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FALLOT’S ANOMALY WITH PERIPHERAL PULMONARY ARTERY MALFORMATIONS
FALLOT'S A N O M A L Y W I T H PERIPHERAL P U L M O N A R Y ARTERY
MALFORMATIONS
V. 0. Bjork, M.D., H. Lodin, M.D., and M. Michaelsson, Uppsala,
M.D.,
Sweden
T
HERE is difference of opinion as to what constitutes Fallot's tetralogy. The term has been used, for example, in cases of ventricular septal defect combined with pulmonary stenosis with normal arterial oxygen saturation. By using the term Fallot's anomaly instead of Fallot's tetralogy we have accepted the recommendation given by Harley. 2 He defines Fallot's anomaly as pulmonary stenosis with reversed or bidirectional shunt through a nonrestrictive ventricular septal defect. Hypertrophy of the right ventricle is excluded because "it is no developmental defect." Dextroposition of the aorta is considered to be "functionally unimportant and extremely variable in degree anatomically. An eponymous title is still used because of its brevity and because Fallot described the condition so well, even though he was not the first to do so."2 In Fallot's anomaly the pulmonary artery and its main branches usually show varying degrees of hypoplasia which is classically symmetric and probably roughly proportionate to the stenosis of the outflow tract. 6 In the most severe stenosis, the whole pulmonary artery tree is atretic (functionally or anatomically). This condition which is hemodynamically quite different from Fallot's anomaly is better classified under a separate heading. The purpose of the present paper is to report 6 cases of Fallot's anomaly and localized peripheral pulmonary artery malformations without persistent ductus or any other aorticopulmonary communication. Several cases have been reported in which Fallot's anomaly was associated with atresia of one of the main pulmonary arteries. 1 ' 6 - 7 This really important combined malformation seems to be less well known as is also the case with the other pulmonary artery malformations in Fallot's anomaly described below. It is then of special interest to note that the following 6 cases constitute a large part of the children with Fallot's anomaly from our hospital, 26 cases in all. CASE REPORTS
(See also Table I)
CASE 1.—A 13-year-old boy, with cyanosis since birth, had clubbing of the ringers and toes and was subject to frequent anoxic spells. Eoentgenographic examination showed a norProm the Department of Thoracic Surgery (Head: V. O. Bjork, M.D.), the Department of Roentg-enolosy (Head: P. Knutsson, M.D.), and the Department of Pediatrics (Head: B. Vahlquist, M.D.), University Hospital, Uppsala, Sweden. Received for publication Dec. 26, 1962. 764
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mal heart volume, a shift of the heart to the left, and a prominence of the outflow tract of the right ventricle. The left lung was hypoplastic with reduced vascular structure, the mediastinum was shifted to the left (Figs. 1A and I B ) , and the right lung was distended with normal vascular structures. Angiocardiography from the right ventricle showed a ventricular septal defect, an overriding of the aorta, a narrow infundibular stricture, and a complete absence of the left pulmonary artery (Figs. 2A and 2B). The patient had also a small left superior vena cava which entered the coronary sinus. The ventricular septal defect was closed by direct suture, the infundibular stricture was resected, and one fused commissure in the pulmonary valves was opened 5 mm. A prosthesis of pericardium was sutured into the outflow tract of the right ventricle. After 77 minutes of perfusion and 27 minutes of anoxic arrest the heart started in sinus rhythm. The pressure in the right ventricle was 55/10 mm. H g and that of the femoral artery 110/80 mm. H g at the end of the operation. When the chest was closed a significant amount of air from a blood transfusion apparatus entered the heart, which resulted in cardiac standstill. The air was evacuated by several large-bore needles in the right ventricle, the right atrium, and the pulmonary artery. The myocardium became very edematous. After massage, the heart started, a complete atrioventricular block was noted, and pacemaker treatment was instituted. The wound was closed, a tracheostomy performed and respirator treatment was instituted. The pacemaker was used for 38 days after which time the heart had a constant sinus rhythm. The respirator treatment was used for 20 days. After that time the parenchymatous changes in the right lung had disappeared. These changes were interpreted as pulmonary edema due to the lesion caused by air entering the pulmonary artery. After 3 weeks the patient could perform the respiratory work himself. He received nutrition for 3 weeks through a catheter placed in the superior vena cava. Ten months after operation the patient was re-evaluated. The cerebral function was normal, working capacity was considerably better than before operation, and no cyanosis was observed. The hemoglobin and the hematocrit were normal. The heart was moderately enlarged, the volume was 640 ml. per square meter of body surface. The electrocardiogram showed the usual postoperative picture with complete right bundle branch block and pathological deep T waves over the right precordium. H e a r t catheterization showed a pressure of 60 mm. H g in the right ventricle and no signs of left-to-right shunt. Angiocardiography from the right ventricle showed an enlarged ventricle with an aneurysm-like bulging of the outflow tract (Fig. 3 ) . Two years after operation the patient is still in a very good clinical condition. CASE 2.—The patient was an 8-year-old boy whose father had Fallot's anomaly. Cyanosis and a systolic murmur had been observed since birth and the patient had had repeated anoxic spells during the first months. At 9 months of age, a right heart catheterization was performed. The right ventricular pressure was 100 mm. H g . The angiocardiogram from the right ventricle showed a ventricular septal defect, a pulmonary valvular stenosis, a narrow pulmonary artery, and a stricture in the distal part of the pulmonary artery. A left-sided Blalock shunt was made at 10 months of age. At 7 years of age the patient was extremely cyanotic with respiratory distress at rest (see Table I ) . A new angiocardiogram showed that the Blalock shunt was functioning. At operation the Blalock anastomosis was first dissected and a ligature was placed around it but not tied until the perfusion was started. Perfusion lasted for 65 minutes and anoxic arrest for 26 minutes at a temperature of 31.2° C. The right ventricular outflow tract was opened all the way out through the valvular ring to the pulmonary artery. There was a very tight valvular stenosis. The ventricular septal defect was closed by two rows of direct sutures, the second row reinforced by a layer of pericardium. An outflow prosthesis of pericardium was sutured in place all the way through the right ventricular outflow tract, through the valvular ring and 1 cm. out on the pulmonary artery. The heart took over circulation in block and a pacemaker was applied.
766
BJORK, LODIN, M I C H A E L S S O N
J. Thoracic and Cardiovas. Surg.
Fig. 1A.—Case 1. Frontal chest roentgenogram. The heart is of normal size but displaced to the left. There is reduced vascular pattern in the left lung.
Fig. IB.—Case 1. Transversal tomogram shows displacement of the mediastinum, including the heart, to the left (-») border between the right and the left hypoplastic lungs.
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Fig. 2A.—Case 1. Frontal angiocardiogram shows narrow infundibular stenosis (-►). complete absence of the left pulmonary artery, wide right pulmonary artery, ventricular septal defect, and overriding aorta.
left pulm.art. V | r_Jl—-Valvular stenosis V— Infundibular stenosis VSD
Fig. IB.—Case 1. Diagram of the operative findings shows atresia of the left pulmonary artery.
Pig. 3.—Case 1. Postoperative frontal angiocardiogram. No ventricular septal defect is visible. The outflow of the right ventricle bulges to the left a t the place of the prosthesis of pericardium (<-).
The systolic pressure in the right ventricle decreased from 95 to 80 mm. Hg, at the same time it decreased in the aorta from 95 to 85 mm. Hg. The pressure in the pulmonary artery rose from 25 to 79 mm. Hg. Thus a gradient over the ostium was abolished but still high pressure remained in the right ventricle. After 4 days, sinus rhythm returned and the recovery was then uneventful. Since the operation there has been a slow but significant improvement. CASE 3.—A 6-year-old girl with cyanosis had been observed since one month of age. She had clubbing of the fingers and toes, shortness of breath, and accentuated cyanosis appeared on mild exertion. The calculated heart volume was normal and the vascular structure of the lungs was apparently normal. Cardiac catheterization showed a pressure of 110 mm. H g in the right ventricle and 90 mm. H g in the aorta. (At 22 months of age the right ventricular pressure was 93 mm. Hg and a large left-to-right shunt was found.) The angiocardiogram from the right ventricle showed a large, high ventricular septal defect, an overriding of the aorta, pulmonary stenosis, and a narrowing of the main pulmonary artery (Figs. 4, A and B). These anatomical findings were confirmed at operation (Fig. 5 ) . The incision was made from the right ventricle through the pulmonary ring and 25 mm. along the narrow pulmonary artery. A direct suture of the ventricular septal defect was made and an outflow prosthesis (from the pericardium of the patient) was sutured into the incision. The perfusion time was 68 minutes; the anoxic arrest was 19 minutes at a temperature of 29.5° C. The right ventricular pressure was 60/10 mm. Hg, the femoral artery pressure 80/40 mm. Hg, and the pulmonary artery pressure 28/8 mm. H g . A 2 : 1 block developed and the pacemaker was used. There were no signs of
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Fie. 4.—Case 3. Frontal (A) and lateral (B) angiocardiograms show infundibular stenosis (-»), valvular pulmonary stenosis, and a narrowing of the main pulmonary artery (|-»). From the upper part of the main pulmonary artery, close to the bifurcation, there is an aneurysm, a residual of the ductus (||->). The high ventricular septal defect (—») and the overriding aorta are also demonstrated.
770
J. Thoracic and Cardiovas. Surg.
B.ToEK, LODIN, M I C H A E L S S O N
Lig. arteriosum Aneurysm Main pulm. artery stenosis Valvular stenosis Infundibular stenosis VSD
pig. 5.—Diagram of the operative findings in Case 3 shows a main pulmonary artery stenosis. cerebral dysfunction.
The child died after 15 hours from hypotension.
Autopsy did not give
an explanation of the cause of her death. CASE 4.—The patient, an 8-year-old boy, was highly cyanotic with clubbing of the fingers and toes. The heart volume was normal. The roentgenographie examination, including transverse tomogram, showed that the left lung was hypoplastic with a dislocation of the mediastinum to the left. The vascular structure was diminished in the left Jung and normal on the right side. Angiocardiography from the right ventricle showed a ventricular septal defect, a tight infundibular stenosis, a valvular pulmonary stenosis, a main pulmonary artery stenosis, and very tight stricture of the left main pulmonary artery (Figs. 6, A and B). At operation the incision in the right ventricle was carried through the valvular ring and 2 cm. out on the pulmonary artery. The ventricular septal defect was closed by direct suture. The infundibular stricture was resected and an outflow prosthesis of pericardium sutured into the whole incision. The left pulmonary artery was dissected and found to be 5 mm. in diameter. An internal stricture at its origin was not seen from the outside and therefore overlooked at the operation (Fig. 7 ) . After 57 minutes of perfusion and 23 minutes of anoxic arrest, the heart started in sinus rhythm. Another period of 10 minutes of perfusion and 3 minutes of arrest allowed closure of a small atrial septal defect. By an accident the patient was not ventilated for about 5 minutes. The esophageal temperature was then 32.2" C. The pressure at the end of the operation was 65/5 mm. H g in the right ventricle and 80/25 mm. H g in the brachial artery. Pacemaker treatment was instituted because of heart block. The patient died after 36 hours with cerebral symptoms. Autopsy showed a good correction of the defects except the left pulmonary artery stricture which had been overlooked. Brain damage of anoxic type was found. Comments: The death of this patient may be ascribed to the accidental anoxic brain damage. The right ventricular pressure was reduced only to 65/8 mm. H g which could probably be explained to a great extent by the remaining stricture of the left pulmonary artery. CASE 5.—The patient was a 5%-year-old girl whose mother had had German measles in the fourth month of the pregnancy. Cyanosis and respiratory distress had been observed
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Pig-. 6.—Case 4. Frontal (A) and lateral (B) angiocardiograms show a ventricular septal defect, an infundibular stenosis, a valvular pulmonary stenosis, a tight stricture of the main pulmonary artery (-»), and the hypoplastic left pulmonary artery (—»).
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J. Thoracic and Cardiovas. Surg.
since the first days of life. She had bilateral cataracts which were removed when she was 1 month of age. Squatting, dyspnea on mild exertion, and increasing cyanosis were evident from the time the patient was 1 year of age. Physical examination revealed a mentally and physically poorly developed child with extreme cyanosis and clubbing of the fingers and toes. She had signs of mild cerebral palsy with ataxia, spasticity of the legs, and hypotonicity of arms and legs. Angiocardiography from the right ventricle showed a ventricular septal defect, a tight infundibular stenosis and an overriding aorta. The main pulmonary artery was not clearly visible but the peripheral part of the pulmonary vascular tree was normal.
Small left pulm.art. STenosis to left pulm.art. Main pulm. artery stenosis Valvular stenosis Infundibular stenosis
Fig:. 7.—Diagram of the operative findings in Case 4 shows the very small left pulmonary artery and stenosis of the main pulmonary artery.
At the incision in the right ventricle, a big branch from the left coronary artery, which supplied most of the right ventricle with blood, was divided where it came within the myocardium. The right outflow channel was divided all the way out in the pulmonary artery. The hole in the pulmonary valvular dome could not be found. The pulmonary artery was narrow and divided all the way out to its bifurcation between the left and right pulmonary artery. A Teflon prosthesis was sutured into the ventricular septal defect. A Teflon prosthesis was also sutured all the way through the right ventricular outflow tract out to the bifurcation of the pulmonary artery. The right ventricular pressure was decreased to 35 mm. H g ; at the same time it was 61 mm. H g in the brachial artery. The patient went into right ventricular failure after the operation and there was cardiac arrest 12 days after operation. She was Resuscitated by thoracotomy; respirator treatment was applied but the patient died on the sixteenth postoperative day in circulatory insufficiency. CASE 6.—This was a 7-month-old boy with cyanosis and a heart murmur which had been observed since birth. Angiocardiography from the right ventricle showed ventricular septal defect, a right-sided aortic arch, an overriding aorta, an infundibular stenosis, no visible
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TABLE
CASE NO.
AGE (YEARS)
WEIGHT (KG.)
1 2 3 4 5
13 8 6 8 5
31.4 17.9 16.0 17.3 13.6
DEGREE OF CYANOSIS
(+, ++, +++) +++ +++
+++ 4+
+++
773
I
(GM./100
HEMATOCEIT
PRESSURE I N RIGHT VENTRICLE (MM. H g )
21.6 20.3 20.3 17.9 23.2
67 68 60 58 75
95 80 110 100 95
HEMOGLOBIN ML.)
(%)
OXYGEN SATURATION IN THE AORTA
(%) 68 65 63
— 60
pulmonary valves, a narrow main pulmonary artery, and no visible left pulmonary artery. The patient died at home 1 year later. No postmortem examination was made. DISCUSSION
The physical findings of the heart were of no aid in the diagnosis of the additional anomaly. No diastolie murmur was heard in any instance except Case 2 who had an open Blalock shunt. The diagnosis can only be made by angiocardiographic investigation. We have accepted all children over the age of 2 years for total correction of the anomaly of Pallot; the smallest child successfully operated upon weighed 9.6 kilograms. In our group of patients with the ordinary anomaly of Fallot there were 16 patients operated upon with 14 survivals. However, in the group with Fallot's anomaly with peripheral stenosis of the pulmonary artery just reported, we have operated upon 5 patients with 2 survivors. This demonstrates the increased risk for these patients. However, as the natural history is so bad in these complicated malformations and the 2 survivors have shown such encouraging results we do think it is advisable not to refuse a corrective procedure. When the pulmonary artery is big enough for a Blalock anastomosis, a total correction is also technically possible. Naturally it is more difficult to reduce the pressure in the right ventricle when there is peripheral pulmonary artery stenosis, although a prosthesis is sutured through the outflow tract over the valvular area and out to the bifurcation of the pulmonary artery. We do not believe there is any place for shunt operations in cases of peripheral pulmonary artery stenosis. In one of the surviving patients, there was a Blalock anastomosis functioning when reoperation was performed. The infundibular resection is easiest performed through a right ventricular incision. However, it is possible to do an infundibular resection through the right atrium with the heart under local hypothermic arrest. We have carried out such operations in 3 cases. If, however, it is necessary to utilize an outflow prosthesis the approach should be from the right ventricle. Regarding the material for outflow prosthesis, we have given up the use of Ivalon after Kirklin reported at least 3 eases of false aneurysm in the outflow tract following rupture of the suture line on the myocardial side. We have seen 2 cases of aneurysmal dilatation in the outflow area after the application of a pericardial prosthesis and are, therefore, at present using Teflon. The question is naturally when should an outflow prosthesis be carried all the way out into the pulmonary artery. We have recently tried to make an infundibular resection
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BJORK, LODIN, MICHABLSSON
J. Thoracic and Cardiovas. Surg.
only to obtain a pulsatile pressure in the pulmonary artery of between 30 and 40 mm. Hg systolic, regardless of the remaining right ventricular pressure. We do not then apply an outflow prosthesis. All ventricular septal defects of more than 1 cm. in diameter are closed with the aid of a Teflon prosthesis. During the closure of the ventricular septal defect, we use arrest of the heart by packing in ice water. The postoperative cardiac insufficiency has been less severe and of shorter duration in the 3 patients who had a complete correction from the right atrium and a separate incision for the pulmonary valvulotomy. SUMMARY
Six patients are described with Fallot's anomaly who had peripheral pulmonary artery malformations as well. Five patients were operated upon and two survived. The good result at follow-up in a patient who had had left main pulmonary atresia has encouraged us to use corrective operations in similar cases. REFERENCES 1. Blalock, A.: Surgical Procedure Employed and Anatomical Variation Encountered in the Treatment of Congenital Pulmonic Stenosis, Surg. Gynec. & Obst. 87: 385, 1948. 2. Harley, H. R. S.: W h a t is Fallot's Tetralogy? Am. H e a r t J . 62: 729, 1961. 3. Kirklin, J. W., and Spencer, W. P . : Surgical Treatment for Tetralogy of Fallot After Previous Anastomosis of Systemic to Pulmonary Arterv, Surg. Gynec. & Obst. 110: 707, 1960. 4. Kirklin, J . W., and Devloo, R. A.: Hypothermic Perfusion and Circulatory Arrest for Surgical Correction of Tetralogy of Fallot With Previously Constructed Potts' Anastomosis, Dis. Chest. 39: 87. 1961. 5. Lillehei, C. W., Cohen, M., Warden, H. E., Read, R. C , Aust, J. P.., DeWall, R. A., and Varco R. L.: Direct Vision I n t r a c a r d i a c Surgical Correction of the Tetralogy of Fallot, Pentalogy of Fallot and Pulmonary Atresia Defects. Report of First Ten Cases, Ann. Surg. 142: 418, 1955. 6. Nadas, A. S., Rosenbaum, H. D., Wittenborg, M. H., and Rudolph, A. M.: Tetralogy of Fallot With Unilateral Pulmonary Atresia. A Clinically Diagnosable and Surgically Significant Variant, Circulation 8: 328, 1953. 7. Thomas, H. W.: Congenital Cardiac Malformations, J . Tech. Meth. 21: 58, 1941.
MALFORMATIONS
V. 0. Bjork, M.D., H. Lodin, M.D., and M. Michaelsson, Uppsala,
M.D.,
Sweden
T
HERE is difference of opinion as to what constitutes Fallot's tetralogy. The term has been used, for example, in cases of ventricular septal defect combined with pulmonary stenosis with normal arterial oxygen saturation. By using the term Fallot's anomaly instead of Fallot's tetralogy we have accepted the recommendation given by Harley. 2 He defines Fallot's anomaly as pulmonary stenosis with reversed or bidirectional shunt through a nonrestrictive ventricular septal defect. Hypertrophy of the right ventricle is excluded because "it is no developmental defect." Dextroposition of the aorta is considered to be "functionally unimportant and extremely variable in degree anatomically. An eponymous title is still used because of its brevity and because Fallot described the condition so well, even though he was not the first to do so."2 In Fallot's anomaly the pulmonary artery and its main branches usually show varying degrees of hypoplasia which is classically symmetric and probably roughly proportionate to the stenosis of the outflow tract. 6 In the most severe stenosis, the whole pulmonary artery tree is atretic (functionally or anatomically). This condition which is hemodynamically quite different from Fallot's anomaly is better classified under a separate heading. The purpose of the present paper is to report 6 cases of Fallot's anomaly and localized peripheral pulmonary artery malformations without persistent ductus or any other aorticopulmonary communication. Several cases have been reported in which Fallot's anomaly was associated with atresia of one of the main pulmonary arteries. 1 ' 6 - 7 This really important combined malformation seems to be less well known as is also the case with the other pulmonary artery malformations in Fallot's anomaly described below. It is then of special interest to note that the following 6 cases constitute a large part of the children with Fallot's anomaly from our hospital, 26 cases in all. CASE REPORTS
(See also Table I)
CASE 1.—A 13-year-old boy, with cyanosis since birth, had clubbing of the ringers and toes and was subject to frequent anoxic spells. Eoentgenographic examination showed a norProm the Department of Thoracic Surgery (Head: V. O. Bjork, M.D.), the Department of Roentg-enolosy (Head: P. Knutsson, M.D.), and the Department of Pediatrics (Head: B. Vahlquist, M.D.), University Hospital, Uppsala, Sweden. Received for publication Dec. 26, 1962. 764
Vol. 45, No. 6 June, 1963
F A L L O T ' S ANOMALY
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mal heart volume, a shift of the heart to the left, and a prominence of the outflow tract of the right ventricle. The left lung was hypoplastic with reduced vascular structure, the mediastinum was shifted to the left (Figs. 1A and I B ) , and the right lung was distended with normal vascular structures. Angiocardiography from the right ventricle showed a ventricular septal defect, an overriding of the aorta, a narrow infundibular stricture, and a complete absence of the left pulmonary artery (Figs. 2A and 2B). The patient had also a small left superior vena cava which entered the coronary sinus. The ventricular septal defect was closed by direct suture, the infundibular stricture was resected, and one fused commissure in the pulmonary valves was opened 5 mm. A prosthesis of pericardium was sutured into the outflow tract of the right ventricle. After 77 minutes of perfusion and 27 minutes of anoxic arrest the heart started in sinus rhythm. The pressure in the right ventricle was 55/10 mm. H g and that of the femoral artery 110/80 mm. H g at the end of the operation. When the chest was closed a significant amount of air from a blood transfusion apparatus entered the heart, which resulted in cardiac standstill. The air was evacuated by several large-bore needles in the right ventricle, the right atrium, and the pulmonary artery. The myocardium became very edematous. After massage, the heart started, a complete atrioventricular block was noted, and pacemaker treatment was instituted. The wound was closed, a tracheostomy performed and respirator treatment was instituted. The pacemaker was used for 38 days after which time the heart had a constant sinus rhythm. The respirator treatment was used for 20 days. After that time the parenchymatous changes in the right lung had disappeared. These changes were interpreted as pulmonary edema due to the lesion caused by air entering the pulmonary artery. After 3 weeks the patient could perform the respiratory work himself. He received nutrition for 3 weeks through a catheter placed in the superior vena cava. Ten months after operation the patient was re-evaluated. The cerebral function was normal, working capacity was considerably better than before operation, and no cyanosis was observed. The hemoglobin and the hematocrit were normal. The heart was moderately enlarged, the volume was 640 ml. per square meter of body surface. The electrocardiogram showed the usual postoperative picture with complete right bundle branch block and pathological deep T waves over the right precordium. H e a r t catheterization showed a pressure of 60 mm. H g in the right ventricle and no signs of left-to-right shunt. Angiocardiography from the right ventricle showed an enlarged ventricle with an aneurysm-like bulging of the outflow tract (Fig. 3 ) . Two years after operation the patient is still in a very good clinical condition. CASE 2.—The patient was an 8-year-old boy whose father had Fallot's anomaly. Cyanosis and a systolic murmur had been observed since birth and the patient had had repeated anoxic spells during the first months. At 9 months of age, a right heart catheterization was performed. The right ventricular pressure was 100 mm. H g . The angiocardiogram from the right ventricle showed a ventricular septal defect, a pulmonary valvular stenosis, a narrow pulmonary artery, and a stricture in the distal part of the pulmonary artery. A left-sided Blalock shunt was made at 10 months of age. At 7 years of age the patient was extremely cyanotic with respiratory distress at rest (see Table I ) . A new angiocardiogram showed that the Blalock shunt was functioning. At operation the Blalock anastomosis was first dissected and a ligature was placed around it but not tied until the perfusion was started. Perfusion lasted for 65 minutes and anoxic arrest for 26 minutes at a temperature of 31.2° C. The right ventricular outflow tract was opened all the way out through the valvular ring to the pulmonary artery. There was a very tight valvular stenosis. The ventricular septal defect was closed by two rows of direct sutures, the second row reinforced by a layer of pericardium. An outflow prosthesis of pericardium was sutured in place all the way through the right ventricular outflow tract, through the valvular ring and 1 cm. out on the pulmonary artery. The heart took over circulation in block and a pacemaker was applied.
766
BJORK, LODIN, M I C H A E L S S O N
J. Thoracic and Cardiovas. Surg.
Fig. 1A.—Case 1. Frontal chest roentgenogram. The heart is of normal size but displaced to the left. There is reduced vascular pattern in the left lung.
Fig. IB.—Case 1. Transversal tomogram shows displacement of the mediastinum, including the heart, to the left (-») border between the right and the left hypoplastic lungs.
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Fig. 2A.—Case 1. Frontal angiocardiogram shows narrow infundibular stenosis (-►). complete absence of the left pulmonary artery, wide right pulmonary artery, ventricular septal defect, and overriding aorta.
left pulm.art. V | r_Jl—-Valvular stenosis V— Infundibular stenosis VSD
Fig. IB.—Case 1. Diagram of the operative findings shows atresia of the left pulmonary artery.
Pig. 3.—Case 1. Postoperative frontal angiocardiogram. No ventricular septal defect is visible. The outflow of the right ventricle bulges to the left a t the place of the prosthesis of pericardium (<-).
The systolic pressure in the right ventricle decreased from 95 to 80 mm. Hg, at the same time it decreased in the aorta from 95 to 85 mm. Hg. The pressure in the pulmonary artery rose from 25 to 79 mm. Hg. Thus a gradient over the ostium was abolished but still high pressure remained in the right ventricle. After 4 days, sinus rhythm returned and the recovery was then uneventful. Since the operation there has been a slow but significant improvement. CASE 3.—A 6-year-old girl with cyanosis had been observed since one month of age. She had clubbing of the fingers and toes, shortness of breath, and accentuated cyanosis appeared on mild exertion. The calculated heart volume was normal and the vascular structure of the lungs was apparently normal. Cardiac catheterization showed a pressure of 110 mm. H g in the right ventricle and 90 mm. H g in the aorta. (At 22 months of age the right ventricular pressure was 93 mm. Hg and a large left-to-right shunt was found.) The angiocardiogram from the right ventricle showed a large, high ventricular septal defect, an overriding of the aorta, pulmonary stenosis, and a narrowing of the main pulmonary artery (Figs. 4, A and B). These anatomical findings were confirmed at operation (Fig. 5 ) . The incision was made from the right ventricle through the pulmonary ring and 25 mm. along the narrow pulmonary artery. A direct suture of the ventricular septal defect was made and an outflow prosthesis (from the pericardium of the patient) was sutured into the incision. The perfusion time was 68 minutes; the anoxic arrest was 19 minutes at a temperature of 29.5° C. The right ventricular pressure was 60/10 mm. Hg, the femoral artery pressure 80/40 mm. Hg, and the pulmonary artery pressure 28/8 mm. H g . A 2 : 1 block developed and the pacemaker was used. There were no signs of
Vol. 45, No. 6 June, 1963
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Fie. 4.—Case 3. Frontal (A) and lateral (B) angiocardiograms show infundibular stenosis (-»), valvular pulmonary stenosis, and a narrowing of the main pulmonary artery (|-»). From the upper part of the main pulmonary artery, close to the bifurcation, there is an aneurysm, a residual of the ductus (||->). The high ventricular septal defect (—») and the overriding aorta are also demonstrated.
770
J. Thoracic and Cardiovas. Surg.
B.ToEK, LODIN, M I C H A E L S S O N
Lig. arteriosum Aneurysm Main pulm. artery stenosis Valvular stenosis Infundibular stenosis VSD
pig. 5.—Diagram of the operative findings in Case 3 shows a main pulmonary artery stenosis. cerebral dysfunction.
The child died after 15 hours from hypotension.
Autopsy did not give
an explanation of the cause of her death. CASE 4.—The patient, an 8-year-old boy, was highly cyanotic with clubbing of the fingers and toes. The heart volume was normal. The roentgenographie examination, including transverse tomogram, showed that the left lung was hypoplastic with a dislocation of the mediastinum to the left. The vascular structure was diminished in the left Jung and normal on the right side. Angiocardiography from the right ventricle showed a ventricular septal defect, a tight infundibular stenosis, a valvular pulmonary stenosis, a main pulmonary artery stenosis, and very tight stricture of the left main pulmonary artery (Figs. 6, A and B). At operation the incision in the right ventricle was carried through the valvular ring and 2 cm. out on the pulmonary artery. The ventricular septal defect was closed by direct suture. The infundibular stricture was resected and an outflow prosthesis of pericardium sutured into the whole incision. The left pulmonary artery was dissected and found to be 5 mm. in diameter. An internal stricture at its origin was not seen from the outside and therefore overlooked at the operation (Fig. 7 ) . After 57 minutes of perfusion and 23 minutes of anoxic arrest, the heart started in sinus rhythm. Another period of 10 minutes of perfusion and 3 minutes of arrest allowed closure of a small atrial septal defect. By an accident the patient was not ventilated for about 5 minutes. The esophageal temperature was then 32.2" C. The pressure at the end of the operation was 65/5 mm. H g in the right ventricle and 80/25 mm. H g in the brachial artery. Pacemaker treatment was instituted because of heart block. The patient died after 36 hours with cerebral symptoms. Autopsy showed a good correction of the defects except the left pulmonary artery stricture which had been overlooked. Brain damage of anoxic type was found. Comments: The death of this patient may be ascribed to the accidental anoxic brain damage. The right ventricular pressure was reduced only to 65/8 mm. H g which could probably be explained to a great extent by the remaining stricture of the left pulmonary artery. CASE 5.—The patient was a 5%-year-old girl whose mother had had German measles in the fourth month of the pregnancy. Cyanosis and respiratory distress had been observed
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Pig-. 6.—Case 4. Frontal (A) and lateral (B) angiocardiograms show a ventricular septal defect, an infundibular stenosis, a valvular pulmonary stenosis, a tight stricture of the main pulmonary artery (-»), and the hypoplastic left pulmonary artery (—»).
772
'
BJORK, LODIN, MICHAfiLSSON
J. Thoracic and Cardiovas. Surg.
since the first days of life. She had bilateral cataracts which were removed when she was 1 month of age. Squatting, dyspnea on mild exertion, and increasing cyanosis were evident from the time the patient was 1 year of age. Physical examination revealed a mentally and physically poorly developed child with extreme cyanosis and clubbing of the fingers and toes. She had signs of mild cerebral palsy with ataxia, spasticity of the legs, and hypotonicity of arms and legs. Angiocardiography from the right ventricle showed a ventricular septal defect, a tight infundibular stenosis and an overriding aorta. The main pulmonary artery was not clearly visible but the peripheral part of the pulmonary vascular tree was normal.
Small left pulm.art. STenosis to left pulm.art. Main pulm. artery stenosis Valvular stenosis Infundibular stenosis
Fig:. 7.—Diagram of the operative findings in Case 4 shows the very small left pulmonary artery and stenosis of the main pulmonary artery.
At the incision in the right ventricle, a big branch from the left coronary artery, which supplied most of the right ventricle with blood, was divided where it came within the myocardium. The right outflow channel was divided all the way out in the pulmonary artery. The hole in the pulmonary valvular dome could not be found. The pulmonary artery was narrow and divided all the way out to its bifurcation between the left and right pulmonary artery. A Teflon prosthesis was sutured into the ventricular septal defect. A Teflon prosthesis was also sutured all the way through the right ventricular outflow tract out to the bifurcation of the pulmonary artery. The right ventricular pressure was decreased to 35 mm. H g ; at the same time it was 61 mm. H g in the brachial artery. The patient went into right ventricular failure after the operation and there was cardiac arrest 12 days after operation. She was Resuscitated by thoracotomy; respirator treatment was applied but the patient died on the sixteenth postoperative day in circulatory insufficiency. CASE 6.—This was a 7-month-old boy with cyanosis and a heart murmur which had been observed since birth. Angiocardiography from the right ventricle showed ventricular septal defect, a right-sided aortic arch, an overriding aorta, an infundibular stenosis, no visible
Vol. 45. No. 6
F A L L O T ' S ANOMALY
June, 1963
TABLE
CASE NO.
AGE (YEARS)
WEIGHT (KG.)
1 2 3 4 5
13 8 6 8 5
31.4 17.9 16.0 17.3 13.6
DEGREE OF CYANOSIS
(+, ++, +++) +++ +++
+++ 4+
+++
773
I
(GM./100
HEMATOCEIT
PRESSURE I N RIGHT VENTRICLE (MM. H g )
21.6 20.3 20.3 17.9 23.2
67 68 60 58 75
95 80 110 100 95
HEMOGLOBIN ML.)
(%)
OXYGEN SATURATION IN THE AORTA
(%) 68 65 63
— 60
pulmonary valves, a narrow main pulmonary artery, and no visible left pulmonary artery. The patient died at home 1 year later. No postmortem examination was made. DISCUSSION
The physical findings of the heart were of no aid in the diagnosis of the additional anomaly. No diastolie murmur was heard in any instance except Case 2 who had an open Blalock shunt. The diagnosis can only be made by angiocardiographic investigation. We have accepted all children over the age of 2 years for total correction of the anomaly of Pallot; the smallest child successfully operated upon weighed 9.6 kilograms. In our group of patients with the ordinary anomaly of Fallot there were 16 patients operated upon with 14 survivals. However, in the group with Fallot's anomaly with peripheral stenosis of the pulmonary artery just reported, we have operated upon 5 patients with 2 survivors. This demonstrates the increased risk for these patients. However, as the natural history is so bad in these complicated malformations and the 2 survivors have shown such encouraging results we do think it is advisable not to refuse a corrective procedure. When the pulmonary artery is big enough for a Blalock anastomosis, a total correction is also technically possible. Naturally it is more difficult to reduce the pressure in the right ventricle when there is peripheral pulmonary artery stenosis, although a prosthesis is sutured through the outflow tract over the valvular area and out to the bifurcation of the pulmonary artery. We do not believe there is any place for shunt operations in cases of peripheral pulmonary artery stenosis. In one of the surviving patients, there was a Blalock anastomosis functioning when reoperation was performed. The infundibular resection is easiest performed through a right ventricular incision. However, it is possible to do an infundibular resection through the right atrium with the heart under local hypothermic arrest. We have carried out such operations in 3 cases. If, however, it is necessary to utilize an outflow prosthesis the approach should be from the right ventricle. Regarding the material for outflow prosthesis, we have given up the use of Ivalon after Kirklin reported at least 3 eases of false aneurysm in the outflow tract following rupture of the suture line on the myocardial side. We have seen 2 cases of aneurysmal dilatation in the outflow area after the application of a pericardial prosthesis and are, therefore, at present using Teflon. The question is naturally when should an outflow prosthesis be carried all the way out into the pulmonary artery. We have recently tried to make an infundibular resection
774
BJORK, LODIN, MICHABLSSON
J. Thoracic and Cardiovas. Surg.
only to obtain a pulsatile pressure in the pulmonary artery of between 30 and 40 mm. Hg systolic, regardless of the remaining right ventricular pressure. We do not then apply an outflow prosthesis. All ventricular septal defects of more than 1 cm. in diameter are closed with the aid of a Teflon prosthesis. During the closure of the ventricular septal defect, we use arrest of the heart by packing in ice water. The postoperative cardiac insufficiency has been less severe and of shorter duration in the 3 patients who had a complete correction from the right atrium and a separate incision for the pulmonary valvulotomy. SUMMARY
Six patients are described with Fallot's anomaly who had peripheral pulmonary artery malformations as well. Five patients were operated upon and two survived. The good result at follow-up in a patient who had had left main pulmonary atresia has encouraged us to use corrective operations in similar cases. REFERENCES 1. Blalock, A.: Surgical Procedure Employed and Anatomical Variation Encountered in the Treatment of Congenital Pulmonic Stenosis, Surg. Gynec. & Obst. 87: 385, 1948. 2. Harley, H. R. S.: W h a t is Fallot's Tetralogy? Am. H e a r t J . 62: 729, 1961. 3. Kirklin, J. W., and Spencer, W. P . : Surgical Treatment for Tetralogy of Fallot After Previous Anastomosis of Systemic to Pulmonary Arterv, Surg. Gynec. & Obst. 110: 707, 1960. 4. Kirklin, J . W., and Devloo, R. A.: Hypothermic Perfusion and Circulatory Arrest for Surgical Correction of Tetralogy of Fallot With Previously Constructed Potts' Anastomosis, Dis. Chest. 39: 87. 1961. 5. Lillehei, C. W., Cohen, M., Warden, H. E., Read, R. C , Aust, J. P.., DeWall, R. A., and Varco R. L.: Direct Vision I n t r a c a r d i a c Surgical Correction of the Tetralogy of Fallot, Pentalogy of Fallot and Pulmonary Atresia Defects. Report of First Ten Cases, Ann. Surg. 142: 418, 1955. 6. Nadas, A. S., Rosenbaum, H. D., Wittenborg, M. H., and Rudolph, A. M.: Tetralogy of Fallot With Unilateral Pulmonary Atresia. A Clinically Diagnosable and Surgically Significant Variant, Circulation 8: 328, 1953. 7. Thomas, H. W.: Congenital Cardiac Malformations, J . Tech. Meth. 21: 58, 1941.