- Email: [email protected]
SURGICAL TREATMENT OF ATRIAL SEPTAL DEFECT AND PARTIAL ANOMALOUS PULMONARY VENOUS DRAINAGE
SURGICAL TREATMENT OF ATRIAL SEPTAL DEFECT AND PARTIAL ANOMALOUS PULMONARY VENOUS DRAINAGE K. Lawrance, M.B., B.S., F.R.C.S. (Eng.), * V. A. Grimshaw, M.B., B.Chir., F.F.A.R.C.S., Gillian Hoyle, M.B., Ch.B.,** J. P. N. Hicks, M.B., . B.S., F.F.A.R.C.S., P. F. G. Nixon, M.B., B.S., M.R.C.P. (Lond.), and G. H. Wooler, M.D., F.R.C.S. (Eng.), Leeds, England of partial anomalous pulmonary venous drainage and atrial T septalassociation defects has been recognized for over 80 years.' Hughes and HE
Rumore" found anomalous venous drainage of the lungs in 0.7 per cent of routine autopsies, and the two abnormalities are reported as occurring together in from 7 per cent" to 19 per cent' of atrial septal defects. Anomalous veins occur most frequently from the right lung, joining the superior vena cava or right atrium, and the atrial septal defect with which they are associated is usually of the high or sinus venosus type. 5 The anomalous veins may connect at other sites, uncommonly joining the inferior vena cava, the azygos vein, or the left subclavian vein. Anomalous veins from the left lung usually connect with the left innominate vein or the coronary sinus. Rarely there may be bilateral partial anomalous pulmonary venous drainage." The developmental basis of this lesion has been fully described by Edwards. 7 It depends either on the retention of an embryonic connection which is normally lost, .or, in those cases in which the anomalous pulmonary venous connection is with the upper aspect of the right atrium, on an abnormal positioning of the atrial septum. The combination of atrial septal defect and partial anomalous pulmonary venous drainage creates surgical and diagnostic problems. Baileys- 9 treated 7 patients by atrioseptopexy; 2 of the 5 survivals were investigated postoperatively and in one of these the lesion was completely corrected. Kirklin and his colleagues" used the "well" technique to close the septal defect in 8 cases and Bailey's atrioseptopexy in one; no patient died, but a shunt was found after operation in 3. Rotthoff and his eo-workers' describe a special suturing technique with the atrium open under hypothermia; they have employed this in 44 patients with fatal outcome in 5 (11 per cent); from the postoperative studies, a shunt remains in 5 patients and one has developed cyanosis. Marion From The University of Leeds and the Department of Thoracic Surgery, The General Infirmary at Leeds. England. Received for publication May 1. 1961. .Present address: Marion County General Hospital, Indianapolis. Ind. "Present address: Post-Graduate. Hospital, Hammersmith, London, England.
622
Vol. 43, No.5 May, 1962
ANOMALOUS PULMONARY VENOUS DRAINAGE
623
and associates" used a similar technique on 3 patients with satisfactory results in 2. Brom'? describes a method used on 22 patients-with the use of hypothermia, any anomalous vein entering high in the superior vena cava is transplanted, lower anomalous vessels are separated from the superior vena cava, and the atrial septal defect sutured in front of the veins. Holmes Sellers" has closed, by direct suture under hypothermia, 18 atrial septal defects with associated partial anomalous pulmonary venous drainage; in those cases in which the right upper lobe vein connects with the superior vena cava, the vein has electively not been disturbed; thus, out of 16 survivors, 7 have a residual shunt. Broek'" prefers suturing the lower margin of the defect transversely to the wall of the superior vena cava above the anomalous veins, after full mobilization of the veins. Fifteen patients were treated surgically, one with bypass and the rest under hypothermia, and the defect was completely corrected in 10. Morrow and his co-workers'< describe two methods of suturing the defect under hypothermia, but found the results unsatisfactory. The important diagnostic problem is to establish, whenever possible, the presence or absence of anomalous veins in a case of atrial septal defect, before operation. This proved to be possible in 7 out of 9 patients in this series. If the catheter is introduced through the long saphenous vein, the atrial septal defect will invariably be demonstrated and the pulmonary veins entered. It then has to be shown that the veins connect with the right atrium (or one of the venae cavae) and not the left atrium. Introducing a cardiac catheter into the veins is usually not sufficient evidence alone, and indicator dilution curves and serial oxygen samples are usually required. The purpose of this paper is to describe a method of treatment which has heen found satisfactory, and to discuss the diagnostic features. CLI:\'ICAL FEATURES
Nine out of 27 patients with atrial septal defects examined, at operation had partial anomalous pulmonary venous drainage. Three patients had little disability and the lesion was discovered at routine examination. Five patients complained of mild breathlessness, lassitude, and palpitations. It is interesting to note that the remaining 2 patients had epileptiform attacks and the cardiac lesion was discovered during investigation of these attacks; it appears probable that the attacks, which had been treated as grand mal for some time, were due to the large left-to-right shunt at atrial leveL The association of atrial septal defect with seizures has been described by Papp." Physical signs in the 9 patients were essentially similar to the 18 who had an atrial septal defect alone; thus there was clinical evidence of right ventricular enlargement, a split second sound, and a pulmonary systolic murmur. The electrocardiograph was similar to that of uncomplicated atrial septal defect, showing a right bundle branch block pattern. The posteroanterior roentgenogram of the chest showed a rather prominent vein entering the superior vena cava in 5 cases, and the lung fields had the vascularity attributable to abnormally great pulmonary flow.
624
LAWRANCE ET AL.
J. Thoraci c and
Cardiovas. Surg.
Thus , apart from the prominent vein, the clinical features do not of themselves suggest the presence of more than an atrial septal defect. PREOPERATIVE CARDIAC CATHETERIZATION
The cardiac catheter may pass into a pulmonary vein draining into the right atrium, left atrium, the superior vena cava, or inferior vena cava. It is not possible to be sure from screening alone that the catheter has reached the vein from right or left atrium (F ig. 1). Indicator dilution curves provide a reliable method of distinguishing between anomalous and normally connected
Fig. l.--eardlac catheter lying In an anomalous pulmonary vein.
pulmonary veins. Dye is injected into the pulmonary vein; when that vein drains into the right side of the heart, the dye curve is similar to that obtained by superior vena caval injection, or injection high in the right atrium (Fig. 2) . When the vein drains into the left atrium a different pattern is obtained. Dye injected into a normally connected pulmonary vein does not pass through the pulmonary circulation and appears at the ear more quickly than dye injected into the superior vena cava (Fig. 3) . Measurement of oxygen saturation in blood samples from different chambers is a well-established method of detecting left-to-right shunts and may give a clue as to the presence of anomalous pulmonary veins draining into.the superior vena cava, inferior vena cava, or left innominate vein . A case of atrial septal defect will show a rise in oxygen saturation in right
Vol. 43, No.5 May, 1962
ANOMALOUS PULMONARY VENOUS DRAINAGE
625
atrial blood, compared with blood withdrawn from the venae cavae. If blood is then withdrawn from the superior vena cava at several sites, 1.0 em. apart, the rise in oxygen saturation may be found to begin at some distance proximal to the right atrium. This indicates that an anomalous pulmonary vein may be draining at this site and manipulation of the catheter may result in catheterization of the vein. In the rarer condition of a pulmonary vein draining into the inferior vena cava, variations in oxygen saturation may be detected.
SUPERIOR VENA CAVAL 11IIJECTION
R. ANOMALOUS PlLMONARY VEIN INJECTION
L. ATRIAL INJECTION
Fig. 2.-Dye dilution curves from a patient with atrial. septal defect with partial anomalous pulmonary venous drainage.
Fig. 3.-Dye dilution curve, with injection of dye into a normally connected pulmonary vein, which shows early appearance time.
In 2 out of the 9 cases of atrial septal defect with anomalous pulmonary veins treated surgically in this department, the anomalous veins were not suspected until they were found at operation. The method of taking serial blood samples from the venae cavae formeasurement of oxygen saturation was not used and might have enabled the veins to be detected in these cases. As well as indicating the anomalous pulmonary venous drainage, it was possible to demonstrate with dye curves that the left-to-right shunt through the atrial septal defect was markedly smaller than that through the anomalous pulmonary veins. The dye curve in Fig. 2 in which the injection was into the superior vena cava represents the total left-to-right shunt, whereas dye injected
LAWRANCE ET AL.
626
J. Thoracic and Cardiovas. Surg.
into the left atrium shows a minimal shunt. This indicates that the majority of the shunt is through the pulmonary veins. In contrast, in the presence of normally connected pulmonary veins, injection of dye into the left atrium shows that the entire left-to-right shunt is through the atrial septal defect (Fig. 4). Morrow and associates" report similar findings with regard to the site of the shunt in the combined lesion. Closure of the atrial septal defect only, therefore, is of little or no benefit to the patient.
Fig. 4.-Dye dilution curve, with injection of dye into left atrium in the presence of an atrial septal defect but normal pulmonary veins, which shows a large left-to-right shunt. ANESTHETIC TECHNIQVE
Every patient, with the exception of 2 weighing 63 and 70 kilograms, respectively, received identical doses of drugs for premedication. This consisted of Nembutal (pentobarbitone), 100 mg. by mouth 2 hours before operation, followed one hour before operation by papaveretum 20 mg. and scopolamine 0.4 mg. intramuscularly. In the case of the 2 patients mentioned above, the dose of pentobarbitone was increased to 200 mg. Induction of anesthesia was carried out using a minimal dose of 5 per cent thiopentone (200 to 300 mg.), followed by d-tubocurarine (0.5 mg. per kilogram). When adequate relaxation was present, the trachea was intubated with a cuffed endotracheal tube. After the establishment of endotracheal anesthesia, a thermistor lead for recording the patient's temperature was passed down the esophagus to the "post cardiac" region. Anesthesia was maintained with nitrous oxide and oxygen, using intermittent positive pressure respiration. The respirator was adjusted to give only a slight degree of hyperventilation and was re-adjusted as indicated by the pH and PC0 2 readings. Pethidine and tubocurarine were given as required in the pre "bypass" stage of the operation. With the patient in position for a right thoracotomy, an intravenous transfusion of blood was set up in the left arm. Electrocardiographic and electroencephalographic electrodes with their respective leads were attached to the patient in the anesthetic room. In the theater, a nylon catheter was inserted into the left radial artery to record blood pressure. Another nylon catheter was passed through a left arm vein for the measurement of superior vena caval pressure and as an alternative route for intravenous therapy.
Vol. 43, No.5 May, 1962
ANOMALOUS PULMONARY VENOUS DRAINAGE
627
Heparin (2.5 mg. per kilogram) was given intravenously to the patient and allowed to circulate immediately prior to the insertion of the cannula into the femoral artery. An additional dose of tubocurarine and pethidine was added to the blood in the oxygenator before cardiopulmonary bypass was started. This prevented dilution of the circulating anesthetic agents in the patients and gave a smoother take over during the establishment of "bypass." During bypass, the lungs were held partially inflated with a mixture of oxygen (20 per cent) and helium (80 per cent) at a pressure of about 5 mm. Hg. When "partial bypass" was recommenced by releasing the superior vena cava and inferior vena cava snares, the lungs were re-inflated with 100 per cent oxygen. This was continued until nitrous oxide was required to maintain anesthesia; no other anesthetic agents were required in the post bypass stage of the operation. Protamine sulfate (3 to 3.5 mg. per kilogram) was given following removal of the arterial and venous cannulae to restore blood clotting mechanisms. Spontaneous respiration was resumed at the end of the operation in all the patients; however, atropine and Prostigmin were given to all patients to reverse any residual curarization that might be present. In 2 of the 7 patients, bronchial secretions were increased and postoperative bronchoscopy was necessary. All the patients spoke soon after the endotracheal tuhe was removed; after removal to the postoperative recovery ward, all the patients were nursed in an oxygen tent for the first 24 hours. PERFUSION
The Extmcorporeal Circulation.-Blood flowed by gravity from caval vein catheters into a modified Melrose oxygenator." A bar pump" returned it to the femoral artery through a New Electronics Products heat exchanger and filter. Technique.-During total heart and lung bypass the patient was perfused with a blood flow of 2.4 L. per square meter per minute. In 6 cases, lowering of the esophageal temperature from 35° to 36° C. to 30° to 32° C. with the heat exchanger allowed the flow to be reduced by one third. Before the end of perfusion a temperature of 34° to 35° C. was restored. Immediately after perfusion, hypovolemia was sought and corrected by repeating injections of 80 m1. of blood into the femoral artery until a transient reflex fall in arterial pressure resulted from the maneuver." The shortest perfusion lasted 33 minutes, and the longest 55 minutes. OPERATIVE TECHNIQUES
Each operation was performed with the patient lying on the left side, the right pleural cavity being opened through the bed of the sixth rib. The internal mammary vessels were divided and sometimes the fifth costal cartilage mobilized at its junction with the sternum. This gave an excellent view of the right
628
LAWRANCE ET AL.
J. Thoracic and
Cardiovas. Surg.
thorax. The right lung invariably looked and felt congested with blood, giving a similar appearance to that seen in mitral valve disease. Retracting the lung posteriorly revealed the enlarged right atrium bulging under the pericardium. The vena azygos entered the superior vena cava high up, and in one patient it joined at the same level as the two innominate veins. Just below the vena azygos, the anterior pulmonary vein usually joined the superior vena cava and, from this point, the diameter of the superior vena cava was doubled. The pericardium was opened by a large flap hinged along the phrenic nerve and vessels, great care being taken to avoid damage to the nerve. The pericardial reflection was dissected off the superior vena cava, and the vena cava
L
SY.c
t
ANOMALOUS
VEfN(S
j __,
'"
'.
, I
~
..
.
~.
...,, 'b
~AS.D.
,,)
';.__.. c
~
LV.C.
Fig. 5.-Diagram to show method of closure of atrial septal defect with anomalous pulmonary veins.
mobilized from the mediastinal tissues for the whole of its length. A tourniquet was passed around the vena cava at its commencement just below the two innominate veins. A tourniquet was applied to the vena azygos, or it was ligated. With the pericardium widely open, a better view was obtained of the arrangement of the anomalous pulmonary veins. Usually the anterior pulmonary vein entered the superior vena cava, with a middle lobe vein just below it; alternatively the middle lobe vein entered the right atrium at a high level. In 5 of the 9 patients the inferior pulmonary vein connected high in the right atrium-usually about the center of the atrium. The right common femoral artery was cannulated; the venous catheters were introduced into the venae cavae through separate incisions in the anterior wall of the right atrium. This gave ample room to open the right atrium through a long posterior incision. The venous catheters were 24 inches long and having crossed each other as they emerged from the atrium, each was laid along one side of the thoracotomy incision so as not to encroach on the operating field. The tip of the superior catheter should be just in the right innominate vein, and the tourniquet around this catheter tightened as high up the vena cava as possible.
Vol. 43.• No.5 May, 1962
ANOMALOUS PULMONARY VENOUS DRAINAGE
629
On full perfusion and with the heart empty, a long incision was made in the right atrium near the inter-atrial groove. This incision was extended up the superior vena cava to display the openings of all the anomalous veins. The cavity of the right atrium was carefully inspected, and the right ventricle and pulmonary outflow tract explored digitally through the tricuspid valve. In the first case the defect was low in the atrial septum, but in the remainder it was of the high or sinus venosus type lying at the junction with the superior vena cava. One patient had a second atrial septal defect which was closed by suture.
Fig. G.-Diagram of operative procedure.
A patch of Teflon felt, fashioned to cover the anomalous veins and the defect, was secured in position with interrupted mattress sutures of 3-0 braided silk. Commencing in the superior vena cava, just above the edge of the high est anomalous vein (Fig. 5), the suturing was carried downward just in front of the anterior edge of the anomalous veins to point b (F ig. 5) and thence down the anterior edge of the septal defect. The patch was tailored to fit properly as each suture was inserted. Next the lower _d m of the defect was sutured
LAWRANCE ET AL.
630 P.A.
4 \, ' -
-
,-
-
-
-
_
, /, t-
]. Thoracic and
Cardiovas. Surg.
' I~
L.STEF\NAL , ' BORDER - . -- - - - - - - .',.., -
PRE· OPEF\ATI VE
~.J
' •
~"''''''''
I
"",...._.;I\
... I
POST · OPERATIVE Fig. 7.-Pre- and postoperative phonocardiograms of a patient with atrial septal defect and partial anomalous pulmonary venous drainage.
p E OP F.A " TII/
~ ...· R.AY
Fig. 8.-A and B, Roentgenograms which show diminution in heart size following repair of ,the combined defect.
Vol. 43, No.5 May, 1962
ANOMALOUS PULMONARY VENOUS DRAINAGE
631
(point C) and the patch brought forward and sutured to the wall of the right atrium in front of the anomalous veins (point d). Finally the repair was completed by suturing from point d to point a. In this way the anomalous veins drained under the patch, through the defect into the left atrium. With the repair complete, any air trapped in the left atrium or the right pulmonary veins was aspirated with a syringe and needle. The intracardiac operation being finished, the right atrium was repaired and the main pulmonary artery aspirated for air. Since, following the repair of the defect, the catheter in the superior vena cava may fit tightly, this catheter was withdrawn into the atrium (when the tourniquet was released) to avoid obstructing the flow. The pericardium was partially closed around a drainage tube, and three drainage tubes were inserted into the right pleural cavity, one anterior, one posterior, and a third along the inside of the thoracotomy opening. The third tube was to prevent blood collecting in the lateral part of the chest. small shunt followi"9 closure of A.5.D & redrection of anomalous • pulmonary venous drall\Cl¥
Fig. 9.-Dye dilution curve in a postoperative patient showing a small residual shunt; pulmonary artery injection.
POSTOPERATIVE FOLLOW-UP
Eight of the 9 patients were admitted for postoperative cardiac catheterization at intervals of 10 to 18 months after operation. The ninth patient had become pregnant. The 5 patients who had complained of dyspnea and fatigue before operation were symptom free when admitted for postoperative cardiac catheterization. All patients retained the widely split pulmonary second sound characteristic of atrial septal defect (Fig. 7). A soft systolic murmur down the left sternal border was audible in only one patient; her subsequent investigations showed no residual shunt. The electrocardiogram still showed a right bundle branch block pattern in all patients. Preoperative chest x-ray studies on 2 of the 9 patients showed considerable cardiac enlargement. There was marked reduction in heart size and pulmonary vascularity at postoperative investigation of these patients (see Fig. 8, A and B).
632
J. Thoracic and Cardiovas. Surg.
LAWRANCE ET AL.
At cardiac eatheterization iin each case, blood oxygen saturations were measured and a dye curve performed, injecting dye into the pulmonary artery. Two patients showed a rise of 5.5 per cent in the oxygen saturation of blood from the right atrium compared with the vena caval blood. Dye curves in these 2 patients showed a very smallieft-to-right shunt (Fig. 9). In the other 6 cases, dye curves and oxygen saturations were completely normal (Fig. 10). A.S.D. WITH ANOMALOUS PULMONARY VENOUS DRAINAGE DYE INJECTION INTO R. PULMONARY ARTERY
POST·OPERATIVE
PRE-OPERATIVE
Fig. lO.-Dye dilution curves which show preoperative shunt and a normal postoperative curve; pulmonary artery injection. COMMENT
Our experience with the combined lesion of atrial septal defect and partial anomalous pulmonary venous drainage has led us to conclusions similar to those of Morrow and his assoeiates.v Since the left-to-right shunt is almost completely through the abnormally connected pulmonary veins, any method of closure is unsatisfactory unless it can be relied upon to re-direct the whole shunt to the normal route. Unhurried and full correction of the defect cannot be achieved in the relatively short time period allowed by hypothermia, and therefore, we prefer to employ total bypass. Using the technique described, 9 patients were operated upon without a death, and, in each case, the defect was completely corrected. When the patients were re-assessed, 10 to 18 months later, all were symptom free, but, in 2 patients, a small residual left-to-right shunt could be detected. In spite of careful preoperative studies, it is not always possible to recognize the presence of abnormally connected pulmonary veins in association with an atrial septal defect. This is an additional reason for preferring to repair atrial septal defects in general, and the combined lesion in particular, with the aid of total body perfusion. SUMMARY ~
A method for completely correcting the combined lesion of atrial septal defect and partial anomalous venous drainage is described; closure of the atrial septal defect only is not sufficient. The technique, which requires the use of total body perfusion, has been used satisfactorily on 9 patients. Methods of diagnosis are also described.
Vol. 43, No. S May, 1962
ANOMALOUS PULMONARY VENOUS DRAINAGE
633
REFERENCES 1. Von Rokitansky, C. F.: Die Defecte der Schcidewande des Herzons, Vienna, 1875, Wilhelm Braumiiller, 156 pp. 2. Hughes, C. H., and Rumore, P. C.: Anomalous Pulmonary Veins, Arch. Path. 37: 364·366, 1944. 3. Holmes Sellors, T.: Sinus Venosus Defects. Paper read at Annual Meeting of the Society of Thoracic Surgeons of Great Britain and Ireland, October, 1960. 4. Rotthoff, G., Lojacono, L., Puehetti, V., and Fahmy, A. R.: Defect of the IntraAtrial Septum With Associated Transposition of the Pulmonary Veins; Surgical Treatment, Ohir, Ital. II: 249-269, 1959. 5. Shaner, R. F.: The "High" Defect in the Atrial Septum, Canad. M. A. J. 78: 688-690, 1958. 6. Ellis, F. H., Jr., Callahan, J. A., DuShane, J. W., Edwards, J. Eo, and Wvod, Eo a.. Partial Anomalous Pulmonary Venous Connections Involving Both Lungs With Inter-Atrial Communications, Proc. Mayo Clin. 33: 65,74, 1958. 7. Edwards, J. E.: Pathologic and Developmental Considerations in Anomalous Pulmonary Venous Connection, Proe. Mayo Clin. 28: 441-452, 1953. 80 Bailey, C. P., Bolton, H. E., Jamison, W. L., and Neptune, W. R: Atrio-septo-pexy for Inter-Atrial Septal Defects, J. THORACIC SURG. 26: 184-219, 1953. 9. Neptune, W. R, Bailey, C. P., and Goldberg, H: The Surgical Correction of Atrial Septal Defects, Associated With Transposition of the Pulmonary Veins, J. THORACIC SURG. 25: 623-634, 1953. 10. Kirklin, J. W., Ellis, F. H., Jr., and Wood, E. H.: Treatment of Anomalous Pulmonary Venous Connection in Association With Inter-Atrial Communications, Surgery 39: 389-398, 1956. 11. Marion, P., Deyrieux, F., Gounot, P., and Estanoue, S.: The Problem of Surgical Treatment of Inter-Auricular Communication (Ostium Secundum) With Partial Abnormal Venous Return, Arch. mal. coeur, 53: 167-185, 1960. 12. Brom, A. G.: Paper read at the annual meeting of the Society of Thoracic Surgeons of Great Britain and Ireland, October, 1960. 13. Brock, R. C., and Ross D. N.: The Sinus Venosus TyPe of Atrial Septal Defect, Guy's Hosp. Rep. 108: 291-304, 1959. 14. Morrow, A. G., Gilbert, J. W., Baker, R. R., and Collins, N. Po: The Closure of Atrial Septal Defects Using General Hypothermia, J. THORACIC SURG. 40: 776-786, 1960. 15. Papp, C.: Cardiac Syncope in Atrial Septal Defect, Brit. Heart J. 20: 9-14, 1958. 16. Nixon, P. G. F., Grimshaw, V. A., Catchpole, L. A., Snow, H. M., and Lawrance, K: Clinical Experience With the Melrose Oxygenator at Normal and Reduced Temperatures, Thorax 15: 193-197, 1960. 17. Nixon, P. G. F.: A Pump for Use in Open Heart Surgery, Lancet 1: 1074, 1959. 18. Nixon, P. G. F., Grimshaw, V. A., and Wooler, G. H.: Clinical Observations on Vasomotor Reflexes in Relation to Blood Volume in Open Heart Surgery, Lancet 2: 1429-1431, 1960.
Rumore" found anomalous venous drainage of the lungs in 0.7 per cent of routine autopsies, and the two abnormalities are reported as occurring together in from 7 per cent" to 19 per cent' of atrial septal defects. Anomalous veins occur most frequently from the right lung, joining the superior vena cava or right atrium, and the atrial septal defect with which they are associated is usually of the high or sinus venosus type. 5 The anomalous veins may connect at other sites, uncommonly joining the inferior vena cava, the azygos vein, or the left subclavian vein. Anomalous veins from the left lung usually connect with the left innominate vein or the coronary sinus. Rarely there may be bilateral partial anomalous pulmonary venous drainage." The developmental basis of this lesion has been fully described by Edwards. 7 It depends either on the retention of an embryonic connection which is normally lost, .or, in those cases in which the anomalous pulmonary venous connection is with the upper aspect of the right atrium, on an abnormal positioning of the atrial septum. The combination of atrial septal defect and partial anomalous pulmonary venous drainage creates surgical and diagnostic problems. Baileys- 9 treated 7 patients by atrioseptopexy; 2 of the 5 survivals were investigated postoperatively and in one of these the lesion was completely corrected. Kirklin and his colleagues" used the "well" technique to close the septal defect in 8 cases and Bailey's atrioseptopexy in one; no patient died, but a shunt was found after operation in 3. Rotthoff and his eo-workers' describe a special suturing technique with the atrium open under hypothermia; they have employed this in 44 patients with fatal outcome in 5 (11 per cent); from the postoperative studies, a shunt remains in 5 patients and one has developed cyanosis. Marion From The University of Leeds and the Department of Thoracic Surgery, The General Infirmary at Leeds. England. Received for publication May 1. 1961. .Present address: Marion County General Hospital, Indianapolis. Ind. "Present address: Post-Graduate. Hospital, Hammersmith, London, England.
622
Vol. 43, No.5 May, 1962
ANOMALOUS PULMONARY VENOUS DRAINAGE
623
and associates" used a similar technique on 3 patients with satisfactory results in 2. Brom'? describes a method used on 22 patients-with the use of hypothermia, any anomalous vein entering high in the superior vena cava is transplanted, lower anomalous vessels are separated from the superior vena cava, and the atrial septal defect sutured in front of the veins. Holmes Sellers" has closed, by direct suture under hypothermia, 18 atrial septal defects with associated partial anomalous pulmonary venous drainage; in those cases in which the right upper lobe vein connects with the superior vena cava, the vein has electively not been disturbed; thus, out of 16 survivors, 7 have a residual shunt. Broek'" prefers suturing the lower margin of the defect transversely to the wall of the superior vena cava above the anomalous veins, after full mobilization of the veins. Fifteen patients were treated surgically, one with bypass and the rest under hypothermia, and the defect was completely corrected in 10. Morrow and his co-workers'< describe two methods of suturing the defect under hypothermia, but found the results unsatisfactory. The important diagnostic problem is to establish, whenever possible, the presence or absence of anomalous veins in a case of atrial septal defect, before operation. This proved to be possible in 7 out of 9 patients in this series. If the catheter is introduced through the long saphenous vein, the atrial septal defect will invariably be demonstrated and the pulmonary veins entered. It then has to be shown that the veins connect with the right atrium (or one of the venae cavae) and not the left atrium. Introducing a cardiac catheter into the veins is usually not sufficient evidence alone, and indicator dilution curves and serial oxygen samples are usually required. The purpose of this paper is to describe a method of treatment which has heen found satisfactory, and to discuss the diagnostic features. CLI:\'ICAL FEATURES
Nine out of 27 patients with atrial septal defects examined, at operation had partial anomalous pulmonary venous drainage. Three patients had little disability and the lesion was discovered at routine examination. Five patients complained of mild breathlessness, lassitude, and palpitations. It is interesting to note that the remaining 2 patients had epileptiform attacks and the cardiac lesion was discovered during investigation of these attacks; it appears probable that the attacks, which had been treated as grand mal for some time, were due to the large left-to-right shunt at atrial leveL The association of atrial septal defect with seizures has been described by Papp." Physical signs in the 9 patients were essentially similar to the 18 who had an atrial septal defect alone; thus there was clinical evidence of right ventricular enlargement, a split second sound, and a pulmonary systolic murmur. The electrocardiograph was similar to that of uncomplicated atrial septal defect, showing a right bundle branch block pattern. The posteroanterior roentgenogram of the chest showed a rather prominent vein entering the superior vena cava in 5 cases, and the lung fields had the vascularity attributable to abnormally great pulmonary flow.
624
LAWRANCE ET AL.
J. Thoraci c and
Cardiovas. Surg.
Thus , apart from the prominent vein, the clinical features do not of themselves suggest the presence of more than an atrial septal defect. PREOPERATIVE CARDIAC CATHETERIZATION
The cardiac catheter may pass into a pulmonary vein draining into the right atrium, left atrium, the superior vena cava, or inferior vena cava. It is not possible to be sure from screening alone that the catheter has reached the vein from right or left atrium (F ig. 1). Indicator dilution curves provide a reliable method of distinguishing between anomalous and normally connected
Fig. l.--eardlac catheter lying In an anomalous pulmonary vein.
pulmonary veins. Dye is injected into the pulmonary vein; when that vein drains into the right side of the heart, the dye curve is similar to that obtained by superior vena caval injection, or injection high in the right atrium (Fig. 2) . When the vein drains into the left atrium a different pattern is obtained. Dye injected into a normally connected pulmonary vein does not pass through the pulmonary circulation and appears at the ear more quickly than dye injected into the superior vena cava (Fig. 3) . Measurement of oxygen saturation in blood samples from different chambers is a well-established method of detecting left-to-right shunts and may give a clue as to the presence of anomalous pulmonary veins draining into.the superior vena cava, inferior vena cava, or left innominate vein . A case of atrial septal defect will show a rise in oxygen saturation in right
Vol. 43, No.5 May, 1962
ANOMALOUS PULMONARY VENOUS DRAINAGE
625
atrial blood, compared with blood withdrawn from the venae cavae. If blood is then withdrawn from the superior vena cava at several sites, 1.0 em. apart, the rise in oxygen saturation may be found to begin at some distance proximal to the right atrium. This indicates that an anomalous pulmonary vein may be draining at this site and manipulation of the catheter may result in catheterization of the vein. In the rarer condition of a pulmonary vein draining into the inferior vena cava, variations in oxygen saturation may be detected.
SUPERIOR VENA CAVAL 11IIJECTION
R. ANOMALOUS PlLMONARY VEIN INJECTION
L. ATRIAL INJECTION
Fig. 2.-Dye dilution curves from a patient with atrial. septal defect with partial anomalous pulmonary venous drainage.
Fig. 3.-Dye dilution curve, with injection of dye into a normally connected pulmonary vein, which shows early appearance time.
In 2 out of the 9 cases of atrial septal defect with anomalous pulmonary veins treated surgically in this department, the anomalous veins were not suspected until they were found at operation. The method of taking serial blood samples from the venae cavae formeasurement of oxygen saturation was not used and might have enabled the veins to be detected in these cases. As well as indicating the anomalous pulmonary venous drainage, it was possible to demonstrate with dye curves that the left-to-right shunt through the atrial septal defect was markedly smaller than that through the anomalous pulmonary veins. The dye curve in Fig. 2 in which the injection was into the superior vena cava represents the total left-to-right shunt, whereas dye injected
LAWRANCE ET AL.
626
J. Thoracic and Cardiovas. Surg.
into the left atrium shows a minimal shunt. This indicates that the majority of the shunt is through the pulmonary veins. In contrast, in the presence of normally connected pulmonary veins, injection of dye into the left atrium shows that the entire left-to-right shunt is through the atrial septal defect (Fig. 4). Morrow and associates" report similar findings with regard to the site of the shunt in the combined lesion. Closure of the atrial septal defect only, therefore, is of little or no benefit to the patient.
Fig. 4.-Dye dilution curve, with injection of dye into left atrium in the presence of an atrial septal defect but normal pulmonary veins, which shows a large left-to-right shunt. ANESTHETIC TECHNIQVE
Every patient, with the exception of 2 weighing 63 and 70 kilograms, respectively, received identical doses of drugs for premedication. This consisted of Nembutal (pentobarbitone), 100 mg. by mouth 2 hours before operation, followed one hour before operation by papaveretum 20 mg. and scopolamine 0.4 mg. intramuscularly. In the case of the 2 patients mentioned above, the dose of pentobarbitone was increased to 200 mg. Induction of anesthesia was carried out using a minimal dose of 5 per cent thiopentone (200 to 300 mg.), followed by d-tubocurarine (0.5 mg. per kilogram). When adequate relaxation was present, the trachea was intubated with a cuffed endotracheal tube. After the establishment of endotracheal anesthesia, a thermistor lead for recording the patient's temperature was passed down the esophagus to the "post cardiac" region. Anesthesia was maintained with nitrous oxide and oxygen, using intermittent positive pressure respiration. The respirator was adjusted to give only a slight degree of hyperventilation and was re-adjusted as indicated by the pH and PC0 2 readings. Pethidine and tubocurarine were given as required in the pre "bypass" stage of the operation. With the patient in position for a right thoracotomy, an intravenous transfusion of blood was set up in the left arm. Electrocardiographic and electroencephalographic electrodes with their respective leads were attached to the patient in the anesthetic room. In the theater, a nylon catheter was inserted into the left radial artery to record blood pressure. Another nylon catheter was passed through a left arm vein for the measurement of superior vena caval pressure and as an alternative route for intravenous therapy.
Vol. 43, No.5 May, 1962
ANOMALOUS PULMONARY VENOUS DRAINAGE
627
Heparin (2.5 mg. per kilogram) was given intravenously to the patient and allowed to circulate immediately prior to the insertion of the cannula into the femoral artery. An additional dose of tubocurarine and pethidine was added to the blood in the oxygenator before cardiopulmonary bypass was started. This prevented dilution of the circulating anesthetic agents in the patients and gave a smoother take over during the establishment of "bypass." During bypass, the lungs were held partially inflated with a mixture of oxygen (20 per cent) and helium (80 per cent) at a pressure of about 5 mm. Hg. When "partial bypass" was recommenced by releasing the superior vena cava and inferior vena cava snares, the lungs were re-inflated with 100 per cent oxygen. This was continued until nitrous oxide was required to maintain anesthesia; no other anesthetic agents were required in the post bypass stage of the operation. Protamine sulfate (3 to 3.5 mg. per kilogram) was given following removal of the arterial and venous cannulae to restore blood clotting mechanisms. Spontaneous respiration was resumed at the end of the operation in all the patients; however, atropine and Prostigmin were given to all patients to reverse any residual curarization that might be present. In 2 of the 7 patients, bronchial secretions were increased and postoperative bronchoscopy was necessary. All the patients spoke soon after the endotracheal tuhe was removed; after removal to the postoperative recovery ward, all the patients were nursed in an oxygen tent for the first 24 hours. PERFUSION
The Extmcorporeal Circulation.-Blood flowed by gravity from caval vein catheters into a modified Melrose oxygenator." A bar pump" returned it to the femoral artery through a New Electronics Products heat exchanger and filter. Technique.-During total heart and lung bypass the patient was perfused with a blood flow of 2.4 L. per square meter per minute. In 6 cases, lowering of the esophageal temperature from 35° to 36° C. to 30° to 32° C. with the heat exchanger allowed the flow to be reduced by one third. Before the end of perfusion a temperature of 34° to 35° C. was restored. Immediately after perfusion, hypovolemia was sought and corrected by repeating injections of 80 m1. of blood into the femoral artery until a transient reflex fall in arterial pressure resulted from the maneuver." The shortest perfusion lasted 33 minutes, and the longest 55 minutes. OPERATIVE TECHNIQUES
Each operation was performed with the patient lying on the left side, the right pleural cavity being opened through the bed of the sixth rib. The internal mammary vessels were divided and sometimes the fifth costal cartilage mobilized at its junction with the sternum. This gave an excellent view of the right
628
LAWRANCE ET AL.
J. Thoracic and
Cardiovas. Surg.
thorax. The right lung invariably looked and felt congested with blood, giving a similar appearance to that seen in mitral valve disease. Retracting the lung posteriorly revealed the enlarged right atrium bulging under the pericardium. The vena azygos entered the superior vena cava high up, and in one patient it joined at the same level as the two innominate veins. Just below the vena azygos, the anterior pulmonary vein usually joined the superior vena cava and, from this point, the diameter of the superior vena cava was doubled. The pericardium was opened by a large flap hinged along the phrenic nerve and vessels, great care being taken to avoid damage to the nerve. The pericardial reflection was dissected off the superior vena cava, and the vena cava
L
SY.c
t
ANOMALOUS
VEfN(S
j __,
'"
'.
, I
~
..
.
~.
...,, 'b
~AS.D.
,,)
';.__.. c
~
LV.C.
Fig. 5.-Diagram to show method of closure of atrial septal defect with anomalous pulmonary veins.
mobilized from the mediastinal tissues for the whole of its length. A tourniquet was passed around the vena cava at its commencement just below the two innominate veins. A tourniquet was applied to the vena azygos, or it was ligated. With the pericardium widely open, a better view was obtained of the arrangement of the anomalous pulmonary veins. Usually the anterior pulmonary vein entered the superior vena cava, with a middle lobe vein just below it; alternatively the middle lobe vein entered the right atrium at a high level. In 5 of the 9 patients the inferior pulmonary vein connected high in the right atrium-usually about the center of the atrium. The right common femoral artery was cannulated; the venous catheters were introduced into the venae cavae through separate incisions in the anterior wall of the right atrium. This gave ample room to open the right atrium through a long posterior incision. The venous catheters were 24 inches long and having crossed each other as they emerged from the atrium, each was laid along one side of the thoracotomy incision so as not to encroach on the operating field. The tip of the superior catheter should be just in the right innominate vein, and the tourniquet around this catheter tightened as high up the vena cava as possible.
Vol. 43.• No.5 May, 1962
ANOMALOUS PULMONARY VENOUS DRAINAGE
629
On full perfusion and with the heart empty, a long incision was made in the right atrium near the inter-atrial groove. This incision was extended up the superior vena cava to display the openings of all the anomalous veins. The cavity of the right atrium was carefully inspected, and the right ventricle and pulmonary outflow tract explored digitally through the tricuspid valve. In the first case the defect was low in the atrial septum, but in the remainder it was of the high or sinus venosus type lying at the junction with the superior vena cava. One patient had a second atrial septal defect which was closed by suture.
Fig. G.-Diagram of operative procedure.
A patch of Teflon felt, fashioned to cover the anomalous veins and the defect, was secured in position with interrupted mattress sutures of 3-0 braided silk. Commencing in the superior vena cava, just above the edge of the high est anomalous vein (Fig. 5), the suturing was carried downward just in front of the anterior edge of the anomalous veins to point b (F ig. 5) and thence down the anterior edge of the septal defect. The patch was tailored to fit properly as each suture was inserted. Next the lower _d m of the defect was sutured
LAWRANCE ET AL.
630 P.A.
4 \, ' -
-
,-
-
-
-
_
, /, t-
]. Thoracic and
Cardiovas. Surg.
' I~
L.STEF\NAL , ' BORDER - . -- - - - - - - .',.., -
PRE· OPEF\ATI VE
~.J
' •
~"''''''''
I
"",...._.;I\
... I
POST · OPERATIVE Fig. 7.-Pre- and postoperative phonocardiograms of a patient with atrial septal defect and partial anomalous pulmonary venous drainage.
p E OP F.A " TII/
~ ...· R.AY
Fig. 8.-A and B, Roentgenograms which show diminution in heart size following repair of ,the combined defect.
Vol. 43, No.5 May, 1962
ANOMALOUS PULMONARY VENOUS DRAINAGE
631
(point C) and the patch brought forward and sutured to the wall of the right atrium in front of the anomalous veins (point d). Finally the repair was completed by suturing from point d to point a. In this way the anomalous veins drained under the patch, through the defect into the left atrium. With the repair complete, any air trapped in the left atrium or the right pulmonary veins was aspirated with a syringe and needle. The intracardiac operation being finished, the right atrium was repaired and the main pulmonary artery aspirated for air. Since, following the repair of the defect, the catheter in the superior vena cava may fit tightly, this catheter was withdrawn into the atrium (when the tourniquet was released) to avoid obstructing the flow. The pericardium was partially closed around a drainage tube, and three drainage tubes were inserted into the right pleural cavity, one anterior, one posterior, and a third along the inside of the thoracotomy opening. The third tube was to prevent blood collecting in the lateral part of the chest. small shunt followi"9 closure of A.5.D & redrection of anomalous • pulmonary venous drall\Cl¥
Fig. 9.-Dye dilution curve in a postoperative patient showing a small residual shunt; pulmonary artery injection.
POSTOPERATIVE FOLLOW-UP
Eight of the 9 patients were admitted for postoperative cardiac catheterization at intervals of 10 to 18 months after operation. The ninth patient had become pregnant. The 5 patients who had complained of dyspnea and fatigue before operation were symptom free when admitted for postoperative cardiac catheterization. All patients retained the widely split pulmonary second sound characteristic of atrial septal defect (Fig. 7). A soft systolic murmur down the left sternal border was audible in only one patient; her subsequent investigations showed no residual shunt. The electrocardiogram still showed a right bundle branch block pattern in all patients. Preoperative chest x-ray studies on 2 of the 9 patients showed considerable cardiac enlargement. There was marked reduction in heart size and pulmonary vascularity at postoperative investigation of these patients (see Fig. 8, A and B).
632
J. Thoracic and Cardiovas. Surg.
LAWRANCE ET AL.
At cardiac eatheterization iin each case, blood oxygen saturations were measured and a dye curve performed, injecting dye into the pulmonary artery. Two patients showed a rise of 5.5 per cent in the oxygen saturation of blood from the right atrium compared with the vena caval blood. Dye curves in these 2 patients showed a very smallieft-to-right shunt (Fig. 9). In the other 6 cases, dye curves and oxygen saturations were completely normal (Fig. 10). A.S.D. WITH ANOMALOUS PULMONARY VENOUS DRAINAGE DYE INJECTION INTO R. PULMONARY ARTERY
POST·OPERATIVE
PRE-OPERATIVE
Fig. lO.-Dye dilution curves which show preoperative shunt and a normal postoperative curve; pulmonary artery injection. COMMENT
Our experience with the combined lesion of atrial septal defect and partial anomalous pulmonary venous drainage has led us to conclusions similar to those of Morrow and his assoeiates.v Since the left-to-right shunt is almost completely through the abnormally connected pulmonary veins, any method of closure is unsatisfactory unless it can be relied upon to re-direct the whole shunt to the normal route. Unhurried and full correction of the defect cannot be achieved in the relatively short time period allowed by hypothermia, and therefore, we prefer to employ total bypass. Using the technique described, 9 patients were operated upon without a death, and, in each case, the defect was completely corrected. When the patients were re-assessed, 10 to 18 months later, all were symptom free, but, in 2 patients, a small residual left-to-right shunt could be detected. In spite of careful preoperative studies, it is not always possible to recognize the presence of abnormally connected pulmonary veins in association with an atrial septal defect. This is an additional reason for preferring to repair atrial septal defects in general, and the combined lesion in particular, with the aid of total body perfusion. SUMMARY ~
A method for completely correcting the combined lesion of atrial septal defect and partial anomalous venous drainage is described; closure of the atrial septal defect only is not sufficient. The technique, which requires the use of total body perfusion, has been used satisfactorily on 9 patients. Methods of diagnosis are also described.
Vol. 43, No. S May, 1962
ANOMALOUS PULMONARY VENOUS DRAINAGE
633
REFERENCES 1. Von Rokitansky, C. F.: Die Defecte der Schcidewande des Herzons, Vienna, 1875, Wilhelm Braumiiller, 156 pp. 2. Hughes, C. H., and Rumore, P. C.: Anomalous Pulmonary Veins, Arch. Path. 37: 364·366, 1944. 3. Holmes Sellors, T.: Sinus Venosus Defects. Paper read at Annual Meeting of the Society of Thoracic Surgeons of Great Britain and Ireland, October, 1960. 4. Rotthoff, G., Lojacono, L., Puehetti, V., and Fahmy, A. R.: Defect of the IntraAtrial Septum With Associated Transposition of the Pulmonary Veins; Surgical Treatment, Ohir, Ital. II: 249-269, 1959. 5. Shaner, R. F.: The "High" Defect in the Atrial Septum, Canad. M. A. J. 78: 688-690, 1958. 6. Ellis, F. H., Jr., Callahan, J. A., DuShane, J. W., Edwards, J. Eo, and Wvod, Eo a.. Partial Anomalous Pulmonary Venous Connections Involving Both Lungs With Inter-Atrial Communications, Proc. Mayo Clin. 33: 65,74, 1958. 7. Edwards, J. E.: Pathologic and Developmental Considerations in Anomalous Pulmonary Venous Connection, Proe. Mayo Clin. 28: 441-452, 1953. 80 Bailey, C. P., Bolton, H. E., Jamison, W. L., and Neptune, W. R: Atrio-septo-pexy for Inter-Atrial Septal Defects, J. THORACIC SURG. 26: 184-219, 1953. 9. Neptune, W. R, Bailey, C. P., and Goldberg, H: The Surgical Correction of Atrial Septal Defects, Associated With Transposition of the Pulmonary Veins, J. THORACIC SURG. 25: 623-634, 1953. 10. Kirklin, J. W., Ellis, F. H., Jr., and Wood, E. H.: Treatment of Anomalous Pulmonary Venous Connection in Association With Inter-Atrial Communications, Surgery 39: 389-398, 1956. 11. Marion, P., Deyrieux, F., Gounot, P., and Estanoue, S.: The Problem of Surgical Treatment of Inter-Auricular Communication (Ostium Secundum) With Partial Abnormal Venous Return, Arch. mal. coeur, 53: 167-185, 1960. 12. Brom, A. G.: Paper read at the annual meeting of the Society of Thoracic Surgeons of Great Britain and Ireland, October, 1960. 13. Brock, R. C., and Ross D. N.: The Sinus Venosus TyPe of Atrial Septal Defect, Guy's Hosp. Rep. 108: 291-304, 1959. 14. Morrow, A. G., Gilbert, J. W., Baker, R. R., and Collins, N. Po: The Closure of Atrial Septal Defects Using General Hypothermia, J. THORACIC SURG. 40: 776-786, 1960. 15. Papp, C.: Cardiac Syncope in Atrial Septal Defect, Brit. Heart J. 20: 9-14, 1958. 16. Nixon, P. G. F., Grimshaw, V. A., Catchpole, L. A., Snow, H. M., and Lawrance, K: Clinical Experience With the Melrose Oxygenator at Normal and Reduced Temperatures, Thorax 15: 193-197, 1960. 17. Nixon, P. G. F.: A Pump for Use in Open Heart Surgery, Lancet 1: 1074, 1959. 18. Nixon, P. G. F., Grimshaw, V. A., and Wooler, G. H.: Clinical Observations on Vasomotor Reflexes in Relation to Blood Volume in Open Heart Surgery, Lancet 2: 1429-1431, 1960.