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Successful correction of total anomalous pulmonary venous drainage with a new surgical technique
Successful correction of total anomalous pulmonary venous drainage with a new surgical technique Yasunaru Kawashima, M.D., Susumu Nakano, M.D., Hikaru Matsuda, M.D., Takashi Miyamoto, M.D., and Hisao Manabe, M.D., Osaka, Japan
The prognosis for patients with total anomalous pulmonary venous drainage (TAPVD) has been extremely poor. Among the factors on which the results of operative intervention for this anomaly depend is the anatomic form of drainage. El-Said and his co-workers' reported that the surgical mortality rate was increased in patients with anomalous pulmonary venous drainage to the right superior vena cava (RSVC) or azygos vein. The purpose of this report is to describe the case of a l-year-old boy with T APVD to the RSVC in whom repair was successfully performed with a new operative technique not described previously in the literature. Case report A 10-month-old boy was first seen at the Osaka University Hospital in September, 1971. He was the first child born to healthy parents following a normal pregnancy and delivery. At I week of age, he was found to have cyanosis and was fed in the incubator for 50 days. He often suffered from From the First Department of Surgery, Osaka University Medical School. Osaka, Japan. Received for publication June 22, 1973. Address for reprints: Yasunaru Kawashima, M.D., First Department of Surgery, Osaka University Hospital, Doojima-hamadoori, Fukushirna-ku, Osaka, Japan.
infections of the upper respiratory tract, and his weight gain was poor. He was admitted for examination at the age of 13 months. On admission, the patient had severe cyanosis and mild clubbing of the fingers and toes. Heart rate was 140 beats per minute, and blood pressures in the arms and legs were within normal limits for his age. The lungs were clear to auscultation. A Grade 3/6 systolic murmur was heard maximally at the third left intercostal space along the sternal border. The liver's edge was palpable 3 ern. below the right midcostal margin. The hemoglobin concentration was 17.3 Gm. per 100 ml., the hematocrit was 55 per cent, and the red blood cell count was 5,460,000 per cubic millimeter. The electrocardiogram showed a normal rhythm, a mean manifest electrical axis of the QRS complex of 150 degrees, and a pattern of right ventricular hypertrophy. The chest roentgenogram demonstrated increased pulmonary vascularity and widening of the upper mediastinal shadow on right side. The cardiothoracic ratio was 0.53. Right heart catheterization revealed a left-toright shunt at the atrial level and markedly elevated right ventricular and pulmonary arterial pressures (Table I). Oxygen saturation of blood from the brachial artery was low. Angiocardiography carried out in the main pulmonary artery showed that all the pulmonary veins joined to form a common anomalous pulmonary vein which ran vertically and drained into the RSVC (Fig. 1). The left atrium was not visualized. Contrast injections into both ventricles showed a large ventricular septal defect with a right-to-left shunt.
959
The Journal of
960
Kawashima et al.
Tho ra cic a nd Card iovasc ular Surgery
Fig . 1. Anteroposterior pulmonary arterial angiogram with diagrammatic representation. The pulmonary venous drainage into the distended right superior vena cava (SVC ) is demonstrated. PA. Pulmonary artery. Common PV. Common pulmonary vein.
Table I. Preoperative cardiac catheterization data September. 1971
May. 1972
Oxygen Site
Superior vena cava Inferior vena cava Right atrium Right ventricle Pulmonary artery Pulmonary artery wedge Brachial artery
Pressure (mm, Hg)
( 6) 84/0-5 76/46 (60) (19) 80/58 (66)
Oxygen
Saturation" I (per cent}
Pressure (mm , Hg)
64 67 78 75 79
90/60 (66)
75
88/56 (68)
( 4)
I
Saturation * (per cen t)
76 63 81 79
(22)
79
Legend: Figures in parentheses indicate mean values . • Measurements were taken with the patient breathing room air .
The patient was discharged and was readmitted for elective surgery 8 months later. Cardiac cath eterization was repeated before the operation. Elevated oxygen content in the RSYC close to its junction with the right atrium was confirmed (Table 1) . The other data were identical with results of the previous catheterization. An operation was performed on July 4, 1972, through a medi an sternotomy. A vertical common anomalous vein was found to dr ain into the posterior aspect of the markedly distended RSYC a few centimeters above its junction with the right atrium. Cardiopulmonary bypass was begun after placement of catheters into the venae cavae and the ascending aorta. A superior caval tape was tightened distal to the junction of the common pulmonary vein with the vena cava. The right atrium was incised longitudina lly, and the incision was extended to the superior vena cava (SYC) . There was no atrial septal defect. The opening of
the common pulmonary vein into the RSYC was 2 em. above its junction with the right atrium and measured 7 by 5 mm. in size. As shown in Fig. 2, the posterior wall of the RSYC was incised longitudinally together with the anterior wall of the common pulmonary vein, which was just behind the SYC . The incis ion was extended longitudinally down to the atrial septum and the superior wall of the left atrium. The lower half of the incision in the anterior aspect of the common pulmonary vein was sutured to the incision on the superior wall of the left atrium. The upper half of the above-mentioned incisio n was sutured to the incision on the posterior wall of the RSYC. With a patch of Teflon , a baffle was created over the incision made on the posterior wall of the SYC and on the atrial septum, thereby directing the pulmonary venous blood under the baffle and into the left atrium. The right ventricle was then incised, and a ventricular septal defect with a
Volume 66
Total anomalous pulmonary venous drainage
Number 6
961
December, 1973
b co~mon
pulm. v.
c
d
p~~ch -, -,
.
----~
J~L~:, : ;o
, Lett atrium
Fig. 2. Method of operation. a, Preoperative anatomy. b, The posterior wall of the right superior vena cava and the atrial septum were incised together with the anterior wall of the common anomalous pulmonary vein and superior wall of the left atrium. Anastomosis of the anterior wall of the common pulmonary vein to the posterior wall of the right superior vena cava was started. c, Anastomosis of the anterior wall of the common pulmonary vein to the posterior wall of the superior vena cava and to the superior wall of the left atrium was completed. Reconstruction of the posterior wall of the superior vena cava and of the incised atrial septum was begun. d, Pulmonary venous blood flow into the left atrium after correction. VSD, Ventricular septal defect. diameter of 10 mm. was closed with a patch of Teflon. The anterior walls of the RSVC and the right atrium were closed after having been enlarged with a patch of pericardium. The right ventriculotomy was closed, and cardiopulmonary bypass was terminated. The total period of cardiopulmonary bypass was 3 hours. After the hemodynamic status became stabilized, the following pressures were recorded: right atrium, 11 mm. Hg (mean); right ventricle, 64/4 to 12 mm. Hg; pulmonary artery, 60/20 mm. Hg (mean, 38); left atrium, 13 mm. Hg (mean); left ventricle, 84/4 to 10 mm. Hg; and aorta, 80/60 mm. Hg (mean, 72). Transient
complete atrioventricular block resulted and necessitated temporary pacing while the chest was being closed. At the termination of the operation, sinus rhythm was restored. The patient recovered uneventfully from the operative procedure and was dismissed on the thirty-ninth postoperative day. At dismissal, the hemoglobin concentration was 10.7 Gm. per 100 ml., hematocrit 31 per cent, and red blood cell count 3,500,000 per cubic millimeter. The patient is now asymptomatic, free of cyanosis, free of cardiac murmur, and living a normal life with much improved activity 11 months after the operation.
The Journal of
962
Kawashima et al.
Thoracic and Cardiovascular Surgery
Comment
Various techniques of surgical repair for patients with supracardiac and infracardiac TAPVD have been utilized up to the present day. Cooley and Ochsner" reported the method of anastomosing the common pulmonary vein and posterior wall of the left atrium from within the left atrium. The right atrium was opened and the left atrial cavity was exposed by an incision through the atrial septum. An incision was made through the posterior wall of the left atrium and then through the anterior wall of the common pulmonary vein. The left atrium and common pulmonary vein were anastomosed together. Shumacker and King' improved this technique. An incision was made through the right atrial wall longitudinally and rather close to the left side. The atrial septum was either excised or opened wide through the curved incision along its right lateral attachment. A horizontal incision was then made into the common pulmonary vein to the right of the heart and was extended across the interatrial groove into right atrial wall. It was then extended through both the posterior left atrial wall and the common pulmonary vein as far as was possible. The two incisions were anastomosed from outside the heart. By continuing the line of suture beyond the left atrial wall across the interatrial groove and bringing the right end of the right atrial incision together with the remaining incision in the pulmonary vein, it was possible to make the anastomosis larger than would be possible through the atrium. Cooley and his co-workers I reported a simpler method. The heart was rotated toward the left, and the pcsterior aspect of the left atrium was anastomosed to the side of the common pulmonary vein. Cooley and Hallman" have since reported a procedure in which the apex of the heart is elevated and the common pulmonary vein is anastomosed side-to-side to the left atrium. Roe" described a technique for correc-
tion of this anomaly by exposure of the posterior aspect of the left atrium through a posterolateral thoracotomy. He reported that the exposure was excellent. Cardiac displacement was unnecessary, and a side-toside anastomosis between the common pulmonary vein and the posterior aspect of the left atrium was comfortably performed. The incision in the posterior wall of the left atrium was extended transversely from the septum to the appendage and, therefore, the resulting orifice was considerably larger than the mitral valve annulus. With this technique, however, extracorporeal circulation should be discontinued from the time that the left atrium is opened until the septal defect is closed. Behrendt and his co-workers' reported that no attempt was made to close the interatrial communication at the time of correction through a left thoracotomy, since this might have provided a safety valve for the left side of the heart should it have proved unable to handle the new load imposed upon it. Gersony and his co-workers' preferred a modification of the technique described by Shumacker and King." The right atrium was opened from the atrial appendage laterally and posteriorly to the interatrial groove. This incision was continued across the posterior aspect of the left atrium to the base of the left atrial appendage. A parallel incision was then made in the anterior wall of the common pulmonary vein, and a sideto-side anastomosis was begun at the left end of the two incisions. An over-sized anastomosis could be made by extending the anastomosis into the posterior wall of the right atrium. At present there are two groups of operative techniques for the correction of the extracardiac type of TAPVD. One is a side-to-side anastomosis of the common pulmonary vein to the left atrium from the left posterior aspect of the heart, with or without elevating the apex of the heart. The other is anastomosis from the right aspect of the heart via the atrial cavity. Both of these techniques are feasible when there is
Volume 66 Number 6
Total anomalous pulmonary venous drainage
963
December, 1973
a common pulmonary vein of good size just behind the left atrium. In patients with a variant form of infracardiac TAPVD,9.10 such as the upper and lower lobe pulmonary veins joining separately to form superior and inferior transverse veins, respectively, it is difficult technically to create an adequatesized anastomosis, because the pulmonary vein behind the left atrium is small in diameter. In patients with common anomalous pulmonary venous drainage to the RSVC, as in the present case, the anatomic situation is also unfavorable for corrective surgery with the previously described techniques, because the left pulmonary veins run separately behind the left atrium and form a common pulmonary vein posterior to the RSVC or to the right of the heart. In the present case, fortunately, the common anomalous pulmonary vein ran close to the superior aspect of the left atrium and posterior aspect to the RSVC, which was dilated. This anatomic situation enabled us to anastomose the common pulmonary vein to the posterior aspect of the RSVC and to the superior aspect of the left atrium; we then reconstructed the posterior wall of the RSVC and the incised atrial septum with a patch of Teflon without producing any residual pulmonary venous obstruction. Cooley and his co-workers:' reported three operative deaths among 7 patients undergoing corrective surgery for TAPVD to the RSVC. Gomes and his co-workers'! reported one operative death among 5 such patients. On the other hand, these investigators encountered nine and four operative deaths among 28 and 32 operative cases of the TAPVD to the left innominate vein. El-Said and his co-workers! reported recently that the operative mortality rate for patients with T APVD, excluding those with the supracardiac type connecting to the RSVC, was 30 per cent (6 deaths among 20 operative cases); none of 5 patients with supracardiac TAPVD connecting to the RSVC survived the corrective surgery. They have attributed the cause of this poor result in the latter group of patients to the
technical difficulties in the correction of the anomaly. We are not convinced that the procedure employed in the present case is always possible for the correction of this type of TAPVD. It is obvious, however, that this technique is easy to perform and serves to avoid residual pulmonary venous obstruction when employed for the suitable case. Whether the result of surgery for this particular type of TAPVD could be improved is an important question which will be answered by further experiences. Summary
A new technique was employed successfully in the surgical treatment of a l-yearold boy with T APVD to the RSVC. The right atrium and SVC were opened. A longitudinal incision was made into the posterior wall of the RSVC starting from the opening of the common pulmonary vein inferiorly. The anterior wall of the common pulmonary vein, which was just behind the RSVC, was also incised. The incision through the posterior wall of the SVC was extended inferiorly across its junction with the right atrium into the atrial septum. The superior wall of the left atrium was also incised. The anterior wall of the common pulmonary vein was anastomosed to the superior aspect of the left atrium and to the posterior aspect of the RSVC. The posterior wall of the SVC and the atrial septum were reconstructed with a patch of Teflon, and the blood from the common pulmonary vein drained into the left atrium without obstruction. The right atrium and the RSVC were closed with an autogenous pericardial patch. The patient survived complete repair and is doing quite well 11 months after the operation. The operative procedures for the correction of the extracardiac type of TAPVD were reviewed. The advantage of the repair employed in the present case was discussed. REFERENCES El-Said, G., Mullius, C. E., and McNamara, D. G.: Management of Total Anomalous Pul-
The Journal of
964
Kawashima et al.
Thoracic and Cardiovascular Surgery
2
3
4
5
6
7
monary Venous Return, Circulation 45: 1240, 1972. Cooley, D. A., and Ochsner, A., Jr.: Correction of Total Anomalous Pulmonary Venous Drainage, Surgery 42: 1014, 1957. Shumacker, H. B, Jr., and King, H.: A Modified Procedure for Complete Repair of Total Anomalous Pulmonary Venous Drainage, Surg. Gynecol, Obstet. 112: 763, 1961. Cooley, D. A., Hallman, G. L., and Leachman, R. D.: Total Anomalous Pulmonary Venous Drainage: Correction With the Use of Cardiopulmonary Bypass in 62 Cases, J. THoRAc. CARDIOVASC. SURG. 51: 88, 1966. Cooley, D. A., and Hallman, G. L.: Surgical Treatment of Congenital Heart Disease, Philadelphia, 1966, Lea & Febiger, Publishers. Roe, B. B.: Posterior Approach to Correction of Total Anomalous Pulmonary Venous Return: Further Experience, J. THORAc. CARDIOVASCo SURG. 59: 748, 1970. Behrendt, D. M., Aberdeen, E., Waterson, D. 1., and Bonham-Carter, R. E.: Total Anomalous Pulmonary Venous Drainage in Infants. I. Clinical and Hemodynamic Findings,
8
9
10
II
Methods, and Results of Operation in 37 Cases, Circulation 46: 347, 1972. Gersony, W. M., Bowman, F. 0., Jr., Steeg, C. N., Hayes, C. J., Jesse, M. J., and Maim, J. R.: Management of Total Anomalous Pulmonary Venous Drainage in Early Infancy, Circulation 43, 44: 19, 1971 (Supp!. I). Trinkle, J. K., Danielson, G. K., Noonan, J. A., and Stephens, C.: Infradiaphragmatic Total Anomalous Pulmonary Venous Return, Report of a New and Correctable Variant, Ann. Thorac. Surg. 5: 55, 1968. Nakano, S., Kawashima, Y., Miyamoto, T., Danno, M., Kitamura, S., Takano, H., Kito, Y., Matsuda, H., Kim, G., Manabe, H., Kozuka, T., and Seki, Y.: Surgical Treatment of Infracardiac Type Total Anomalous Pulmonary Venous Drainage, Heart 5: 532, 1973 (in Japanese). Gomes, M. M. R., Feldt, R. H., McGoon, D. C., and Danielson, G. K.: Total Anomalous Pulmonary Venous Connection: Surgical Considerations and Results of Operation, J. THoRAC. CARDIOVASC. SURG. 60: 116, 1970.
The prognosis for patients with total anomalous pulmonary venous drainage (TAPVD) has been extremely poor. Among the factors on which the results of operative intervention for this anomaly depend is the anatomic form of drainage. El-Said and his co-workers' reported that the surgical mortality rate was increased in patients with anomalous pulmonary venous drainage to the right superior vena cava (RSVC) or azygos vein. The purpose of this report is to describe the case of a l-year-old boy with T APVD to the RSVC in whom repair was successfully performed with a new operative technique not described previously in the literature. Case report A 10-month-old boy was first seen at the Osaka University Hospital in September, 1971. He was the first child born to healthy parents following a normal pregnancy and delivery. At I week of age, he was found to have cyanosis and was fed in the incubator for 50 days. He often suffered from From the First Department of Surgery, Osaka University Medical School. Osaka, Japan. Received for publication June 22, 1973. Address for reprints: Yasunaru Kawashima, M.D., First Department of Surgery, Osaka University Hospital, Doojima-hamadoori, Fukushirna-ku, Osaka, Japan.
infections of the upper respiratory tract, and his weight gain was poor. He was admitted for examination at the age of 13 months. On admission, the patient had severe cyanosis and mild clubbing of the fingers and toes. Heart rate was 140 beats per minute, and blood pressures in the arms and legs were within normal limits for his age. The lungs were clear to auscultation. A Grade 3/6 systolic murmur was heard maximally at the third left intercostal space along the sternal border. The liver's edge was palpable 3 ern. below the right midcostal margin. The hemoglobin concentration was 17.3 Gm. per 100 ml., the hematocrit was 55 per cent, and the red blood cell count was 5,460,000 per cubic millimeter. The electrocardiogram showed a normal rhythm, a mean manifest electrical axis of the QRS complex of 150 degrees, and a pattern of right ventricular hypertrophy. The chest roentgenogram demonstrated increased pulmonary vascularity and widening of the upper mediastinal shadow on right side. The cardiothoracic ratio was 0.53. Right heart catheterization revealed a left-toright shunt at the atrial level and markedly elevated right ventricular and pulmonary arterial pressures (Table I). Oxygen saturation of blood from the brachial artery was low. Angiocardiography carried out in the main pulmonary artery showed that all the pulmonary veins joined to form a common anomalous pulmonary vein which ran vertically and drained into the RSVC (Fig. 1). The left atrium was not visualized. Contrast injections into both ventricles showed a large ventricular septal defect with a right-to-left shunt.
959
The Journal of
960
Kawashima et al.
Tho ra cic a nd Card iovasc ular Surgery
Fig . 1. Anteroposterior pulmonary arterial angiogram with diagrammatic representation. The pulmonary venous drainage into the distended right superior vena cava (SVC ) is demonstrated. PA. Pulmonary artery. Common PV. Common pulmonary vein.
Table I. Preoperative cardiac catheterization data September. 1971
May. 1972
Oxygen Site
Superior vena cava Inferior vena cava Right atrium Right ventricle Pulmonary artery Pulmonary artery wedge Brachial artery
Pressure (mm, Hg)
( 6) 84/0-5 76/46 (60) (19) 80/58 (66)
Oxygen
Saturation" I (per cent}
Pressure (mm , Hg)
64 67 78 75 79
90/60 (66)
75
88/56 (68)
( 4)
I
Saturation * (per cen t)
76 63 81 79
(22)
79
Legend: Figures in parentheses indicate mean values . • Measurements were taken with the patient breathing room air .
The patient was discharged and was readmitted for elective surgery 8 months later. Cardiac cath eterization was repeated before the operation. Elevated oxygen content in the RSYC close to its junction with the right atrium was confirmed (Table 1) . The other data were identical with results of the previous catheterization. An operation was performed on July 4, 1972, through a medi an sternotomy. A vertical common anomalous vein was found to dr ain into the posterior aspect of the markedly distended RSYC a few centimeters above its junction with the right atrium. Cardiopulmonary bypass was begun after placement of catheters into the venae cavae and the ascending aorta. A superior caval tape was tightened distal to the junction of the common pulmonary vein with the vena cava. The right atrium was incised longitudina lly, and the incision was extended to the superior vena cava (SYC) . There was no atrial septal defect. The opening of
the common pulmonary vein into the RSYC was 2 em. above its junction with the right atrium and measured 7 by 5 mm. in size. As shown in Fig. 2, the posterior wall of the RSYC was incised longitudinally together with the anterior wall of the common pulmonary vein, which was just behind the SYC . The incis ion was extended longitudinally down to the atrial septum and the superior wall of the left atrium. The lower half of the incision in the anterior aspect of the common pulmonary vein was sutured to the incision on the superior wall of the left atrium. The upper half of the above-mentioned incisio n was sutured to the incision on the posterior wall of the RSYC. With a patch of Teflon , a baffle was created over the incision made on the posterior wall of the SYC and on the atrial septum, thereby directing the pulmonary venous blood under the baffle and into the left atrium. The right ventricle was then incised, and a ventricular septal defect with a
Volume 66
Total anomalous pulmonary venous drainage
Number 6
961
December, 1973
b co~mon
pulm. v.
c
d
p~~ch -, -,
.
----~
J~L~:, : ;o
, Lett atrium
Fig. 2. Method of operation. a, Preoperative anatomy. b, The posterior wall of the right superior vena cava and the atrial septum were incised together with the anterior wall of the common anomalous pulmonary vein and superior wall of the left atrium. Anastomosis of the anterior wall of the common pulmonary vein to the posterior wall of the right superior vena cava was started. c, Anastomosis of the anterior wall of the common pulmonary vein to the posterior wall of the superior vena cava and to the superior wall of the left atrium was completed. Reconstruction of the posterior wall of the superior vena cava and of the incised atrial septum was begun. d, Pulmonary venous blood flow into the left atrium after correction. VSD, Ventricular septal defect. diameter of 10 mm. was closed with a patch of Teflon. The anterior walls of the RSVC and the right atrium were closed after having been enlarged with a patch of pericardium. The right ventriculotomy was closed, and cardiopulmonary bypass was terminated. The total period of cardiopulmonary bypass was 3 hours. After the hemodynamic status became stabilized, the following pressures were recorded: right atrium, 11 mm. Hg (mean); right ventricle, 64/4 to 12 mm. Hg; pulmonary artery, 60/20 mm. Hg (mean, 38); left atrium, 13 mm. Hg (mean); left ventricle, 84/4 to 10 mm. Hg; and aorta, 80/60 mm. Hg (mean, 72). Transient
complete atrioventricular block resulted and necessitated temporary pacing while the chest was being closed. At the termination of the operation, sinus rhythm was restored. The patient recovered uneventfully from the operative procedure and was dismissed on the thirty-ninth postoperative day. At dismissal, the hemoglobin concentration was 10.7 Gm. per 100 ml., hematocrit 31 per cent, and red blood cell count 3,500,000 per cubic millimeter. The patient is now asymptomatic, free of cyanosis, free of cardiac murmur, and living a normal life with much improved activity 11 months after the operation.
The Journal of
962
Kawashima et al.
Thoracic and Cardiovascular Surgery
Comment
Various techniques of surgical repair for patients with supracardiac and infracardiac TAPVD have been utilized up to the present day. Cooley and Ochsner" reported the method of anastomosing the common pulmonary vein and posterior wall of the left atrium from within the left atrium. The right atrium was opened and the left atrial cavity was exposed by an incision through the atrial septum. An incision was made through the posterior wall of the left atrium and then through the anterior wall of the common pulmonary vein. The left atrium and common pulmonary vein were anastomosed together. Shumacker and King' improved this technique. An incision was made through the right atrial wall longitudinally and rather close to the left side. The atrial septum was either excised or opened wide through the curved incision along its right lateral attachment. A horizontal incision was then made into the common pulmonary vein to the right of the heart and was extended across the interatrial groove into right atrial wall. It was then extended through both the posterior left atrial wall and the common pulmonary vein as far as was possible. The two incisions were anastomosed from outside the heart. By continuing the line of suture beyond the left atrial wall across the interatrial groove and bringing the right end of the right atrial incision together with the remaining incision in the pulmonary vein, it was possible to make the anastomosis larger than would be possible through the atrium. Cooley and his co-workers I reported a simpler method. The heart was rotated toward the left, and the pcsterior aspect of the left atrium was anastomosed to the side of the common pulmonary vein. Cooley and Hallman" have since reported a procedure in which the apex of the heart is elevated and the common pulmonary vein is anastomosed side-to-side to the left atrium. Roe" described a technique for correc-
tion of this anomaly by exposure of the posterior aspect of the left atrium through a posterolateral thoracotomy. He reported that the exposure was excellent. Cardiac displacement was unnecessary, and a side-toside anastomosis between the common pulmonary vein and the posterior aspect of the left atrium was comfortably performed. The incision in the posterior wall of the left atrium was extended transversely from the septum to the appendage and, therefore, the resulting orifice was considerably larger than the mitral valve annulus. With this technique, however, extracorporeal circulation should be discontinued from the time that the left atrium is opened until the septal defect is closed. Behrendt and his co-workers' reported that no attempt was made to close the interatrial communication at the time of correction through a left thoracotomy, since this might have provided a safety valve for the left side of the heart should it have proved unable to handle the new load imposed upon it. Gersony and his co-workers' preferred a modification of the technique described by Shumacker and King." The right atrium was opened from the atrial appendage laterally and posteriorly to the interatrial groove. This incision was continued across the posterior aspect of the left atrium to the base of the left atrial appendage. A parallel incision was then made in the anterior wall of the common pulmonary vein, and a sideto-side anastomosis was begun at the left end of the two incisions. An over-sized anastomosis could be made by extending the anastomosis into the posterior wall of the right atrium. At present there are two groups of operative techniques for the correction of the extracardiac type of TAPVD. One is a side-to-side anastomosis of the common pulmonary vein to the left atrium from the left posterior aspect of the heart, with or without elevating the apex of the heart. The other is anastomosis from the right aspect of the heart via the atrial cavity. Both of these techniques are feasible when there is
Volume 66 Number 6
Total anomalous pulmonary venous drainage
963
December, 1973
a common pulmonary vein of good size just behind the left atrium. In patients with a variant form of infracardiac TAPVD,9.10 such as the upper and lower lobe pulmonary veins joining separately to form superior and inferior transverse veins, respectively, it is difficult technically to create an adequatesized anastomosis, because the pulmonary vein behind the left atrium is small in diameter. In patients with common anomalous pulmonary venous drainage to the RSVC, as in the present case, the anatomic situation is also unfavorable for corrective surgery with the previously described techniques, because the left pulmonary veins run separately behind the left atrium and form a common pulmonary vein posterior to the RSVC or to the right of the heart. In the present case, fortunately, the common anomalous pulmonary vein ran close to the superior aspect of the left atrium and posterior aspect to the RSVC, which was dilated. This anatomic situation enabled us to anastomose the common pulmonary vein to the posterior aspect of the RSVC and to the superior aspect of the left atrium; we then reconstructed the posterior wall of the RSVC and the incised atrial septum with a patch of Teflon without producing any residual pulmonary venous obstruction. Cooley and his co-workers:' reported three operative deaths among 7 patients undergoing corrective surgery for TAPVD to the RSVC. Gomes and his co-workers'! reported one operative death among 5 such patients. On the other hand, these investigators encountered nine and four operative deaths among 28 and 32 operative cases of the TAPVD to the left innominate vein. El-Said and his co-workers! reported recently that the operative mortality rate for patients with T APVD, excluding those with the supracardiac type connecting to the RSVC, was 30 per cent (6 deaths among 20 operative cases); none of 5 patients with supracardiac TAPVD connecting to the RSVC survived the corrective surgery. They have attributed the cause of this poor result in the latter group of patients to the
technical difficulties in the correction of the anomaly. We are not convinced that the procedure employed in the present case is always possible for the correction of this type of TAPVD. It is obvious, however, that this technique is easy to perform and serves to avoid residual pulmonary venous obstruction when employed for the suitable case. Whether the result of surgery for this particular type of TAPVD could be improved is an important question which will be answered by further experiences. Summary
A new technique was employed successfully in the surgical treatment of a l-yearold boy with T APVD to the RSVC. The right atrium and SVC were opened. A longitudinal incision was made into the posterior wall of the RSVC starting from the opening of the common pulmonary vein inferiorly. The anterior wall of the common pulmonary vein, which was just behind the RSVC, was also incised. The incision through the posterior wall of the SVC was extended inferiorly across its junction with the right atrium into the atrial septum. The superior wall of the left atrium was also incised. The anterior wall of the common pulmonary vein was anastomosed to the superior aspect of the left atrium and to the posterior aspect of the RSVC. The posterior wall of the SVC and the atrial septum were reconstructed with a patch of Teflon, and the blood from the common pulmonary vein drained into the left atrium without obstruction. The right atrium and the RSVC were closed with an autogenous pericardial patch. The patient survived complete repair and is doing quite well 11 months after the operation. The operative procedures for the correction of the extracardiac type of TAPVD were reviewed. The advantage of the repair employed in the present case was discussed. REFERENCES El-Said, G., Mullius, C. E., and McNamara, D. G.: Management of Total Anomalous Pul-
The Journal of
964
Kawashima et al.
Thoracic and Cardiovascular Surgery
2
3
4
5
6
7
monary Venous Return, Circulation 45: 1240, 1972. Cooley, D. A., and Ochsner, A., Jr.: Correction of Total Anomalous Pulmonary Venous Drainage, Surgery 42: 1014, 1957. Shumacker, H. B, Jr., and King, H.: A Modified Procedure for Complete Repair of Total Anomalous Pulmonary Venous Drainage, Surg. Gynecol, Obstet. 112: 763, 1961. Cooley, D. A., Hallman, G. L., and Leachman, R. D.: Total Anomalous Pulmonary Venous Drainage: Correction With the Use of Cardiopulmonary Bypass in 62 Cases, J. THoRAc. CARDIOVASC. SURG. 51: 88, 1966. Cooley, D. A., and Hallman, G. L.: Surgical Treatment of Congenital Heart Disease, Philadelphia, 1966, Lea & Febiger, Publishers. Roe, B. B.: Posterior Approach to Correction of Total Anomalous Pulmonary Venous Return: Further Experience, J. THORAc. CARDIOVASCo SURG. 59: 748, 1970. Behrendt, D. M., Aberdeen, E., Waterson, D. 1., and Bonham-Carter, R. E.: Total Anomalous Pulmonary Venous Drainage in Infants. I. Clinical and Hemodynamic Findings,
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Methods, and Results of Operation in 37 Cases, Circulation 46: 347, 1972. Gersony, W. M., Bowman, F. 0., Jr., Steeg, C. N., Hayes, C. J., Jesse, M. J., and Maim, J. R.: Management of Total Anomalous Pulmonary Venous Drainage in Early Infancy, Circulation 43, 44: 19, 1971 (Supp!. I). Trinkle, J. K., Danielson, G. K., Noonan, J. A., and Stephens, C.: Infradiaphragmatic Total Anomalous Pulmonary Venous Return, Report of a New and Correctable Variant, Ann. Thorac. Surg. 5: 55, 1968. Nakano, S., Kawashima, Y., Miyamoto, T., Danno, M., Kitamura, S., Takano, H., Kito, Y., Matsuda, H., Kim, G., Manabe, H., Kozuka, T., and Seki, Y.: Surgical Treatment of Infracardiac Type Total Anomalous Pulmonary Venous Drainage, Heart 5: 532, 1973 (in Japanese). Gomes, M. M. R., Feldt, R. H., McGoon, D. C., and Danielson, G. K.: Total Anomalous Pulmonary Venous Connection: Surgical Considerations and Results of Operation, J. THoRAC. CARDIOVASC. SURG. 60: 116, 1970.