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Total correction of tetralogy of Fallot associated with an anomalous left pulmonary artery arising from the aortic arch
Total correction of tetralogy of Fallot associated with an anomalous left pulmonary artery arising from the aortic arch A case of tetralogy of Fallot com plicated by the anomalous origin of the left pulmonary artery from the aortic arch is report ed. Total correction by me ans of a modified Y-shaped patch with a monocusp porcine valve was performed with success.
Yasuharu Imai, M.D., Yasushi Nishiya, M.D., Tetsuo Morikawa, M.D ., Hiromi Kurosawa, M.D ., and Souji Konno, M.D., Tokyo, Japan
Tetralogy of Fallot associated with anomalous origin of the unilateral pulmonary artery from the aorta is a rare condition. Approximately 20 such cases have been documented to date, but only 3 cases of successful total correction have been reported .•' 1 3, 16 The purpose of this communication is to report an additional case in which an anomalous left pulmonary artery originated from the aortic arch. The outflow tract was reconstructed with a monocusp patch graft whose arm was anastomosed to the left pulmonary artery . The pulmonary trunk in this case was severely hypoplastic, and outflow reconstruction with a patch seemed mandatory. In addition, the relatively long distance between the pulmonary trunk and the left pulmonary artery made a direct anastomosis infeasible. The usual procedure of choice in this situation is to use a Y-shaped vascular prosthesis as a bypass graft with anastomoses to both pulmonary arteries. Since we believed that From the Heart In stitute of J apan. Tokyo Women's Med ica l Colle ge. 10 Kawadacho , Shin jukuku, Tokyo, Japan. Re ceived for publicat ion Nov. 26, 1973.
incorporation of the host tissue in the outflow reconstruction would facilitate future growth of the pulmonary trunk, we utilized an outflow patch with a porcine monocusp sutured to its inner surface; one arm was attached to the patch for the anastomosis to the left pulmonary artery (see Fig. 6) . The
Fig. 1. Preoperative chest x-ray film. Decreased pulmonary blood flow is noted.
51
The Journal of
52
Imai et ai.
Thoro cic and Cardiovascular Surgery
Fig. 2. Preoperative electrocardiogram (Feb. 18, 1972).
Table I Parameter RBC (rnillion/ c.c. ) Hemoglobin (Gm.) Hematocrit ( %) Capillary oxygen tension (mm. Hg)
Preop . value
Postop , value
6.7 18.8 56
4.8 14.6 44
42
89
Le gend: RBC. Red blood count.
operation was performed with relative ease by this method, and the patient is leading a
normal life 20 months after surgery. Case report The patient was a 6-year-01d boy who was first found to have cyanosis and a heart murmur 2 days after birth. The diagnosis of tetralogy of Fallot was made by cardiac catheterization and angiography when the child was 16 months old. On admi ssion, physical examination revealed a moderately well-developed but slender boy in no acute distre ss. His height was 123 em. and his body weight 21.5 kilograms. There was moderate cyanosis of the lips and marked finger clubbing (Table I). The pulse rate was 76 beats per minute and regular. On auscultation, there was a Grade 4/6 systolic ejection murmur best heard in the fourth intercostal space along the left sternal border; a Grade 2/6 continuous murmur could be heard in the left second intercostal space . The chest x-ray film showed a decrease in pulmonary vascularity without cardiomegaly (Fig. I ). The electrocardiogram revealed a regular sinus rhythm, right axis deviation with a mean frontal QRS axis of 110 degrees, and right ventricular hypertrophy (Fig. 2). The
right ventriculogram demonstrated early opacification of the aorta as well as infundibular and pulmonary valvular stenoses (Fig. 3). The pulmonary valve ring was 7 mm. in diameter, and the pulmonary trunk was markedly hypoplastic with a pulmonary artery-aorta ratio of 40 per cent. The proximal portion of the left pulmonary artery was absent, and the intrapulmonary artery was only faintly opacified via the patent ductus (Fig. 4) . Findings of retrograde aortography substantiated the diagnosis of anomalous origin of the left pulmonary artery from the arch of the aorta. Therefore, total correction was undertaken with the diagnosis of tetralogy of Fallot with anomalous origin of the left pulmonary artery. The operation was performed on March 15, 1972. A median sternotomy incision was employed with the patient in the supine position . F1uothane and nitrous oxide anesthesia was administered endotracheally. Extracorporeal circulation was then instituted via our disposable bubble oxygenator with profound perfusion cooling down to 22 C. The right ventricle was opened through a long itudinal incision that extended up into the pulmonary trunk. A ventricular septal defect mea suring 2 by 3 em. was closed with Teflon velour. The area of the pulmonary bifurcation was dissected meticulously to locate the left pulmonary artery. The vessel was found to originate from the patent ductu s, which was 3 mm . in diameter. A fibrous strand without a lumen connected the left pulmonary artery and the pulmonary trunk. The diameter of the distal pulmonary artery was 6 mm. (Fig. 5). In order to reconstruct the outflow tract of the right ventricle, we employed a V-shaped vascular graft. One arm of the graft was divided and suture-closed at its origin, and the inferior aspect of the vertical limb was trimmed away in an oblique fashion to create an oval opening . A 0
Volume 68 Number 1
Tetralogy of Fallot
July, 1974
Fig. 3. Preoperative right ventriculograms. Arrows in the lateral view (left) indicate patent ductus arteriosus (PDA) and valvular pulmonic stenosis (PS). In the frontal projection (right), note absence of the proximal portion of the left pulmonary artery. Ao, Aorta. RPA, Right pulmonary artery. RV, Right ventricle.
Fig. 4. Retrograde aortograms revealed a patent ductus arteriosus (PDA) and the left pulmonary artery (LPA). Ao, Aorta. CoA, Coronary artery, left.
53
54
Imai et al.
The Journol of Thoracic and Card iovascular Surgery
Fig. 5. Threads A are passed around the patent ductus arteriosus, B is around the distal portion of the left pulmonary artery , and C encircles the fibrous strand without bridging the lumen between the left pulmonary artery and the trunk.
Fig. 6. A modified Y-graft patch used for reconstruction of the outflow tract.
Fig. 7. Reconstruction of the outflow tract and of the anomalous left pulmonary artery. The broken line indicates the line of division.
monocusp porcine valve preserved in buffered formalin solution was attached to the inner surface of the graft (Fig. 6). The outflow reconstruction was thus completed with this modified Y-shaped graft. The remaining arm of the graft was anastomosed to the proximal end of the left pulmonary artery (Figs. 7 and 8) . The patient made an uneventful recovery post-
operatively . One month after the operation, right heart angiography provided good visualization of the left pulmonary artery as well as displaying a well-formed right ventricular outflow tract (Fig . 9) . The chest x-ray film showed a slightly larger cardiac silhouette and normal pulmonary vascularity (Fig. 10). The child is doing well with no problems.
Volume 68
55
Tetralogy of Fallot
Number 1 July, 1974
Discussion The pathogenesis of unilateral anomalous origin of the pulmonary artery was interpreted by Cucci" from embryology. According to his classification, this entity falls into three types: A. The unilateral anomalous pulmonary artery arises from the ascending aorta, with persistence of the proximal sixth arch and regression of its distal portion. B. The unilateral anomalous pulmonary artery arises from the aortic arch, with persistence of the distal sixth arch and regression of its proximal portion. C. The pulmonary artery is unilaterally absent, with regression of both the proximal and distal arches. Our case is Type B according to this classification. Pool- made a statistical survey on other anomalies associated with anomalous origin of the unilateral pulmonary artery. Tetralogy of Fallot was found in 40 per cent of cases in which the left pulmonary artery originated anomalously (Table II). Surgical correction of this anomaly was first done on the right side in 1961 by Armer. 3 He disconnected the anomalous artery from the ascending aorta and reestablished its continuity by interposing a Dacron graft between the pulmonary trunk and the right pulmonary artery. On the other hand, anomalous left pulmonary artery from the ascending aorta was first corrected by Weintraub? in 1966 by direct anastomosis of the left pulmonary artery to the pulmonary trunk. Although 13 cases of successful surgical correction have been reported to date,"?" there were no associated intracardiac anomalies in most instances (Table III). Kirklin' was the first to perform total correction on a patient with anomalous left pulmonary artery associated with an intracardiac anomaly. In his patient the anomalous artery originated from the aorta, and the associated defect was tetralogy of Fallot. The patient was cured by intracardiac repair with direct anastomosis between the pul-
Fig. 8. The reconstructed outflow tract and left pulmonary artery upon completion of the procedure. P, Patch. A, Aorta.
Tahle 11* Associated anomaly None Tetralogy of Fallot PDA VSD Miscellaneous Totals
I
Anomalous Anomalous
LPA
RPA
14
18
16
1
1 5 4
10
40
4
5 38
Legend: LPA, Left pulmonary artery. RPA, Right pulmonary artery. PDA, Patent ductus arteriosus. VSD, Ventricular septal defect. 'From Pool, P. E., et al.: Am. J. Cardiol. 10:706, 1962, published by Dun-Donnelley Publishing Corporation, New
York, N. Y.
monary trunk and the left pulmonary artery. No detailed information on the operation was given. Subsequently, Kawada'" in 1971 performed a successful two-stage operation in a case of atypical tetralogy with anomalous left pulmonary artery from the aortic arch and absence of the pulmonary valve. In 1972 Morgan'" successfully performed a
56
Imai et al.
The Journal of Thorocic ond Cardiovascular Surgery
Fig. 9. Postoperative right heart angiograms. Arrow indicates the porcine monocusp. Two solid lines indicate the extent of the outflow patch. Note normal opacification of the left pulmonary artery (LPA). RPA, Right pulmonary artery. RV, Right ventricle.
Fig. 10. Postoperative chest x-ray film shows normal pulmonary vascularity with a slight increase in cardiac size.
single-stage correction of tetralogy associated with an anomalous left pulmonary artery from the ascending aorta. Czarnecki" in 1964 reported a similar case of tetralogy in which the left pulmonary artery originated
from the distal portion of the aortic arch. However, failure to recognize this anomaly at operation resulted in the patient's death. Although total correction was successful in the 3 reported cases of this anomaly associated with tetralogy, direct anastomosis between the pulmonary trunk and the anomalous pulmonary artery was feasible in all. Therefore, our patient seems to be the first to have required the method of repair that we devised-that is, anastomosis between the left pulmonary artery and the prosthetic graft as well as reconstruction of the outflow tract with a patch retaining a porcine monocusp. Our method of reconstruction would have a wider application for those cases in which the anomalous artery has a stenotic segment at or near its origin. Controversies still exist regarding the significance of pulmonary regurgitation on right ventricular function. It is our impression from clinical experience that pulmonic regurgitation plays an important role in causing right ventricular failure in the immediate postoperative
Volume 68 Number 1 July, 1974
Tetralogy of Fallot
i
57
'1
I , j ._
1·1
..;.Ji...........
y' I ' I. I
I
I
!
....c..J...L.L 1
I
-. I
IT
ill ~I
I
.1
Vs Fig. 11. Postoperative electrocardiogram (April 3, 1972).
Table III. Unilateral anomalous pulmonary artery Author
Year
Side
Associated anomaly
Armer et al .> Kirklin et al.! Redo et a\.5 Weintraub et a\.6 Kirkpatrick et a1. 7 Green et a\.s Kuypers et a1. 9 Caudill et al.r° Flege et al. l l Takeuchi et a\.12 Kawada et al. 13 Hosokawa et al. 14 Schiller et al.I 5 Morgan-s Imai et a\.
1961 1965 1965 1966 1967 1968 1969 1969 1970 1970 1971 1971 1971 1972 1974
RPA-AA LPA-arch RPA-AA LPA-AA RPA-AA LPA-arch RPA-AA LPA-AA RPA-AA RPA-AA LPA-arch RPA-AA LPA-AA LPA-AA LPA-arch
Patent ductus Tetralogy Patent ductus None Patent ductus None Patent ductus None Ventricular septal defect Patent ductus Tetralogy Patent ductus None Tetralogy Tetralogy
Legend: RP A-AA, Origin of right pulmonary artery from ascending aorta. LP A·AA, Origin of left pulmonary artery from ascending aorta. LP A-arch, Origin of left pulmonary artery from aortic arch.
period, even though it has little significance in a normal heart. The fate of the porcine valve is still in doubt. We have followed this material for only 3 years. The tissue was in good functioning condition at that time, but a longer period of observation is necessary.
4
5
REFERENCES Cucci , C. E., Doyle, E. F., and Lewis, E. W., Jr .: Absence of a Primary Division of the Pulmonary Trunk, Circulation 29: 124, 1964. 2 Pool, P. E., Vogel, J. H. K., and Blount, G. S., Jr.: Congenital Unilateral Absence of a Pulmonary Artery, Am. 1. Cardio\. 10: 706, 1962. 3 Armer, R. M., Shumacker, H. B, and Klatte,
6
7
E. C.: Origin of the Right Pulmonary Artery From the Ascending Aorta, Circulation 24: 662, 1961. Kirklin, J. W., Wallace, R. B., McGoon, D. C., and DuShane, J. W.: Early and Late Results After Intracardiac Repair of Tetralogy of Fallot, Ann . Surg. 162: 578, 1965. Redo , S. F. , Foster, H. R., Engle, M. A., and Ehlers, K. H.: Anomalous Origin of the Right Pulmonary Artery From the Ascending Aorta, J. THORAC. CARDIOVASC. SURG. SO: 726, 1965. Weintraub, R. A, Fabian, C. E., and Adams, D. F.: Ectopic Origin of One Pulmonary Artery From the Ascending Aorta, Radiology 86: 666, 1966. Kirkpatrick, S. E., Girod, D. A., and King, H.: Aortic Orig in of the Right Pulmonary Artery, Circulation 36: 777, 1967.
The Journal of
5 8 Imai et at.
Thoracic and Cordiovasculor
Surgery
8 Green, G. E., Reppert, E. H., Cohlan, S. Q., and Spencer, F. C.: Surgical Correction of Absence of Proximal Segment of Left Pulmonary Artery, Circulation 37: 62, 1968. 9 Kuypers, P. J., van der Maas, A. H., and Busch, H. J.: Origin of the Right Pulmonary Artery From the Aorta With Patent Ductus Arteriosus, 1. THoRAe. CARDIOVASC. SURG. 57: 185, 1969. 10 Caudill, D. R., Helmsworth, J. A., Daoud, G., and Kaplan, S.: Anomalous Origin of Left Pulmonary Artery From Ascending Aorta, J. THORAC. CARDIOVASC. SURG. 57: 493, 1969. 11 Flege, 1. B., Durnin, R. E., and Rossi, N. P.: Aortic Origin of the Right Pulmonary Artery and Ventricular Septal Defect, J. THORAC. CARDIOVASC. SURG. 59: 468, 1970. 12 Takeuchi, N., Fukuda, T., Tsuji, A., and Shozu, A.: Successful Surgical Correction of a Case With the Right Pulmonary Artery Originating From Ascending Aorta and Patent Ductus Arteriosus, Shinzo 2: 737, 1970 (in Japanese). 13 Kawada, S., Nagamine, I., Nishikawa, K., Kawada, K., Inoue, T., Hata, J., Kojima, E., Ishiwatari, K., and Kamimura, H.: Successful
14
15
16
17
Surgical Correction of a Case of Tetralogy of Fallot Associated With an Anomalous Left Pulmonary Artery Originating From Aortic Arch and Absence of the Pulmonary Valve, Shinzo 3: 1073, 1971 (in Japanese). Hosokawa, Y., Okamura, H., Nakamura, T., Sagara, T., Matsuoka, T., Kamishimo, J., Kobayashi, H., Endo, T., Matsumoto, H., Kabe, T., Saito, M., and Soda, T.: Successful Surgical Correction of a Case With Right Pulmonary Artery Originating From Ascending Aorta and Patent Ductus Arteriosus Using a Simple Deep Hypothermia Technique, Shinzo 5: 126 1971 (in Japanese). Schiller, M., Williams, T. E., Craenen, J., Hosier, D. M., and Sirak, H. D.: Anomalous Origin of Left Pulmonary Artery From the Ascending Aorta, Vase. Surg. 5: 126, 1971. Morgan, J. R.: Left Pulmonary Artery From Ascending Aorta in Tetralogy of Fallot, Circulation 45: 653, 1972. Czarnecki, S. W., Hopeman, A. R., and Child, P. L.: Tetralogy of Fallot With Aortic Origin of the Left Pulmonary Artery, Chest 46: 97, 1964.
Yasuharu Imai, M.D., Yasushi Nishiya, M.D., Tetsuo Morikawa, M.D ., Hiromi Kurosawa, M.D ., and Souji Konno, M.D., Tokyo, Japan
Tetralogy of Fallot associated with anomalous origin of the unilateral pulmonary artery from the aorta is a rare condition. Approximately 20 such cases have been documented to date, but only 3 cases of successful total correction have been reported .•' 1 3, 16 The purpose of this communication is to report an additional case in which an anomalous left pulmonary artery originated from the aortic arch. The outflow tract was reconstructed with a monocusp patch graft whose arm was anastomosed to the left pulmonary artery . The pulmonary trunk in this case was severely hypoplastic, and outflow reconstruction with a patch seemed mandatory. In addition, the relatively long distance between the pulmonary trunk and the left pulmonary artery made a direct anastomosis infeasible. The usual procedure of choice in this situation is to use a Y-shaped vascular prosthesis as a bypass graft with anastomoses to both pulmonary arteries. Since we believed that From the Heart In stitute of J apan. Tokyo Women's Med ica l Colle ge. 10 Kawadacho , Shin jukuku, Tokyo, Japan. Re ceived for publicat ion Nov. 26, 1973.
incorporation of the host tissue in the outflow reconstruction would facilitate future growth of the pulmonary trunk, we utilized an outflow patch with a porcine monocusp sutured to its inner surface; one arm was attached to the patch for the anastomosis to the left pulmonary artery (see Fig. 6) . The
Fig. 1. Preoperative chest x-ray film. Decreased pulmonary blood flow is noted.
51
The Journal of
52
Imai et ai.
Thoro cic and Cardiovascular Surgery
Fig. 2. Preoperative electrocardiogram (Feb. 18, 1972).
Table I Parameter RBC (rnillion/ c.c. ) Hemoglobin (Gm.) Hematocrit ( %) Capillary oxygen tension (mm. Hg)
Preop . value
Postop , value
6.7 18.8 56
4.8 14.6 44
42
89
Le gend: RBC. Red blood count.
operation was performed with relative ease by this method, and the patient is leading a
normal life 20 months after surgery. Case report The patient was a 6-year-01d boy who was first found to have cyanosis and a heart murmur 2 days after birth. The diagnosis of tetralogy of Fallot was made by cardiac catheterization and angiography when the child was 16 months old. On admi ssion, physical examination revealed a moderately well-developed but slender boy in no acute distre ss. His height was 123 em. and his body weight 21.5 kilograms. There was moderate cyanosis of the lips and marked finger clubbing (Table I). The pulse rate was 76 beats per minute and regular. On auscultation, there was a Grade 4/6 systolic ejection murmur best heard in the fourth intercostal space along the left sternal border; a Grade 2/6 continuous murmur could be heard in the left second intercostal space . The chest x-ray film showed a decrease in pulmonary vascularity without cardiomegaly (Fig. I ). The electrocardiogram revealed a regular sinus rhythm, right axis deviation with a mean frontal QRS axis of 110 degrees, and right ventricular hypertrophy (Fig. 2). The
right ventriculogram demonstrated early opacification of the aorta as well as infundibular and pulmonary valvular stenoses (Fig. 3). The pulmonary valve ring was 7 mm. in diameter, and the pulmonary trunk was markedly hypoplastic with a pulmonary artery-aorta ratio of 40 per cent. The proximal portion of the left pulmonary artery was absent, and the intrapulmonary artery was only faintly opacified via the patent ductus (Fig. 4) . Findings of retrograde aortography substantiated the diagnosis of anomalous origin of the left pulmonary artery from the arch of the aorta. Therefore, total correction was undertaken with the diagnosis of tetralogy of Fallot with anomalous origin of the left pulmonary artery. The operation was performed on March 15, 1972. A median sternotomy incision was employed with the patient in the supine position . F1uothane and nitrous oxide anesthesia was administered endotracheally. Extracorporeal circulation was then instituted via our disposable bubble oxygenator with profound perfusion cooling down to 22 C. The right ventricle was opened through a long itudinal incision that extended up into the pulmonary trunk. A ventricular septal defect mea suring 2 by 3 em. was closed with Teflon velour. The area of the pulmonary bifurcation was dissected meticulously to locate the left pulmonary artery. The vessel was found to originate from the patent ductu s, which was 3 mm . in diameter. A fibrous strand without a lumen connected the left pulmonary artery and the pulmonary trunk. The diameter of the distal pulmonary artery was 6 mm. (Fig. 5). In order to reconstruct the outflow tract of the right ventricle, we employed a V-shaped vascular graft. One arm of the graft was divided and suture-closed at its origin, and the inferior aspect of the vertical limb was trimmed away in an oblique fashion to create an oval opening . A 0
Volume 68 Number 1
Tetralogy of Fallot
July, 1974
Fig. 3. Preoperative right ventriculograms. Arrows in the lateral view (left) indicate patent ductus arteriosus (PDA) and valvular pulmonic stenosis (PS). In the frontal projection (right), note absence of the proximal portion of the left pulmonary artery. Ao, Aorta. RPA, Right pulmonary artery. RV, Right ventricle.
Fig. 4. Retrograde aortograms revealed a patent ductus arteriosus (PDA) and the left pulmonary artery (LPA). Ao, Aorta. CoA, Coronary artery, left.
53
54
Imai et al.
The Journol of Thoracic and Card iovascular Surgery
Fig. 5. Threads A are passed around the patent ductus arteriosus, B is around the distal portion of the left pulmonary artery , and C encircles the fibrous strand without bridging the lumen between the left pulmonary artery and the trunk.
Fig. 6. A modified Y-graft patch used for reconstruction of the outflow tract.
Fig. 7. Reconstruction of the outflow tract and of the anomalous left pulmonary artery. The broken line indicates the line of division.
monocusp porcine valve preserved in buffered formalin solution was attached to the inner surface of the graft (Fig. 6). The outflow reconstruction was thus completed with this modified Y-shaped graft. The remaining arm of the graft was anastomosed to the proximal end of the left pulmonary artery (Figs. 7 and 8) . The patient made an uneventful recovery post-
operatively . One month after the operation, right heart angiography provided good visualization of the left pulmonary artery as well as displaying a well-formed right ventricular outflow tract (Fig . 9) . The chest x-ray film showed a slightly larger cardiac silhouette and normal pulmonary vascularity (Fig. 10). The child is doing well with no problems.
Volume 68
55
Tetralogy of Fallot
Number 1 July, 1974
Discussion The pathogenesis of unilateral anomalous origin of the pulmonary artery was interpreted by Cucci" from embryology. According to his classification, this entity falls into three types: A. The unilateral anomalous pulmonary artery arises from the ascending aorta, with persistence of the proximal sixth arch and regression of its distal portion. B. The unilateral anomalous pulmonary artery arises from the aortic arch, with persistence of the distal sixth arch and regression of its proximal portion. C. The pulmonary artery is unilaterally absent, with regression of both the proximal and distal arches. Our case is Type B according to this classification. Pool- made a statistical survey on other anomalies associated with anomalous origin of the unilateral pulmonary artery. Tetralogy of Fallot was found in 40 per cent of cases in which the left pulmonary artery originated anomalously (Table II). Surgical correction of this anomaly was first done on the right side in 1961 by Armer. 3 He disconnected the anomalous artery from the ascending aorta and reestablished its continuity by interposing a Dacron graft between the pulmonary trunk and the right pulmonary artery. On the other hand, anomalous left pulmonary artery from the ascending aorta was first corrected by Weintraub? in 1966 by direct anastomosis of the left pulmonary artery to the pulmonary trunk. Although 13 cases of successful surgical correction have been reported to date,"?" there were no associated intracardiac anomalies in most instances (Table III). Kirklin' was the first to perform total correction on a patient with anomalous left pulmonary artery associated with an intracardiac anomaly. In his patient the anomalous artery originated from the aorta, and the associated defect was tetralogy of Fallot. The patient was cured by intracardiac repair with direct anastomosis between the pul-
Fig. 8. The reconstructed outflow tract and left pulmonary artery upon completion of the procedure. P, Patch. A, Aorta.
Tahle 11* Associated anomaly None Tetralogy of Fallot PDA VSD Miscellaneous Totals
I
Anomalous Anomalous
LPA
RPA
14
18
16
1
1 5 4
10
40
4
5 38
Legend: LPA, Left pulmonary artery. RPA, Right pulmonary artery. PDA, Patent ductus arteriosus. VSD, Ventricular septal defect. 'From Pool, P. E., et al.: Am. J. Cardiol. 10:706, 1962, published by Dun-Donnelley Publishing Corporation, New
York, N. Y.
monary trunk and the left pulmonary artery. No detailed information on the operation was given. Subsequently, Kawada'" in 1971 performed a successful two-stage operation in a case of atypical tetralogy with anomalous left pulmonary artery from the aortic arch and absence of the pulmonary valve. In 1972 Morgan'" successfully performed a
56
Imai et al.
The Journal of Thorocic ond Cardiovascular Surgery
Fig. 9. Postoperative right heart angiograms. Arrow indicates the porcine monocusp. Two solid lines indicate the extent of the outflow patch. Note normal opacification of the left pulmonary artery (LPA). RPA, Right pulmonary artery. RV, Right ventricle.
Fig. 10. Postoperative chest x-ray film shows normal pulmonary vascularity with a slight increase in cardiac size.
single-stage correction of tetralogy associated with an anomalous left pulmonary artery from the ascending aorta. Czarnecki" in 1964 reported a similar case of tetralogy in which the left pulmonary artery originated
from the distal portion of the aortic arch. However, failure to recognize this anomaly at operation resulted in the patient's death. Although total correction was successful in the 3 reported cases of this anomaly associated with tetralogy, direct anastomosis between the pulmonary trunk and the anomalous pulmonary artery was feasible in all. Therefore, our patient seems to be the first to have required the method of repair that we devised-that is, anastomosis between the left pulmonary artery and the prosthetic graft as well as reconstruction of the outflow tract with a patch retaining a porcine monocusp. Our method of reconstruction would have a wider application for those cases in which the anomalous artery has a stenotic segment at or near its origin. Controversies still exist regarding the significance of pulmonary regurgitation on right ventricular function. It is our impression from clinical experience that pulmonic regurgitation plays an important role in causing right ventricular failure in the immediate postoperative
Volume 68 Number 1 July, 1974
Tetralogy of Fallot
i
57
'1
I , j ._
1·1
..;.Ji...........
y' I ' I. I
I
I
!
....c..J...L.L 1
I
-. I
IT
ill ~I
I
.1
Vs Fig. 11. Postoperative electrocardiogram (April 3, 1972).
Table III. Unilateral anomalous pulmonary artery Author
Year
Side
Associated anomaly
Armer et al .> Kirklin et al.! Redo et a\.5 Weintraub et a\.6 Kirkpatrick et a1. 7 Green et a\.s Kuypers et a1. 9 Caudill et al.r° Flege et al. l l Takeuchi et a\.12 Kawada et al. 13 Hosokawa et al. 14 Schiller et al.I 5 Morgan-s Imai et a\.
1961 1965 1965 1966 1967 1968 1969 1969 1970 1970 1971 1971 1971 1972 1974
RPA-AA LPA-arch RPA-AA LPA-AA RPA-AA LPA-arch RPA-AA LPA-AA RPA-AA RPA-AA LPA-arch RPA-AA LPA-AA LPA-AA LPA-arch
Patent ductus Tetralogy Patent ductus None Patent ductus None Patent ductus None Ventricular septal defect Patent ductus Tetralogy Patent ductus None Tetralogy Tetralogy
Legend: RP A-AA, Origin of right pulmonary artery from ascending aorta. LP A·AA, Origin of left pulmonary artery from ascending aorta. LP A-arch, Origin of left pulmonary artery from aortic arch.
period, even though it has little significance in a normal heart. The fate of the porcine valve is still in doubt. We have followed this material for only 3 years. The tissue was in good functioning condition at that time, but a longer period of observation is necessary.
4
5
REFERENCES Cucci , C. E., Doyle, E. F., and Lewis, E. W., Jr .: Absence of a Primary Division of the Pulmonary Trunk, Circulation 29: 124, 1964. 2 Pool, P. E., Vogel, J. H. K., and Blount, G. S., Jr.: Congenital Unilateral Absence of a Pulmonary Artery, Am. 1. Cardio\. 10: 706, 1962. 3 Armer, R. M., Shumacker, H. B, and Klatte,
6
7
E. C.: Origin of the Right Pulmonary Artery From the Ascending Aorta, Circulation 24: 662, 1961. Kirklin, J. W., Wallace, R. B., McGoon, D. C., and DuShane, J. W.: Early and Late Results After Intracardiac Repair of Tetralogy of Fallot, Ann . Surg. 162: 578, 1965. Redo , S. F. , Foster, H. R., Engle, M. A., and Ehlers, K. H.: Anomalous Origin of the Right Pulmonary Artery From the Ascending Aorta, J. THORAC. CARDIOVASC. SURG. SO: 726, 1965. Weintraub, R. A, Fabian, C. E., and Adams, D. F.: Ectopic Origin of One Pulmonary Artery From the Ascending Aorta, Radiology 86: 666, 1966. Kirkpatrick, S. E., Girod, D. A., and King, H.: Aortic Orig in of the Right Pulmonary Artery, Circulation 36: 777, 1967.
The Journal of
5 8 Imai et at.
Thoracic and Cordiovasculor
Surgery
8 Green, G. E., Reppert, E. H., Cohlan, S. Q., and Spencer, F. C.: Surgical Correction of Absence of Proximal Segment of Left Pulmonary Artery, Circulation 37: 62, 1968. 9 Kuypers, P. J., van der Maas, A. H., and Busch, H. J.: Origin of the Right Pulmonary Artery From the Aorta With Patent Ductus Arteriosus, 1. THoRAe. CARDIOVASC. SURG. 57: 185, 1969. 10 Caudill, D. R., Helmsworth, J. A., Daoud, G., and Kaplan, S.: Anomalous Origin of Left Pulmonary Artery From Ascending Aorta, J. THORAC. CARDIOVASC. SURG. 57: 493, 1969. 11 Flege, 1. B., Durnin, R. E., and Rossi, N. P.: Aortic Origin of the Right Pulmonary Artery and Ventricular Septal Defect, J. THORAC. CARDIOVASC. SURG. 59: 468, 1970. 12 Takeuchi, N., Fukuda, T., Tsuji, A., and Shozu, A.: Successful Surgical Correction of a Case With the Right Pulmonary Artery Originating From Ascending Aorta and Patent Ductus Arteriosus, Shinzo 2: 737, 1970 (in Japanese). 13 Kawada, S., Nagamine, I., Nishikawa, K., Kawada, K., Inoue, T., Hata, J., Kojima, E., Ishiwatari, K., and Kamimura, H.: Successful
14
15
16
17
Surgical Correction of a Case of Tetralogy of Fallot Associated With an Anomalous Left Pulmonary Artery Originating From Aortic Arch and Absence of the Pulmonary Valve, Shinzo 3: 1073, 1971 (in Japanese). Hosokawa, Y., Okamura, H., Nakamura, T., Sagara, T., Matsuoka, T., Kamishimo, J., Kobayashi, H., Endo, T., Matsumoto, H., Kabe, T., Saito, M., and Soda, T.: Successful Surgical Correction of a Case With Right Pulmonary Artery Originating From Ascending Aorta and Patent Ductus Arteriosus Using a Simple Deep Hypothermia Technique, Shinzo 5: 126 1971 (in Japanese). Schiller, M., Williams, T. E., Craenen, J., Hosier, D. M., and Sirak, H. D.: Anomalous Origin of Left Pulmonary Artery From the Ascending Aorta, Vase. Surg. 5: 126, 1971. Morgan, J. R.: Left Pulmonary Artery From Ascending Aorta in Tetralogy of Fallot, Circulation 45: 653, 1972. Czarnecki, S. W., Hopeman, A. R., and Child, P. L.: Tetralogy of Fallot With Aortic Origin of the Left Pulmonary Artery, Chest 46: 97, 1964.