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END-TO-END SUPERIOR VENA CAVA-RIGHT PULMONARY ARTERY SHUNT IN TRICUSPID ATRESIA
E N D - T O - E N D SUPERIOR V E N A C A V A - R I G H T P U L M O N A R Y ARTERY S H U N T I N TRICUSPID ATRESIA Ernest H. Meese*
Lieutenant
Commander
(MC)
USN,
Thomas B. Delaney,**
Commander
(MC)
USN, and
Donald J. Doohen,***
Commander
(MC)
USN, St. A/bans, N.
and Joseph ]. Timmes,
M.D.,****
Y.,
Jersey City, N. J.
19491 the procedure of partial right heart bypass has moved from the experimental stage in dogs to clinical usage. This procedure finds its great est application in cardiac defects in which there is diminished pulmonary flow not amenable to complete correction at a later date. Therefore, tricuspid atresia, Ebstein 's anomaly, and complicated defects associated with abdominal heterotaxy present themselves as lesions for which this operation is ideal. Unfortunately, although this is a nice concept, the surgical procedure on patients under one year of age, and especially under 6 months of age, has been attendant with a high mortality. Indeed, Reed and his associates2 recommend that the use of this procedure be discontinued in patients under one year of age. What is more unfortunate is the fact that many babies won't survive to the age of one year to become a candidate for this palliation. This is particularly true of the Type la tricuspid atresia. 3 We believe that our experience with a small number of cases in this age group with the use of end-to-end anastomoses has allowed survival and less mor bidity than the end-to-side method. Assuming that this procedure also has an equivalent or more acceptable mortality than systemic artery shunts, it is pref erable because of the decreased work on the heart and the presentation to the lungs of lower saturated blood at a lower pressure. It is the purpose of this paper to present the cases of 4 patients with tri cuspid atresia on whom an SVC-RPA shunt was performed successfully. Three of the patients are under the age of one year. The 3 patients who had end-to-end
S
INCE
From the U. S. Naval Hospital, St. Albans, N. Y., and Seton Hall College of Medicine, Jersey City, N. J. Opinions expressed herein are those of the authors and do not necessarily reflect the views of the Navy Department of the Naval Service at large. Received for publication June 12, 1963. •Senior Resident, Department of Thoracic and Cardiovascular Surgery, U. S. Naval Hospital, St. Albans, N. Y. *'Cardiologist, Department of Pediatrics, U. S. Naval Hospital, St. Albans, N. Y. **'Chief, Department of Thoracic and Cardiovascular Surgery, U. S. Naval Hospital. St. Albans, N. Y. ••••Professor of Surgery, Seton Hall College of Medicine, Jersey City, N. J. 261
MEESE ET AL.
262
J. Thoracic and Cardiovas. Surg.
anastomoses did significanth- better than the patient in whom the anastomosis was end-to-side. The surgical procedure on the former group will be presented. MATERIAL, AND
METHODS
The 4 patients in this series ranged in age from 2 months to 6 years. All had tricuspid atresia proved by cardiac catheterization and/or selective angio cardiography. Operation was performed on the 3 infants because of severe anoxic difficulties. The 6-year-old child was operated upon because of severe restriction and cyanosis. The right pleural cavity was exposed by a posterolateral thoracotomy in cision through the fourth intercostal space. The right pulmonary artery and superior vena cava were quickly but carefully mobilized from adherent adjacent tissues. The azygos vein was ligated and divided immediately proximal to its entrance into the superior vena cava. This aided in the mobilization of this great vein and provided a greater length to the caval limb of the future anastomosis. The right pulmonary artery was clamped just distal to the bifurcation of the main vessel and divided. The vena cava was similarly clamped and divided immediately proximal to the right atrium. A rapid end-to-end anastomosis was then achieved between the proximal superior vena cava and distal pulmonary artery (Fig. 1, B). Two continuous, over-and-over, 5-0 arterial silk sutures were used for the anterior and posterior rows of this anastomosis. The two sutures were not joined at either end to allow for growth at the anastomotic site. The total time for clamping of the superior vena cava was 10 minutes or less in all instances. After removal of the clamps, the distal right pulmonary artery be came rapidly distended, demonstrating a functionally patent anastomosis. The proximal pulmonary artery and the right atrium were then closed by ligation and sutures, respectively. The operative wound was closed and the pleural cavity was drained by a small Foley catheter attached to underwater seal suction. The average procedure was usually performed within 30 minutes. Total operating time has been shortened in these bad risk patients by avoiding bypass shunts and pressure monitoring. Postoperatively the patients with an end-to-end anastomosis have been kept in a semi-Fowler's position for no longer than 48 hours. The vise of this elevation of the head and trunk is believed not only to aid in respiratory exchange but also to aid gravity in promoting blood flow through the anastomosis during its early hours of function. Blood loss during and following the procedure was replaced by plasma because of the pronounced polycythemia. Other postopera tive routines do not differ significantly from standard post-thoracotomy care. CASE REPORTS CASE 1.—J. De M. was a full-term male who weighed 5 pounds, 11 ounces. Cyanosis and a murmur were noted while he was in the newborn nursery. Because of deepening cyano sis he was admitted to the hospital at 3 weeks of age for evaluation.
Vol. 47, No. 2 February, 1964
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Physical examination disclosed cyanosis and a Grade 3 ejection systolic murmur at the left intercostal space. The electrocardiogram showed the QRS axis to be -60 degrees. The P waves were notched and peaked; there was left ventricular hypertrophy. The x-ray study revealed a normal-sized heart with diminished pulmonary vascularity. Selective angiocardio grams demonstrated tricuspid atresia (Type l a ) with no evidence of a right ventricle or main pulmonary artery. The pulmonary arteries filled from a patent ductus arteriosus. The hemo globin was 17 Gm. and the hematocrit was 53 vols. per cent. On May 5, 1961, when the patient was 2 months of age, because of his deepening cyanosis, an SVC-RPA shunt was performed with an end-to-end anastomosis. The postoperative course was uneventful; he had only slight facial edema at 24 hours and his color improved. He has grown well, although his development has been more slow than that of his siblings. He was examined in December, 1962, at which time the oxygen saturation by means of ear oximetry was 83 per cent. The hemoglobin was 14 Gm. and the hematocrit was 43 vols. per cent. The child is starting to walk at 24 months of age. CASE 2.—M. C. was a full-term male who weighed 6 pounds, 15 ounces. Cyanosis and a murmur were noted while he was still in the newborn nursery. An electrocardiogram at that time showed a QKS axis of -15 degrees, P pulmonale, and adult R / S progression in the precordial leads. The x-ray examination disclosed a normal-sized heart with diminished pul monary vascularity. After discharge from the hospital, the child's cyanosis deepened and the hemoglobin ranged between 17 and 18 Gm. and the hematocrit between 51 and 55 vols. per cent. At 5 months of age, the patient began having anoxic spells. When he was 6 months of age, a selective angiogram made at that time was consistent with tricuspid atresia (Type l b ) . In August, 1961, when he was 8 months of age, because of continued anoxic spells, an end-toend SVC-RPA anastomosis was performed. He did well postoperatively, with improved color and no more spells of anoxia. In December, 1962, a selective angiogram showed a wellfunctioning shunt with a good-sized distal pulmonary artery. When last seen in February, 1963, the child was crawling and standing with support. The hemoglobin was 15 Gm. and the hematocrit was 46 vols. per cent. The oxygen saturation by means of ear oximetry was 84 per cent. CASE 3.—R. S. was a full-term male who weighed 6 pounds, 12 ounces. Cyanosis and a murmur were noted at his 6-week examination. The cyanosis deepened and, at 18 months, he began having anoxic spells which were followed by attacks of cerebral thrombosis with a left hemiplegia. Diagnostic work-up at that time showed a Grade 3 murmur at the left sternal border. The electrocardiogram revealed a QRS axis of -45 degrees with biatrial en largement and a dominant left ventricle. The x-ray study of the chest demonstrated a normalsized heart with diminished pulmonary vascularity. The hemoglobin was 15 Gm. and the hematocrit was 65 vols. per cent. He was treated with iron, administered intramuscularly, and was discharged. When the child was 20 months of age because of continued anoxic spells, a left Blalock shunt was performed. Postoperatively he did well. When he was 4 years of age, cardiac catheterization with selective angiocardiograms confirmed the diagnosis of tricuspid atresia (Type l b ) . During the next 2 years, the child became progressively more cyanotic and showed markedly decreased exercise tolerance. I n October, 1962, clinical evaluation indicated that the Blalock shunt was grossly inadequate. The hemoglobin was 22 Gm. and the hematocrit was 77 vols. per cent. An SVC-RPA shunt with the use of the end-to-end technique was performed in No vember, 1962. He did remarkably well with essentially no " S V C syndrome." He now runs and climbs stairs and is considerably stronger. The oxygen saturation by means of ear oximetry was 85 per cent in January, 1963. The hemoglobin was 15 Gm. and the hematocrit was 47 vols. per cent. A selective angiogram revealed a well-functioning shunt (Type l b ) .
M E E S E E T AL.
Fig. 1.—Selective venous angiogram of patient R. S. made 2 months after an end-to-end superior vena cava-distal right pulmonary artery anastomosis demonstrates a patent, wellfunctioning shunt (A). Diagrammatic representation of the end-to-end SVC-RPA shunt in tricuspid atresia (B).
CASE 4.—K. M. was a full-term female who weighed 5 pounds, 10 ounces. Her mother had suffered for 10 years from a bizarre neurologic disease which closely resembled multiple sclerosis or Friedreich 's ataxia. A murmur was noted in the child while she was still in the nursery. Cyanosis was first recognized when she was 5 weeks of age. Shortly thereafter she began having anoxic spells; she was hospitalized and kept in oxygen. Transfer was made to this hospital because of repeated spells of anoxia. Physical examination on admission demonstrated 3+ cyanosis with a Grade 3 ejection systolic murmur along the left sternal border. The electrocardiogram showed a QBS axis of 0 degrees, P pulmonale, and left ventricular hypertrophy. The x-ray study revealed a normal-sized heart with diminished pulmonary vascularity. The hemoglobin was 16 Gm. and the hematocrit was 52 vols. per cent. Selective angiograms showed tricuspid ^tresia (Type l b ) with a patent ductus arteriosus.
Vol. 47, No. 2 February, 1964
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In June, 1962, when the child was 2 months of age, a SVC-EPA shunt was performed with the use of the end-to-side technique. Postoperatively, she had marked suffusion and edema of the head and upper extremities; these complications cleared after about 3 days. However, she had marked respiratory problems for the first week. This in turn was followed by febrile diarrhea. She did fairly well for a week and then developed a progressive ' ' SVC syndrome'' again. The child did poorly and one month postoperatively a left Blalock anasto mosis was performed. She improved, with subsidence of the edema and cyanosis. At no time, however, was a continuous murmur heard. Her general course since then has been poor. She has gained very little weight and weighs 10 pounds at 10 months of age in spite of a high caloric diet. Her development is at the 3- to 4-month level. However, she has had no spells of anoxia and the oxygen satu ration by means of ear oximetry was 85 per cent in December, 1962. Selective angiocardio grams revealed a well-functioning SCV-RPA shunt. There was also evidence of collateral circulation of the azygos vein with the highest intercostal vein. The major pulmonary artery to the right upper lobe was thrombosed. RESULTS
Table I lists the pertinent data on our 4 patients. Two of the patients, J. De M. and K. M., were 2 months of age at the time of operation and the third patient, M. C , was 8 months. Thus, 3 of the 4 patients were under one year of age at the time of operation. Three of the 4 patients had end-to-end anastomoses. Note that the total clamping time of the SVC was 10 minutes or less. All of the patients under one year of age had a pulmonary artery that was one-half or less that of the SVC. One of these patients, K. M., had an end-to-side anastomosis. It should be noted that the suffusion and edema of the head and upper ex tremities were much less in the patients with end-to-end anastomoses. In fact, the older patient, R. S., had this finding only for one hour. The prolongation of this phase in end-to-side anastomosis was not only true of our one patient but also of those patients in the literature who had similar anastomoses. None of our infant patients had preoperative oxygen saturation determina tions made. Because of the severity of their symptoms, selective angiocardio grams plus the clinical data formed the diagnostic work-up. However, the fall in hemoglobin and hematocrit levels attested to the benefit derived. The post operative oxygen saturation determinations made by ear oximetry were all re cently accomplished and were remarkably similar at 83 to 85 per cent. All patients were kept in a semi-Fowler's position for 24 to 48 hours after suffusion subsided. Thereafter, they have slept flat. In view of Glenn's admo nition, 4 we wonder how much this may have contributed to our problems in pa tient K. M. who had an end-to-side anastomosis and required a second procedure one month postoperatively. The first 2 patients have been followed 22 and 18 months, respectively, and they have had no ill effects from the recumbent position. Patient K. M. warrants special mention. She was the only patient in our series who had an end-to-side anastomosis. She also had a complicated post operative course and evidence that there was a thrombosis of one of the right pulmonary artery branches. Her case will be presented in more detail in a sub sequent report. Precisely why she had SVC obstruction one month postopera-
T
Cardiovas. Surg. TABLE I.
PREOP. HGB. (GM.)
HCT.
TRICUSPID ATRESIA
TYPE OF ANASTO MOSIS
DURATION SVC CLAMP ING
RATIO RPA: SVC
PATIENT
TYPE
AGE AT OPERATION
J.De M. M
la
2 mo.
17
53
End-to-end
10 min.
1:2
M. C. M
lb
8 mo.
18
55
End-to-end
9 min.
1:3
R. S. M
lb
6 yr.
22
77
End-to-end
9 min.
3:4
K. M. F
lb
2 mo.
16
52
End-to-side
Partial occlusion
1:3
(%)
tively is not quite clear. "What is also unclear is the good response to a left Blaloek shunt. Theoretically, a well-functioning systemic artery shunt should have ensured the failure of the SVC-RPA anastomosis. One disturbing result in our patients is the subsequent growth and de velopment. Patient J. De M. is just starting to walk at the age of 2 years. While his height and weight gains have been excellent, his general development is slow. Patient M. C. is sitting alone and crawling at 20 months of age but is not walking; yet his height and weight are excellent. On the other hand, pa tient R. S., who was mentally normal before the operation but was severely limited physically, is now running and very active. His mother and teacher feel that he is much more alert since operation. It would appear that the slow development is not necessarily secondary to any brain damage subsequent to SVC clamping. 5 Patient K. M. who had partial occlusion only is doing the poorest of all. She is well below the third percentile in height and weight and is functioning at a 3- to 4-month level. The best answer that we can give is the possible as sociation of other defects so commonly seen with congenital heart disease. DISCUSSION
No comprehensive review of the literature will be attempted in this article insofar as it has been so recently accomplished by Bopp and his co-workers.4 We have tried to classify our patients prior to surgery and have selected Keith's 3 Type la and Type lb as those cases in which this procedure is most suitable. Operations in Type Ic and Type I I patients for the most part would be contraindicated. The end-to-end anastomosis has been very successful in our hands. The mortality has been nil and the morbidity minimal. We believe that this success may be due to the fact that the operation was brief and the total clamping time of the SVC was kept around 10 minutes. Bakulev and Kolesnikov" indicate that the SVC can be clamped for longer periods, but they do not present enough data on which to make a judgment. Whereas the pulmonary artery was one half or
TR1CUSPID AT RESIA
Vol. 47. No. 2 Februar> , 1964
267
W I T H SVC-RPA S H U N T POSTOP.
POSTOP.
SVC SYNDROME
HGB. (GM.)
HCT.
02SAT.
(%)
(%)
24 hr.
14
43
83
22
Height and weight excellent, starting to walk, no anoxic spells
24 hr.
15
46
84
18
Height and weight excellent, crawling, no anoxic spells
1 hr.
15
47
85
3
Runs, climbs stairs, more alert
3 da.
14
41
84
8
Recurrent SVC syndrome, left Blalock; failure to thrive, less than 3 percentile in height and weight
FOLLOW-UP (MO.)
COURSE
less than that of the SVC in the 2 infants, it was still technically possible to make this anastomosis within the arbitrary 10-minute period. Brechner's 5 dem onstration of electroencephalographic changes coincident to SVC obstruction during operation were transient and quickly reversible. We have had no electroencephalographic monitoring but suspect that they were equally reversible in our patients. It seems reasonable that an end-to-end anastomosis should function better than an angulated end-to-side anastomosis when the velocity of blood flow through a small conduit at a low pressure is considered.7 The turbulence, resistance to flow, and tendency to thrombosis all should be less. The head of pressure consequent to total clamping of the SVC may allow flow at a greater pressure initially. 8 The size and firmness of the distal pulmonary artery upon release of the clamps would indicate this. This should be quickly dissipated, but may be of sufficient duration to overcome the inherently high pulmonary resistance in these infants in order to establish a good functioning shunt. Cer tainly the resistance to flow was less in these patients as demonstrated by the short duration of suffusion of the head and upper extremities. On the other hand, our infant with the end-to-side anastomosis had sig nificantly greater morbidity with suffusion of longer duration; reappearance of evidence of obstruction one month later; a second procedure and evidence, on a later study, of thrombosis of one of the branches of the distal right pulmonary artery. SUMMARY
Four patients with trieuspid atresia treated by SVC-RPA shunts have been presented. Three patients had end-to-end anastomoses. One patient was treated with end-to-side technique. Three patients were under one year of age. There were no deaths. The 3 patients with end-to-end shunts had no morbidity. The patient with end-to-side anastomosis had multiple complications which resulted in a second surgical procedure. The possible advantages of end-to-end technique are discussed.
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J. Thoracic and Cardiovas. Surg.
REFERENCES
1. Eodbard, S., and Wagner, D . : By-passing the Eight Ventricle, Proc. Soc. Exper. Biol. 7 1 : 69-70, 1949. 2. Eeed, W. A., Kittle, C. F . , and Heilbrunn, A . : Superior Vena Cava-Pulmonary Artery Anastomosis, A. M. A. Arch. Surg. 86: 87-95, 1963. 3. Keith, J . D., Bowe, E. D., and Vlad, P . : Heart Disease in Infancy and Childhood, NewYork, 1958, The Macmillan Company, p. 437. 4. Bopp, E. K., Larsen, P . B., Caddell, J . L., Patrick, J . E., Hipona, F . A., and Glenn, VV. W. L . : Surgical Considerations for Treatment of Congenital Tricuspid Atresia and Stenosis, With Particular Eeference to Vena Cava-Pulmonary Artery Anasto mosis, J . THORACIC & CARDIOVAS. SURG. 4 3 : 97-113, 1962.
5. Brechner, V. L., Kavan, E. M., and Dillon, J . B . : The Electroencephalographic Effect of Compression of the Superior Vena Cava During Thoracotomy, J . THORACIC SURG. 37: 352-360, 1959. 6. Bakulev, A. N., and Kolesnikov, S. A . : Anastomosis of Superior Vena Cava and Pulmo nary Artery in the Surgical Treatment of Certain Congenital Defects of the Heart, J . THORACIC SURG. 37: 693-702, 1959.
7. Bushmer, E. F . : Cardiovascular Dynamics, ed. 2, Philadelphia and London, 1961, W. B. Saunders Company, pp. 6-8. 8. Glenn, W. W. L., and Patino, J . F . : Circulatory By-Pass of the Eight Heart. 1. Pre liminary Observations on the Direct Delivery of Vena Caval Blood Into the Pul monary Arterial Circulation. Azygos Vein-Pulmonary Artery Shunt, Yale J . Biol. Med. 2 7 : 147-151, 1954.
Lieutenant
Commander
(MC)
USN,
Thomas B. Delaney,**
Commander
(MC)
USN, and
Donald J. Doohen,***
Commander
(MC)
USN, St. A/bans, N.
and Joseph ]. Timmes,
M.D.,****
Y.,
Jersey City, N. J.
19491 the procedure of partial right heart bypass has moved from the experimental stage in dogs to clinical usage. This procedure finds its great est application in cardiac defects in which there is diminished pulmonary flow not amenable to complete correction at a later date. Therefore, tricuspid atresia, Ebstein 's anomaly, and complicated defects associated with abdominal heterotaxy present themselves as lesions for which this operation is ideal. Unfortunately, although this is a nice concept, the surgical procedure on patients under one year of age, and especially under 6 months of age, has been attendant with a high mortality. Indeed, Reed and his associates2 recommend that the use of this procedure be discontinued in patients under one year of age. What is more unfortunate is the fact that many babies won't survive to the age of one year to become a candidate for this palliation. This is particularly true of the Type la tricuspid atresia. 3 We believe that our experience with a small number of cases in this age group with the use of end-to-end anastomoses has allowed survival and less mor bidity than the end-to-side method. Assuming that this procedure also has an equivalent or more acceptable mortality than systemic artery shunts, it is pref erable because of the decreased work on the heart and the presentation to the lungs of lower saturated blood at a lower pressure. It is the purpose of this paper to present the cases of 4 patients with tri cuspid atresia on whom an SVC-RPA shunt was performed successfully. Three of the patients are under the age of one year. The 3 patients who had end-to-end
S
INCE
From the U. S. Naval Hospital, St. Albans, N. Y., and Seton Hall College of Medicine, Jersey City, N. J. Opinions expressed herein are those of the authors and do not necessarily reflect the views of the Navy Department of the Naval Service at large. Received for publication June 12, 1963. •Senior Resident, Department of Thoracic and Cardiovascular Surgery, U. S. Naval Hospital, St. Albans, N. Y. *'Cardiologist, Department of Pediatrics, U. S. Naval Hospital, St. Albans, N. Y. **'Chief, Department of Thoracic and Cardiovascular Surgery, U. S. Naval Hospital. St. Albans, N. Y. ••••Professor of Surgery, Seton Hall College of Medicine, Jersey City, N. J. 261
MEESE ET AL.
262
J. Thoracic and Cardiovas. Surg.
anastomoses did significanth- better than the patient in whom the anastomosis was end-to-side. The surgical procedure on the former group will be presented. MATERIAL, AND
METHODS
The 4 patients in this series ranged in age from 2 months to 6 years. All had tricuspid atresia proved by cardiac catheterization and/or selective angio cardiography. Operation was performed on the 3 infants because of severe anoxic difficulties. The 6-year-old child was operated upon because of severe restriction and cyanosis. The right pleural cavity was exposed by a posterolateral thoracotomy in cision through the fourth intercostal space. The right pulmonary artery and superior vena cava were quickly but carefully mobilized from adherent adjacent tissues. The azygos vein was ligated and divided immediately proximal to its entrance into the superior vena cava. This aided in the mobilization of this great vein and provided a greater length to the caval limb of the future anastomosis. The right pulmonary artery was clamped just distal to the bifurcation of the main vessel and divided. The vena cava was similarly clamped and divided immediately proximal to the right atrium. A rapid end-to-end anastomosis was then achieved between the proximal superior vena cava and distal pulmonary artery (Fig. 1, B). Two continuous, over-and-over, 5-0 arterial silk sutures were used for the anterior and posterior rows of this anastomosis. The two sutures were not joined at either end to allow for growth at the anastomotic site. The total time for clamping of the superior vena cava was 10 minutes or less in all instances. After removal of the clamps, the distal right pulmonary artery be came rapidly distended, demonstrating a functionally patent anastomosis. The proximal pulmonary artery and the right atrium were then closed by ligation and sutures, respectively. The operative wound was closed and the pleural cavity was drained by a small Foley catheter attached to underwater seal suction. The average procedure was usually performed within 30 minutes. Total operating time has been shortened in these bad risk patients by avoiding bypass shunts and pressure monitoring. Postoperatively the patients with an end-to-end anastomosis have been kept in a semi-Fowler's position for no longer than 48 hours. The vise of this elevation of the head and trunk is believed not only to aid in respiratory exchange but also to aid gravity in promoting blood flow through the anastomosis during its early hours of function. Blood loss during and following the procedure was replaced by plasma because of the pronounced polycythemia. Other postopera tive routines do not differ significantly from standard post-thoracotomy care. CASE REPORTS CASE 1.—J. De M. was a full-term male who weighed 5 pounds, 11 ounces. Cyanosis and a murmur were noted while he was in the newborn nursery. Because of deepening cyano sis he was admitted to the hospital at 3 weeks of age for evaluation.
Vol. 47, No. 2 February, 1964
TKI CUSPID A T R E S I A
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Physical examination disclosed cyanosis and a Grade 3 ejection systolic murmur at the left intercostal space. The electrocardiogram showed the QRS axis to be -60 degrees. The P waves were notched and peaked; there was left ventricular hypertrophy. The x-ray study revealed a normal-sized heart with diminished pulmonary vascularity. Selective angiocardio grams demonstrated tricuspid atresia (Type l a ) with no evidence of a right ventricle or main pulmonary artery. The pulmonary arteries filled from a patent ductus arteriosus. The hemo globin was 17 Gm. and the hematocrit was 53 vols. per cent. On May 5, 1961, when the patient was 2 months of age, because of his deepening cyanosis, an SVC-RPA shunt was performed with an end-to-end anastomosis. The postoperative course was uneventful; he had only slight facial edema at 24 hours and his color improved. He has grown well, although his development has been more slow than that of his siblings. He was examined in December, 1962, at which time the oxygen saturation by means of ear oximetry was 83 per cent. The hemoglobin was 14 Gm. and the hematocrit was 43 vols. per cent. The child is starting to walk at 24 months of age. CASE 2.—M. C. was a full-term male who weighed 6 pounds, 15 ounces. Cyanosis and a murmur were noted while he was still in the newborn nursery. An electrocardiogram at that time showed a QKS axis of -15 degrees, P pulmonale, and adult R / S progression in the precordial leads. The x-ray examination disclosed a normal-sized heart with diminished pul monary vascularity. After discharge from the hospital, the child's cyanosis deepened and the hemoglobin ranged between 17 and 18 Gm. and the hematocrit between 51 and 55 vols. per cent. At 5 months of age, the patient began having anoxic spells. When he was 6 months of age, a selective angiogram made at that time was consistent with tricuspid atresia (Type l b ) . In August, 1961, when he was 8 months of age, because of continued anoxic spells, an end-toend SVC-RPA anastomosis was performed. He did well postoperatively, with improved color and no more spells of anoxia. In December, 1962, a selective angiogram showed a wellfunctioning shunt with a good-sized distal pulmonary artery. When last seen in February, 1963, the child was crawling and standing with support. The hemoglobin was 15 Gm. and the hematocrit was 46 vols. per cent. The oxygen saturation by means of ear oximetry was 84 per cent. CASE 3.—R. S. was a full-term male who weighed 6 pounds, 12 ounces. Cyanosis and a murmur were noted at his 6-week examination. The cyanosis deepened and, at 18 months, he began having anoxic spells which were followed by attacks of cerebral thrombosis with a left hemiplegia. Diagnostic work-up at that time showed a Grade 3 murmur at the left sternal border. The electrocardiogram revealed a QRS axis of -45 degrees with biatrial en largement and a dominant left ventricle. The x-ray study of the chest demonstrated a normalsized heart with diminished pulmonary vascularity. The hemoglobin was 15 Gm. and the hematocrit was 65 vols. per cent. He was treated with iron, administered intramuscularly, and was discharged. When the child was 20 months of age because of continued anoxic spells, a left Blalock shunt was performed. Postoperatively he did well. When he was 4 years of age, cardiac catheterization with selective angiocardiograms confirmed the diagnosis of tricuspid atresia (Type l b ) . During the next 2 years, the child became progressively more cyanotic and showed markedly decreased exercise tolerance. I n October, 1962, clinical evaluation indicated that the Blalock shunt was grossly inadequate. The hemoglobin was 22 Gm. and the hematocrit was 77 vols. per cent. An SVC-RPA shunt with the use of the end-to-end technique was performed in No vember, 1962. He did remarkably well with essentially no " S V C syndrome." He now runs and climbs stairs and is considerably stronger. The oxygen saturation by means of ear oximetry was 85 per cent in January, 1963. The hemoglobin was 15 Gm. and the hematocrit was 47 vols. per cent. A selective angiogram revealed a well-functioning shunt (Type l b ) .
M E E S E E T AL.
Fig. 1.—Selective venous angiogram of patient R. S. made 2 months after an end-to-end superior vena cava-distal right pulmonary artery anastomosis demonstrates a patent, wellfunctioning shunt (A). Diagrammatic representation of the end-to-end SVC-RPA shunt in tricuspid atresia (B).
CASE 4.—K. M. was a full-term female who weighed 5 pounds, 10 ounces. Her mother had suffered for 10 years from a bizarre neurologic disease which closely resembled multiple sclerosis or Friedreich 's ataxia. A murmur was noted in the child while she was still in the nursery. Cyanosis was first recognized when she was 5 weeks of age. Shortly thereafter she began having anoxic spells; she was hospitalized and kept in oxygen. Transfer was made to this hospital because of repeated spells of anoxia. Physical examination on admission demonstrated 3+ cyanosis with a Grade 3 ejection systolic murmur along the left sternal border. The electrocardiogram showed a QBS axis of 0 degrees, P pulmonale, and left ventricular hypertrophy. The x-ray study revealed a normal-sized heart with diminished pulmonary vascularity. The hemoglobin was 16 Gm. and the hematocrit was 52 vols. per cent. Selective angiograms showed tricuspid ^tresia (Type l b ) with a patent ductus arteriosus.
Vol. 47, No. 2 February, 1964
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In June, 1962, when the child was 2 months of age, a SVC-EPA shunt was performed with the use of the end-to-side technique. Postoperatively, she had marked suffusion and edema of the head and upper extremities; these complications cleared after about 3 days. However, she had marked respiratory problems for the first week. This in turn was followed by febrile diarrhea. She did fairly well for a week and then developed a progressive ' ' SVC syndrome'' again. The child did poorly and one month postoperatively a left Blalock anasto mosis was performed. She improved, with subsidence of the edema and cyanosis. At no time, however, was a continuous murmur heard. Her general course since then has been poor. She has gained very little weight and weighs 10 pounds at 10 months of age in spite of a high caloric diet. Her development is at the 3- to 4-month level. However, she has had no spells of anoxia and the oxygen satu ration by means of ear oximetry was 85 per cent in December, 1962. Selective angiocardio grams revealed a well-functioning SCV-RPA shunt. There was also evidence of collateral circulation of the azygos vein with the highest intercostal vein. The major pulmonary artery to the right upper lobe was thrombosed. RESULTS
Table I lists the pertinent data on our 4 patients. Two of the patients, J. De M. and K. M., were 2 months of age at the time of operation and the third patient, M. C , was 8 months. Thus, 3 of the 4 patients were under one year of age at the time of operation. Three of the 4 patients had end-to-end anastomoses. Note that the total clamping time of the SVC was 10 minutes or less. All of the patients under one year of age had a pulmonary artery that was one-half or less that of the SVC. One of these patients, K. M., had an end-to-side anastomosis. It should be noted that the suffusion and edema of the head and upper ex tremities were much less in the patients with end-to-end anastomoses. In fact, the older patient, R. S., had this finding only for one hour. The prolongation of this phase in end-to-side anastomosis was not only true of our one patient but also of those patients in the literature who had similar anastomoses. None of our infant patients had preoperative oxygen saturation determina tions made. Because of the severity of their symptoms, selective angiocardio grams plus the clinical data formed the diagnostic work-up. However, the fall in hemoglobin and hematocrit levels attested to the benefit derived. The post operative oxygen saturation determinations made by ear oximetry were all re cently accomplished and were remarkably similar at 83 to 85 per cent. All patients were kept in a semi-Fowler's position for 24 to 48 hours after suffusion subsided. Thereafter, they have slept flat. In view of Glenn's admo nition, 4 we wonder how much this may have contributed to our problems in pa tient K. M. who had an end-to-side anastomosis and required a second procedure one month postoperatively. The first 2 patients have been followed 22 and 18 months, respectively, and they have had no ill effects from the recumbent position. Patient K. M. warrants special mention. She was the only patient in our series who had an end-to-side anastomosis. She also had a complicated post operative course and evidence that there was a thrombosis of one of the right pulmonary artery branches. Her case will be presented in more detail in a sub sequent report. Precisely why she had SVC obstruction one month postopera-
T
Cardiovas. Surg. TABLE I.
PREOP. HGB. (GM.)
HCT.
TRICUSPID ATRESIA
TYPE OF ANASTO MOSIS
DURATION SVC CLAMP ING
RATIO RPA: SVC
PATIENT
TYPE
AGE AT OPERATION
J.De M. M
la
2 mo.
17
53
End-to-end
10 min.
1:2
M. C. M
lb
8 mo.
18
55
End-to-end
9 min.
1:3
R. S. M
lb
6 yr.
22
77
End-to-end
9 min.
3:4
K. M. F
lb
2 mo.
16
52
End-to-side
Partial occlusion
1:3
(%)
tively is not quite clear. "What is also unclear is the good response to a left Blaloek shunt. Theoretically, a well-functioning systemic artery shunt should have ensured the failure of the SVC-RPA anastomosis. One disturbing result in our patients is the subsequent growth and de velopment. Patient J. De M. is just starting to walk at the age of 2 years. While his height and weight gains have been excellent, his general development is slow. Patient M. C. is sitting alone and crawling at 20 months of age but is not walking; yet his height and weight are excellent. On the other hand, pa tient R. S., who was mentally normal before the operation but was severely limited physically, is now running and very active. His mother and teacher feel that he is much more alert since operation. It would appear that the slow development is not necessarily secondary to any brain damage subsequent to SVC clamping. 5 Patient K. M. who had partial occlusion only is doing the poorest of all. She is well below the third percentile in height and weight and is functioning at a 3- to 4-month level. The best answer that we can give is the possible as sociation of other defects so commonly seen with congenital heart disease. DISCUSSION
No comprehensive review of the literature will be attempted in this article insofar as it has been so recently accomplished by Bopp and his co-workers.4 We have tried to classify our patients prior to surgery and have selected Keith's 3 Type la and Type lb as those cases in which this procedure is most suitable. Operations in Type Ic and Type I I patients for the most part would be contraindicated. The end-to-end anastomosis has been very successful in our hands. The mortality has been nil and the morbidity minimal. We believe that this success may be due to the fact that the operation was brief and the total clamping time of the SVC was kept around 10 minutes. Bakulev and Kolesnikov" indicate that the SVC can be clamped for longer periods, but they do not present enough data on which to make a judgment. Whereas the pulmonary artery was one half or
TR1CUSPID AT RESIA
Vol. 47. No. 2 Februar> , 1964
267
W I T H SVC-RPA S H U N T POSTOP.
POSTOP.
SVC SYNDROME
HGB. (GM.)
HCT.
02SAT.
(%)
(%)
24 hr.
14
43
83
22
Height and weight excellent, starting to walk, no anoxic spells
24 hr.
15
46
84
18
Height and weight excellent, crawling, no anoxic spells
1 hr.
15
47
85
3
Runs, climbs stairs, more alert
3 da.
14
41
84
8
Recurrent SVC syndrome, left Blalock; failure to thrive, less than 3 percentile in height and weight
FOLLOW-UP (MO.)
COURSE
less than that of the SVC in the 2 infants, it was still technically possible to make this anastomosis within the arbitrary 10-minute period. Brechner's 5 dem onstration of electroencephalographic changes coincident to SVC obstruction during operation were transient and quickly reversible. We have had no electroencephalographic monitoring but suspect that they were equally reversible in our patients. It seems reasonable that an end-to-end anastomosis should function better than an angulated end-to-side anastomosis when the velocity of blood flow through a small conduit at a low pressure is considered.7 The turbulence, resistance to flow, and tendency to thrombosis all should be less. The head of pressure consequent to total clamping of the SVC may allow flow at a greater pressure initially. 8 The size and firmness of the distal pulmonary artery upon release of the clamps would indicate this. This should be quickly dissipated, but may be of sufficient duration to overcome the inherently high pulmonary resistance in these infants in order to establish a good functioning shunt. Cer tainly the resistance to flow was less in these patients as demonstrated by the short duration of suffusion of the head and upper extremities. On the other hand, our infant with the end-to-side anastomosis had sig nificantly greater morbidity with suffusion of longer duration; reappearance of evidence of obstruction one month later; a second procedure and evidence, on a later study, of thrombosis of one of the branches of the distal right pulmonary artery. SUMMARY
Four patients with trieuspid atresia treated by SVC-RPA shunts have been presented. Three patients had end-to-end anastomoses. One patient was treated with end-to-side technique. Three patients were under one year of age. There were no deaths. The 3 patients with end-to-end shunts had no morbidity. The patient with end-to-side anastomosis had multiple complications which resulted in a second surgical procedure. The possible advantages of end-to-end technique are discussed.
MEESE E T AL.
268
J. Thoracic and Cardiovas. Surg.
REFERENCES
1. Eodbard, S., and Wagner, D . : By-passing the Eight Ventricle, Proc. Soc. Exper. Biol. 7 1 : 69-70, 1949. 2. Eeed, W. A., Kittle, C. F . , and Heilbrunn, A . : Superior Vena Cava-Pulmonary Artery Anastomosis, A. M. A. Arch. Surg. 86: 87-95, 1963. 3. Keith, J . D., Bowe, E. D., and Vlad, P . : Heart Disease in Infancy and Childhood, NewYork, 1958, The Macmillan Company, p. 437. 4. Bopp, E. K., Larsen, P . B., Caddell, J . L., Patrick, J . E., Hipona, F . A., and Glenn, VV. W. L . : Surgical Considerations for Treatment of Congenital Tricuspid Atresia and Stenosis, With Particular Eeference to Vena Cava-Pulmonary Artery Anasto mosis, J . THORACIC & CARDIOVAS. SURG. 4 3 : 97-113, 1962.
5. Brechner, V. L., Kavan, E. M., and Dillon, J . B . : The Electroencephalographic Effect of Compression of the Superior Vena Cava During Thoracotomy, J . THORACIC SURG. 37: 352-360, 1959. 6. Bakulev, A. N., and Kolesnikov, S. A . : Anastomosis of Superior Vena Cava and Pulmo nary Artery in the Surgical Treatment of Certain Congenital Defects of the Heart, J . THORACIC SURG. 37: 693-702, 1959.
7. Bushmer, E. F . : Cardiovascular Dynamics, ed. 2, Philadelphia and London, 1961, W. B. Saunders Company, pp. 6-8. 8. Glenn, W. W. L., and Patino, J . F . : Circulatory By-Pass of the Eight Heart. 1. Pre liminary Observations on the Direct Delivery of Vena Caval Blood Into the Pul monary Arterial Circulation. Azygos Vein-Pulmonary Artery Shunt, Yale J . Biol. Med. 2 7 : 147-151, 1954.