This is begun by bringing in line the apex of the atrial appendage and the upper end of the incision on the superior vena cava. By approximating these two points, the auricular appendage is drawn upward and now overlies the incised superior vena cava. Anastomosis is completed by suturing the cut edges of the atrial appendage to those of the superior vena cava on both sides. This angioplasty creates a wide, free flowing cavo-auricular tunnel. To minimize further the risks of air embolism, needle aspiration of the cardiac cavities and venting of the aortic root are done before ventricular fibrillation is ended. The caval catheters are withdrawn to the right atrium and, after a short period of circulatory assistance, cardiopulmonary bypass is discontinued. Results All patients had an uneventful postoperative course. Follow-up studies carried out 1 to 3 years after surgery (an average of 21 months) included clinical, electrocardiographic, radiological, hemodynamic, and angiographic examinations. Clinically, all children were asymptomatic. Except for a Grade 2/6 systolic ejection murmur heard in 4 patients, findings on physical examination were normal. On electrocardiogram, right ventricular hypertrophy and right axis deviation had decreased. Prior to surgery, 2 patients had coronary sinus rhythm and 3 had negative P waves in D3. Postoperatively, coronary
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Fig. 3. This biplane angiogram obtained in Case 9 shows the unobstructed superior vena cava. The diameter of the new atriocaval tunnel is adequate. sinus rhythm was noted in 3 additional patients who had sinus rhythm and normal P-wave axis before surgery. On chest roentgenogram, pulmonary vascular markings and the cardiothoracic ratio had diminished significantly. These improvements were more pronounced in the children with the longest follow-up. Right heart catheterization was performed in all patients. Pressures were recorded in the superior vena cava above and below the site of angioplasty, and attention was paid to the right and left pulmonary wedge pressures. Cardiac index was determined, and the peripheral and pulmonary resistances calculated. The presence of shunts was searched for by oximerry, dye dilution, and hydrogen curves. At the end of the procedure, angiographic visualization of the superior vena cava and of the pulmonary venous return was performed. Hemodynamic findings were normal in 7 patients. The data obtained in Case 5 illustrate the absence of pressure gradient across the angioplasty, the absence of shunts as assessed by oximetry, dye dilution, and hydrogen curves, and also the normal right pulmonary wedge pressure as compared to the left (Table I). An 11 per cent left-to-right shunt was noted in Patient 3, in whom a 4 mm. anomalous pulmonary vein was left untouched at the time of the operation because of its small size and very high position. In Case 7, a 30 per cent stenosis of the superior vena
cava was demonstrated angiographically. At operation, this vein measured only 6 mm. and was associated with a left superior vena cava (Fig. 2). In 8 patients, the superior vena cava was unobstructed as illustrated on the biplane angiogram obtained in Case 9 (Fig. 3). Also, the diameter of the new atriocaval tunnel was adequate as demonstrated by anteroposterior and lateral cavograms. The angiographic evaluation of the pulmonary venous return was normal in all cases and correlated well with the normal wedge pressure. The veins of the right upper lobes, as compared to those of the left, were normal. The new pulmonary conduit was of good size and the right pulmonary flow was comparable to the left in every case (Fig. 4). Discussion Many surgical techniques have been proposed for correction of partial anomalous pulmonary venous drainage. These include atrioseptopexy,6, 7 suture of the lower margin of the atrial septal defect to the wall of the superior vena cava above the anomalous veins,8 simple partitioning of the superior vena cava,1, 2 partitioning with synthetic or pericardial grafts,2' 4' 9 widening of the remaining lumen of the superior vena cava with a pericardial patch,9' 10 and disinsertion and reimplantation of the anomalous veins into the left atrium.2' 3 ' 8 Partial corrections have also been advocated, such as leaving a high anomalous pulmonary
Volume 71 Number 1 January, 1976
vein untouched,1,8 ligature of a small anomalous vein,8 or lobectomy.3 Apart from operative deaths, obstruction of the superior vena cava or of the pulmonary veins and a high incidence of residual left-to-right or right-to-left shunts have been reported in the immediate postoperative phase. Follow-up studies including hemodynamic evaluation have been scarce and have shown the same incidence of residual shunts. However, postoperative angiographic evaluations of this anomaly have not been published by other groups than ours. Our report consisted of a long-term follow-up of 14 children treated for partial anomalous pulmonary venous return by the previously described techniques.5 Clinically, they were all asymptomatic and findings from their physical examinations were normal. However, cardiac catheterization and angiography demonstrated complications in 40 per cent of the cases. These occurred when anomalous drainage was to the superior vena cava and more so when the right superior vena cava, associated with a left superior vena cava, was small. These findings brought evidence that a definite risk was inherent to these techniques and that hemodynamic and angiographic data are necessary to evaluate the adequacy of surgical procedures aimed at correction of this anomaly. Included in this group were children who had angioplasty of the superior vena cava with a free pericardial graft and who presented at late follow-up the same incidence of narrowing or total obstruction of the superior vena cava. Because these complications occurred with the use of pericardial graft, we undertook experiments on dogs in order to investigate the pathophysiology of these obstructions.11 Acute inflammation of the pericardium used for replacement of the superior vena cava occurred in the first postoperative days and was followed shortly by retraction of the graft. Thrombus then formed on its inner surface, at which stage progressive tubular stenosis was demonstrated by angiography. As a rule, this was followed by total obstruction with development of collateral circulation within 15 days. In most instances, these occlusions did not become manifest clinically as a superior vena cava syndrome. These findings confirm the hazards of free pericardial graft on the superior vena cava. They also illustrate the fact that clinical examination does not always reveal underlying pathology brought to light by hemodynamic and angiographic data. The clinical experience of other groups along with our own and the above-mentioned experimental findings prompted us to search for a better approach to the correction of partial anomalous pulmonary venous drainage into the superior vena cava.
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Fig. 4. This angiogram obtained in Case 9 shows normal pulmonary venous return. The right pulmonary veins are normal as compared to the left, and the pulmonary venous conduit is of good size. The operation that we have described in this paper has been performed in 9 children with anomalous pulmonary venous return to the superior vena cava. The postoperative course has been good in all. However, coronary sinus rhythm occurred in 3 patients. This was of minor importance, since all had normal heart rates after the operation and no functional or hemodynamic consequences of this arrhythmia were observed. Surgical trauma of the sinus node or of the internodal tracts could be held responsible. However, implying trauma as the sole cause of this abnormality would be oversimplification, since 2 of these children had coronary sinus rhythm and 3 others had abnormal P-wave axis prior to the operation, such as Hancock12 reported. Slight narrowing of the superior vena cava occurred in one case only. The vena cava measured but 6 mm. in diameter and was associated to a left superior vena cava. Although exceptional, this complication may suggest that there is a potential hazard related to our surgical approach when the superior vena cava is of very small diameter. Yet, all patients had a patent superior vena cava, and, in 8 of the 9 children operated upon, the diameter of the new atriocaval tunnel was adequate as demonstrated by cavograms. Also, the pulmonary venous return was normal in all cases as documented by hemodynamic and angiographic evaluation. Finally, in all totally corrected patients, no shunts could be detected. These over-all results are
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encouraging and indicate that this new technique is superior to those which have previously been reported.
REFERENCES
1 Lewis, F. J.: High Defects of the Atrial Septum, J. THORAC. SURG. 36: 1,
1958.
2 Kirklin, J. W., Ellis, F. H., and Wood, E. H.: Treatment of Anomalous Pulmonary Venous Connections in Association With Interatrial Communications, Surgery 39: 389, 1956. 3 Risch, F., and Hahn, C : The Technique of Surgical Correction of Anomalies of the Pulmonary Veins in a Series of 25 Cases, Thorax 13: 251, 1958. 4 Lawrance, K., Grimshaw, V. A., Hoyle, G., Hicks, J. P. N., Nixon, P. F. G., and Wooler, G. H.: Surgical Treatment of Atrial Septal Defect and Partial Anomalous Pulmonary Venous Drainage, J. THORAC. CARDIOVASC. SURG. 43: 622,
1962.
5 Friedli, B., Guerin, R., Davignon, A., Fouron, J. C , and Stanley, P.: Surgical Treatment of Partial Anomalous Pulmonary Venous Drainage: A Long-Term Follow-up Study, Circulation 55: 159, 1972. 6 Bailey, C. P., Bolton, H. E., Jamison, W. L., and Neptune, W. B.: Atrio-septo-pexy for Interatrial Septal Defects, J. THORAC. SURG. 26:
184,
1953.
7 Neptune, W. B., Bailey, C. P., and Goldberg, H.: The Surgical Correction of Atrial Septal Defects Associated With Transposition of the Pulmonary Veins, J. THORAC SURG. 25: 623,
1953.
8 Brock, R., and Ross, D. N.: The Sinus Venosus Type of Atrial Septal Defect: Surgical Treatment, Guys Hosp. Rep. 108: 291, 1959. 9 Schuster, S. R., Gross, R. E., and Colodny, A. H.: Surgical Management of Anomalous Right Pulmonary Venous Drainage to the Superior Vena Cava, Associated With Superior Marginal Defect of the Atrial Septum, Surgery 51: 805, 1962. 10 Puig-Massana, M., Murta, M., and Revuelta, J. M.: A New Technique in the Correction of Partial Anomalous Pulmonary Venous Drainage, J. THORAC CARDIOVASC SURG. 64:
108,
1972.
11 Brais, M., Bertranou, E., Brassard, A., Stanley, P., and Chartrand, C : Effect of Dextran on Patency of Pericardial Tubular Graft of the Superior Vena Cava in the Dog, J. THORAC CARDIOVASC SURG. 65: 296,
1973.
12 Hancock, E. W.: Coronary Sinus Rhythm in Sinus Venosus Defect and Persistent Left Superior Vena Cava, Am. J. Cardiol. 14: 608, 1964.
Discussion D R . N I C H O L A S J.
DEMOS
Short Hills, N. J.
I would like to present a very interesting case of ours. A 15-year-old girl complained of easy fatigability. Eight years previously she had had closure of an atrial septal defect.
On physical examination, she was very small for her age. She had the very interesting physical finding of a very loud systolic hum in the right supraclavicular area medially. Cardiac catheterization revealed a 2:1 left-to-right shunt in the supracardiac level, a bilateral upper lobe, and anomalous venous drainage to the superior vena cava. Catheterization through systemic left arm vein revealed the anomalous pulmonary veins on the left side and one on the right side. At the operation, the right upper lobe pulmonary anomalous vein was ligated, being small in size, and the left was transplanted to the left atrium in a very slant fashion. As postoperative arteriograms indicated, the anastomosis is patent 6 months later. DR. ROY H. C L A U S S New York, N. Y.
Dr. Reed, who was unable to be here this afternoon, asked me to comment on the paper he and his former associates published in Surgery, Gynecology and Obstetrics in December, 1962. In essence, the procedure was similar in that it did bivalve the right atrial appendage and bring point A down to point B, as Dr. Chartrand mentioned. Much emphasis was just placed on this latest procedure being superior to those previously described. We would concede that since we used a patch and Dr. Chartrand's group does not, they may have a superior procedure. The remainder of our technique was the same. There is one other aspect in which the current authors may have superior results: We have not performed the late follow-up studies by cardiac catheterization, being satisfied with the clinical results. D R . C H A R T R A N D (Closing) I would like to thank the discussers for their comments. Should a pericardial patch be used on the superior vena cava as proposed by Schuster and other authors? In the previous study that I mentioned in my article, a free pericardial patch was used to widen the superior vena cava in 5 patients. Unfortunately, hemodynamic and angiographic examination demonstrated narrowing or total obstruction in 40 per cent of the cases. Because of these results, experiments were carried out on dogs in order to elucidate the pathophysiology of this complication. Part of the superior vena cava was replaced by a fresh pericardial patch in normal dogs. Within a few days after surgery, acute inflammation of the patch occurred followed by retraction and formation of fibrinous thrombus on the inner surface of that pericardium. At this stage, progressive tubular stenosis was demonstrated angiographically. Within 14 days after the operation, mixed thrombus completed the obstruction; at this stage collateral circulation was well developed. These experimental findings as well as our previous clinical results prompted us to abandon the use of pericardial patch on the superior vena cava and to develop the technique that I have just described.
Claude Chartrand, M.D., F.R.C.S.(C)* (by invitation), Maurice Payot, M.D. (by invitation), Andre Davignon, M.D., F.R.C.P.(C) (by invitation), Ronald Guerin, M.D., F.R.C.P.(C) (by invitation), and Paul Stanley, M.D. (by invitation), Montreal, Quebec, Canada Sponsored by Jacques Bruneau, M.D., F.R.C.S.(C), Montreal, Quebec, Canada
V-corrective techniques for partial anomalous pulmonary venous drainage into the superior vena cava have not been adequate up to the present time. Apart from mortality and immediate postoperative morbidity rates, a high rate of occlusion of the superior vena cava or of the pulmonary veins in addition to residual shunts was reported in follow-up studies. 1-4 Recently, our group5 presented a long-term evaluation of children treated for this anomaly by different surgical methods, and the incidence of late complications has been similar to that of other authors. These results prompted us to search for a better approach. The purpose of this paper is to describe a new surgical technique for correction of partial anomalous pulmonary venous drainage into the superior vena cava and to present the results of late follow-up evaluation. From the Section of Cardiovascular Surgery, Department of Surgery, Hopital Sainte-Justine, Universite de Montreal, 3175 Ch. Sainte-Catherine, Montreal H3T 1C5, Quebec, Canada. Read at the Fifty-fifth Annual Meeting of The American Association for Thoracic Surgery, New York, N. Y., April 14, 15, and 16, 1975. •Scholar of the Canadian Medical Research Council.
Clinical material Nine patients, aged 4 to 9 years (average 6 years, 2 months), had anomalous pulmonary veins entering the superior vena cava. One to three veins from the right superior or middle lobes drained into the superior vena cava. In 6 patients, a sinus venosus type of atrial septal defect was associated to the anomalous veins and in 2 an ostium secundum was present. Two children who had a left superior vena cava entering the coronary sinus presented with a small right superior vena cava. Surgical technique The operation is carried out through a median sternotomy. The anterior aspect of the vena cava is dissected free from the atrium to the azygos vein. The superior caval tourniquet is placed above the azygos vein, which has been ligated. The pulmonary venous return is inspected, and the anomalous veins are identified (Fig. 1). The caval catheters are introduced through the lateral wall of the right atrium in order to preserve the right atrial appendage. The tip of the superior caval catheter is positioned above the ligated azygos vein. Car29
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©
©
©
© Fig. 1. Surgical technique. /, Caval catheters introduced through the lateral wall of the right atrium. Tip of the superior catheter positioned above the ligated azygos vein. Vertical incision extending from the apex of the right atrial appendage (point A) up the anterior aspect of the superior vena cava to a point above the highest anomalous pulmonary vein (point B). 2, Adequate exposure of the orifices of the anomalous veins and of the atrial septal defect. Atrial septum disinserted from the right inferior rim of the septal defect (point C) at a distance of 3 cm. in the direction of the inferior vena cava (point D). Approximating point C to point E will displace the free edge of the septum to the right. 3, Disinserted edge of the septum sutured to the right atrial wall from point £ to point D. Partitioning of the superior vena cava is begun above the entrance of the highest anomalous vein (point F) and carried out by impinging generously on the lateral walls of the superior vena cava at points far anterior to the orifices of the pulmonary veins as indicated by the dotted line. 4, Partitioning completed by continuous suture down to point E. Right atrial appendage-superior vena cava angioplasty carried out first by bringing in line the apex of the atrial appendage (point A) and the upper end of the incision on the superior vena cava (point B). 5, With point A approximated to point B, the auricular appendage is drawn upward and now overlies the incised superior vena cava. The anastomosis is completed by suturing the cut edges of the atrial appendage to those of the superior vena cava on both sides. RAA, Right atrial appendage, IVC, Inferior vena cava. SVC, Superior vena cava. AV, Azygos vein. PV, Pulmonary veins.
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Table I. Summary of hemodynamic data obtained in Case 5 (Patient M. T.) Site SVC (H) SVC (L) RA RV PA RPW LPW FA
Pressure (mm. Hg)
Oxygen sat.
4 4 5 22/0 18/8 11 11 105/70
73 75 75 75 75 97 97 97
(%)
L-R shunt
R-L shunt
0 0 0 0 0
_ -
-
0
Legend: Note the absence of pressure gradient across the angioplasty.the absence of shunts as assessed by oximetry and dilution curves, and the normal right pulmonary wedge pressure as compared to the left. Abbreviations: L-R shunt. Left-to-right shunt. R-Lshunt. Right-to-left shunt. SVC(H),Superior vena cava (high). SVC (L). Superior vena cava (low). RA. Right atrium. RV. Right ventricle. PA. Pulmonary artery. RPW. Right pulmonary wedge. LPW. Left pulmonary wedge. FA. Femoral artery.
diopulmonary bypass is instituted, and electrical fibrillation of the heart is induced to minimize chances of air embolism. A vertical incision extending from the apex of the right atrial appendage up the anterior aspect of the superior vena cava to a point cephalad to the highest anomalous pulmonary vein is carried out. The superior caval catheter is drawn upward, and the edges of the incision are retracted laterally with stay sutures. This gives excellent exposure of the orifices of the anomalous veins and of the atrial septal defect. The atrial septum is disinserted from the right inferior border of the septal defect for a distance of 3 cm. in the direction of the inferior vena cava. Bringing the inferior rim of the defect 2 cm. up on the right atrial wall moves the septum to the right. The disinserted edge of the septum is then sutured to the right atrial wall. This procedure not only widens the atrial septal defect but also orientates the partitioning of the superior vena cava. This partitioning is begun above the entrance of the highest anomalous pulmonary vein. It is carried out by impinging generously on the lateral walls of the superior vena cava at points far anterior to the orifices of the pulmonary veins. It is completed by continuous suture down to the inferior border of the septal defect after air has been expelled from the left cavities. The right pulmonary venous flow is now diverted to the left atrium through this wide posterior conduit and the enlarged atrial septal defect. The remaining portions of the vena cava are consequently reduced in width. Should they be sutured directly, the diameter of the superior vena cava would be reduced significantly causing obstruction. In order to enlarge the anterior cavo-auricular tunnel, a right atrial appendage-superior vena cava angioplasty is
Fig. 2. In Case 7, a 30 per cent stenosis of the superior vena cava is demonstrated angiographically. At the operation, this vein associated with a left superior vena cava measured only 6 mm. performed. This is begun by bringing in line the apex of the atrial appendage and the upper end of the incision on the superior vena cava. By approximating these two points, the auricular appendage is drawn upward and now overlies the incised superior vena cava. Anastomosis is completed by suturing the cut edges of the atrial appendage to those of the superior vena cava on both sides. This angioplasty creates a wide, free flowing cavo-auricular tunnel. To minimize further the risks of air embolism, needle aspiration of the cardiac cavities and venting of the aortic root are done before ventricular fibrillation is ended. The caval catheters are withdrawn to the right atrium and, after a short period of circulatory assistance, cardiopulmonary bypass is discontinued. Results All patients had an uneventful postoperative course. Follow-up studies carried out 1 to 3 years after surgery (an average of 21 months) included clinical, electrocardiographic, radiological, hemodynamic, and angiographic examinations. Clinically, all children were asymptomatic. Except for a Grade 2/6 systolic ejection murmur heard in 4 patients, findings on physical examination were normal. On electrocardiogram, right ventricular hypertrophy and right axis deviation had decreased. Prior to surgery, 2 patients had coronary sinus rhythm and 3 had negative P waves in D3. Postoperatively, coronary
The Journal of Thoracic and Cardiovascular Surgery
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Fig. 3. This biplane angiogram obtained in Case 9 shows the unobstructed superior vena cava. The diameter of the new atriocaval tunnel is adequate. sinus rhythm was noted in 3 additional patients who had sinus rhythm and normal P-wave axis before surgery. On chest roentgenogram, pulmonary vascular markings and the cardiothoracic ratio had diminished significantly. These improvements were more pronounced in the children with the longest follow-up. Right heart catheterization was performed in all patients. Pressures were recorded in the superior vena cava above and below the site of angioplasty, and attention was paid to the right and left pulmonary wedge pressures. Cardiac index was determined, and the peripheral and pulmonary resistances calculated. The presence of shunts was searched for by oximerry, dye dilution, and hydrogen curves. At the end of the procedure, angiographic visualization of the superior vena cava and of the pulmonary venous return was performed. Hemodynamic findings were normal in 7 patients. The data obtained in Case 5 illustrate the absence of pressure gradient across the angioplasty, the absence of shunts as assessed by oximetry, dye dilution, and hydrogen curves, and also the normal right pulmonary wedge pressure as compared to the left (Table I). An 11 per cent left-to-right shunt was noted in Patient 3, in whom a 4 mm. anomalous pulmonary vein was left untouched at the time of the operation because of its small size and very high position. In Case 7, a 30 per cent stenosis of the superior vena
cava was demonstrated angiographically. At operation, this vein measured only 6 mm. and was associated with a left superior vena cava (Fig. 2). In 8 patients, the superior vena cava was unobstructed as illustrated on the biplane angiogram obtained in Case 9 (Fig. 3). Also, the diameter of the new atriocaval tunnel was adequate as demonstrated by anteroposterior and lateral cavograms. The angiographic evaluation of the pulmonary venous return was normal in all cases and correlated well with the normal wedge pressure. The veins of the right upper lobes, as compared to those of the left, were normal. The new pulmonary conduit was of good size and the right pulmonary flow was comparable to the left in every case (Fig. 4). Discussion Many surgical techniques have been proposed for correction of partial anomalous pulmonary venous drainage. These include atrioseptopexy,6, 7 suture of the lower margin of the atrial septal defect to the wall of the superior vena cava above the anomalous veins,8 simple partitioning of the superior vena cava,1, 2 partitioning with synthetic or pericardial grafts,2' 4' 9 widening of the remaining lumen of the superior vena cava with a pericardial patch,9' 10 and disinsertion and reimplantation of the anomalous veins into the left atrium.2' 3 ' 8 Partial corrections have also been advocated, such as leaving a high anomalous pulmonary
Volume 71 Number 1 January, 1976
vein untouched,1,8 ligature of a small anomalous vein,8 or lobectomy.3 Apart from operative deaths, obstruction of the superior vena cava or of the pulmonary veins and a high incidence of residual left-to-right or right-to-left shunts have been reported in the immediate postoperative phase. Follow-up studies including hemodynamic evaluation have been scarce and have shown the same incidence of residual shunts. However, postoperative angiographic evaluations of this anomaly have not been published by other groups than ours. Our report consisted of a long-term follow-up of 14 children treated for partial anomalous pulmonary venous return by the previously described techniques.5 Clinically, they were all asymptomatic and findings from their physical examinations were normal. However, cardiac catheterization and angiography demonstrated complications in 40 per cent of the cases. These occurred when anomalous drainage was to the superior vena cava and more so when the right superior vena cava, associated with a left superior vena cava, was small. These findings brought evidence that a definite risk was inherent to these techniques and that hemodynamic and angiographic data are necessary to evaluate the adequacy of surgical procedures aimed at correction of this anomaly. Included in this group were children who had angioplasty of the superior vena cava with a free pericardial graft and who presented at late follow-up the same incidence of narrowing or total obstruction of the superior vena cava. Because these complications occurred with the use of pericardial graft, we undertook experiments on dogs in order to investigate the pathophysiology of these obstructions.11 Acute inflammation of the pericardium used for replacement of the superior vena cava occurred in the first postoperative days and was followed shortly by retraction of the graft. Thrombus then formed on its inner surface, at which stage progressive tubular stenosis was demonstrated by angiography. As a rule, this was followed by total obstruction with development of collateral circulation within 15 days. In most instances, these occlusions did not become manifest clinically as a superior vena cava syndrome. These findings confirm the hazards of free pericardial graft on the superior vena cava. They also illustrate the fact that clinical examination does not always reveal underlying pathology brought to light by hemodynamic and angiographic data. The clinical experience of other groups along with our own and the above-mentioned experimental findings prompted us to search for a better approach to the correction of partial anomalous pulmonary venous drainage into the superior vena cava.
Partial anomalous pulmonary venous drainage
33
Fig. 4. This angiogram obtained in Case 9 shows normal pulmonary venous return. The right pulmonary veins are normal as compared to the left, and the pulmonary venous conduit is of good size. The operation that we have described in this paper has been performed in 9 children with anomalous pulmonary venous return to the superior vena cava. The postoperative course has been good in all. However, coronary sinus rhythm occurred in 3 patients. This was of minor importance, since all had normal heart rates after the operation and no functional or hemodynamic consequences of this arrhythmia were observed. Surgical trauma of the sinus node or of the internodal tracts could be held responsible. However, implying trauma as the sole cause of this abnormality would be oversimplification, since 2 of these children had coronary sinus rhythm and 3 others had abnormal P-wave axis prior to the operation, such as Hancock12 reported. Slight narrowing of the superior vena cava occurred in one case only. The vena cava measured but 6 mm. in diameter and was associated to a left superior vena cava. Although exceptional, this complication may suggest that there is a potential hazard related to our surgical approach when the superior vena cava is of very small diameter. Yet, all patients had a patent superior vena cava, and, in 8 of the 9 children operated upon, the diameter of the new atriocaval tunnel was adequate as demonstrated by cavograms. Also, the pulmonary venous return was normal in all cases as documented by hemodynamic and angiographic evaluation. Finally, in all totally corrected patients, no shunts could be detected. These over-all results are
34
The Journal of Thoracic and Cardiovascular Surgery
Chartrand et al.
encouraging and indicate that this new technique is superior to those which have previously been reported.
REFERENCES
1 Lewis, F. J.: High Defects of the Atrial Septum, J. THORAC. SURG. 36: 1,
1958.
2 Kirklin, J. W., Ellis, F. H., and Wood, E. H.: Treatment of Anomalous Pulmonary Venous Connections in Association With Interatrial Communications, Surgery 39: 389, 1956. 3 Risch, F., and Hahn, C : The Technique of Surgical Correction of Anomalies of the Pulmonary Veins in a Series of 25 Cases, Thorax 13: 251, 1958. 4 Lawrance, K., Grimshaw, V. A., Hoyle, G., Hicks, J. P. N., Nixon, P. F. G., and Wooler, G. H.: Surgical Treatment of Atrial Septal Defect and Partial Anomalous Pulmonary Venous Drainage, J. THORAC. CARDIOVASC. SURG. 43: 622,
1962.
5 Friedli, B., Guerin, R., Davignon, A., Fouron, J. C , and Stanley, P.: Surgical Treatment of Partial Anomalous Pulmonary Venous Drainage: A Long-Term Follow-up Study, Circulation 55: 159, 1972. 6 Bailey, C. P., Bolton, H. E., Jamison, W. L., and Neptune, W. B.: Atrio-septo-pexy for Interatrial Septal Defects, J. THORAC. SURG. 26:
184,
1953.
7 Neptune, W. B., Bailey, C. P., and Goldberg, H.: The Surgical Correction of Atrial Septal Defects Associated With Transposition of the Pulmonary Veins, J. THORAC SURG. 25: 623,
1953.
8 Brock, R., and Ross, D. N.: The Sinus Venosus Type of Atrial Septal Defect: Surgical Treatment, Guys Hosp. Rep. 108: 291, 1959. 9 Schuster, S. R., Gross, R. E., and Colodny, A. H.: Surgical Management of Anomalous Right Pulmonary Venous Drainage to the Superior Vena Cava, Associated With Superior Marginal Defect of the Atrial Septum, Surgery 51: 805, 1962. 10 Puig-Massana, M., Murta, M., and Revuelta, J. M.: A New Technique in the Correction of Partial Anomalous Pulmonary Venous Drainage, J. THORAC CARDIOVASC SURG. 64:
108,
1972.
11 Brais, M., Bertranou, E., Brassard, A., Stanley, P., and Chartrand, C : Effect of Dextran on Patency of Pericardial Tubular Graft of the Superior Vena Cava in the Dog, J. THORAC CARDIOVASC SURG. 65: 296,
1973.
12 Hancock, E. W.: Coronary Sinus Rhythm in Sinus Venosus Defect and Persistent Left Superior Vena Cava, Am. J. Cardiol. 14: 608, 1964.
Discussion D R . N I C H O L A S J.
DEMOS
Short Hills, N. J.
I would like to present a very interesting case of ours. A 15-year-old girl complained of easy fatigability. Eight years previously she had had closure of an atrial septal defect.
On physical examination, she was very small for her age. She had the very interesting physical finding of a very loud systolic hum in the right supraclavicular area medially. Cardiac catheterization revealed a 2:1 left-to-right shunt in the supracardiac level, a bilateral upper lobe, and anomalous venous drainage to the superior vena cava. Catheterization through systemic left arm vein revealed the anomalous pulmonary veins on the left side and one on the right side. At the operation, the right upper lobe pulmonary anomalous vein was ligated, being small in size, and the left was transplanted to the left atrium in a very slant fashion. As postoperative arteriograms indicated, the anastomosis is patent 6 months later. DR. ROY H. C L A U S S New York, N. Y.
Dr. Reed, who was unable to be here this afternoon, asked me to comment on the paper he and his former associates published in Surgery, Gynecology and Obstetrics in December, 1962. In essence, the procedure was similar in that it did bivalve the right atrial appendage and bring point A down to point B, as Dr. Chartrand mentioned. Much emphasis was just placed on this latest procedure being superior to those previously described. We would concede that since we used a patch and Dr. Chartrand's group does not, they may have a superior procedure. The remainder of our technique was the same. There is one other aspect in which the current authors may have superior results: We have not performed the late follow-up studies by cardiac catheterization, being satisfied with the clinical results. D R . C H A R T R A N D (Closing) I would like to thank the discussers for their comments. Should a pericardial patch be used on the superior vena cava as proposed by Schuster and other authors? In the previous study that I mentioned in my article, a free pericardial patch was used to widen the superior vena cava in 5 patients. Unfortunately, hemodynamic and angiographic examination demonstrated narrowing or total obstruction in 40 per cent of the cases. Because of these results, experiments were carried out on dogs in order to elucidate the pathophysiology of this complication. Part of the superior vena cava was replaced by a fresh pericardial patch in normal dogs. Within a few days after surgery, acute inflammation of the patch occurred followed by retraction and formation of fibrinous thrombus on the inner surface of that pericardium. At this stage, progressive tubular stenosis was demonstrated angiographically. Within 14 days after the operation, mixed thrombus completed the obstruction; at this stage collateral circulation was well developed. These experimental findings as well as our previous clinical results prompted us to abandon the use of pericardial patch on the superior vena cava and to develop the technique that I have just described.