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Anastomosis of the subclavian artery to the descending aorta for the correction of interrupted aortic arch
Anastomosis of the subclavian artery to the descending aorta for the correction of interrupted aortic arch Miguel Barbero Marcial, * Francisco Gregori, Ir., ** Rachel Snitcowsky ,*** Munir Ebaid, **** Geraldo Verginelli, * and E. J. Zerbini, ***** Sao Paulo, Brazil
Icongenital nterruption of the aortic arch is a rare anomaly (0.4 per cent) ."
11-11
There is a high mortality rate (approximately 80 per cent) n, " during the first year of life. There have been 200 known cases of interrupted aortic arch"?" up to now but only 15 of them were successfully treated surgically."?" The present case (Type A, Celoria and Patton's" classification) is reported because of the technical difficulties in surgical management. End-to-end anastomosis of the subclavian artery to the descending aorta was done to re-establish continuity of the aorta. Case report M. S. F., a 6-year-old Brazilian girl, was first seen in June, 1967, at the age of 15 months. At the time a history of frequent episodes of bronchitis was presented. Clinical, laboratory, and hemodynamic studies were done and the diagnosis of intraventricular From the Heart Institute of the University of Sao Paulo Medical School, Sao Paulo, Brazil. Address for reprints: Heart Institute, Caixa Postal 8091, Sao Paulo, Brazil. Received for publication May 25, 1973. • Assistant Professor, Department of Surgery. •• Fellow of Cardiac Surgery. ••• Assistant, Department of Medical Clinic. •••• Assistant Professor, Department of Medical Clinic. *****Professor of Surgery, Honorary Member of The American Association for Thoracic Surgery.
septal defect (IYSD) with patent ductus arteriosus and pulmonary hypertension was made. She underwent surgical Ireatment approximately three years later. The operation was limited to thoracotomy because the canal could not be closed. The aortic arch between the pulmonary artery and the descending aorta was completely interrupted. The case was reviewed and reoperation was proposed. At this time physical examination showed the child to be underdeveloped, acyanotic, without dyspnea, and in rather good general condition. The blood pressure in the upper limbs was 180/100 mm. Hg and in the lower limbs it was 120/60 mm. Hg. The heart rate was 92 beats per minute. On auscultation, the second sound in the pulmonary area was hyperphonetic, with the second component louder than first. An intense systolic murmur of regurgitation irradiating throughout the precordium and dorsum was audible in the mesocardium. The preoperative electrocardiogram (ECG) is shown in Fig. 1. The x-ray (Fig. 2) showed increased pulmonary vasculature, especially in the hilum. It was more developed in the apexes than in the bases. The cardiac area was increased at the expense of the right atrium and both ventricles. The pulmonary artery was obviously dilated. The results of the hemodynamic studies are shown in Table I. The IYSD, patent ductus arteriosus, and interrupted aortic arch were shown by cineangiocardiography. Fig. 3 shows diagrammatically the interruption of the aotic arch corresponding to Type A (Celoria and Patton") . The patient was operated upon in August, 1972. By means of a left posterolateral thoracotomy approach, the descending aorta, ductus, left sub-
653
65 4
Barbero Marcial et al.
The Journol of Thoracic and Card iovascular
Surg ery
Fig. 1. Preoperative ECG showing signs of biventricular overload.
Fig. 2. Preoperative chest x-ray show ing increased pulmonary circulation. ma inly hilar, a nd increased ca rdiac area due to left he art enl ar gement. The pulmonar y artery is also visibly enlarged . cla vian a rtery. and proxim al ao rta were dissected . In view of the impossibility of re-establi shin g co ntinui ty of the aorta by end-to-end anasto mosis, a nd becau se the left subcla vian showed a good ca liber, a n end -to-end ana stom osis between the latte r a nd the descending aorta was perf ormed. Th e ca na l was sectioned and sutured . The postoperative course was very sati sfactory and the patient is presently asymptomatic a nd in good genera l health. On ausc ultation, the second sound continues hyperphonetic in the pulmonary area with the second co mponent louder than the first. A systolic mu rmu r of med ium intensity in the mesocardium a nd an intense aspira ting diasto lic murm ur, mo re clea rly a udible in the seco nd left intercosta l spac e, a re presen t. The ECG in Fig. 4 sho ws o nly a decrease in the right ventricular o verlo ad . The x-ray (Fig. 5 ) shows a significant decr ease of the hea rt size. Th e results of the hem odyn am ic study a re show n in T abl e II . Lef t cinea ngioca rdio gra phy ( Fig. 6) shows the existenc e of a n IV defect with left-to-right shunt and with good contras t of the pulm on ary a rtery. I n Fig. 7 the end -to-e nd ana stomosis bet ween the left subclavia n and the descend ing ao rta is clearly visible.
Fig. 3. Schematic drawing showing an aortic arch inte rru ption of T ype A. The descendin g thoracic ao rta is communica ting with the pu lmonary arte ry through the patent d uctu s.
Discussion This is a very serious congenital anomaly; the average life span is 10 days, with a mortality rate of 80 per cent during the first month after birth." On the other hand. according to Van Praagh and associates ," 31 per cent of the deaths during the first month of life due to congenital card iovascular anom alies are caused by this type of malformaiton . The most common causes of dea th arc heart failure and pulmonary hypertension. In the present case , the fact that the pat ient lived to the age of six without serious symptoms attracted our attention. We know of only 15 patients successfull y operated upon.' :" It is hop ed that , as the diagnosis and technique improve, prognosis will be better. The surgical technique used may be pal-
Volume 66 Number 4 October. 1973
Correction of interrupted aortic arch
655
Fig. 4. Postoperative ECG showing diminished right ventricular overload.
Fig. 6. Left heart cineangiocardiography showing a termino-terrninal anastomosis between the left subclavian artery and the thoracic descending aorta.
Fig 5. Postoperative chest roentgenogram showing diminished cardiac area
liative or corrective. Palliation consists of placing a graft between the pulmonary artery and the descending aorta. Th is technique is used mainly in children in whom the ductus is closing and a graft cannot be placed in the ascending aorta. Corrective technique consists of placing the graft between the ascending and descending aorta; rarely is direct anastomosis between the two stumps possible. Finally, if the caliber of the subclavian artery is good , it may be anastomosed to the descending aorta. In the case presented, the latter technique was
Fig. 7. Left heart cineangiocardiography show ing a high ventricular septal defect and a good filling of the pulmonary artery.
used because of the long distance between the aortic stumps and the difficulty of getting close to the ascending aorta through the left posterolateral thoracotomy approach. The hemod ynamic study done three months
656
The Journal of Thorocic ond Cardiovascular Surgery
Barbero Marcial et al.
Table I. Preoperative catheterization»
Chambers Right ventricle Pulmonary artery Left ventricle Aorta (asc.) Aorta (desc.)
Systolic pressure
Initial diastolic pressure
End-diastolic pressure
Mean pressure
(mm, Hg]
(mm. Hg)
(mm, Hg)
(mm. Hg)
67 57 100 110 50
2.5 42 3.5 66 41
9 11
43 52.5 94 94 49
'Conclusion: Pulmonary artery hypertension; significant gradient, ascending aorta-descending aorta.
Table II. Postoperative catheterization"
Chambers Right atrium Right ventricle Pulmonary artery Wedge pressure Left ventricle Aorta (asc.) Aorta (desc.)
Systolic pressure
Initial diastolic pressure
End-diastolic pressure
Mean pressure
(mm. Hg)
(mm, Hg)
(mm. Hg)
(mm. Hg)
4.2 123 123
-10 95
9.8
137 137 89.4
-10 58.8 60.2
9.8
70 12 102.2 79.8
*Conc1usion: Right ventricular hypertension; pulmonary artery hypertension; significant gradient. ascending aorta-descending aorta.
postoperatively showed the presence of an A-V shunt (Op/Os = 2: 1) because of the IV defect, moderate pulmonary hyperresistance (Rp/Rs = 0.27) (Ni = 0.08), and moderate gradient across the subclavian artery. The good postoperative result was confirmed by the improvement of the ECG and x-ray. The IV defect will be corrected at the time of six-month follow-up.
REFERENCES
2
3
Summary
The case of a 6-year-old girl with interruption of the aortic arch (Type A, Celoria and Patton classification), ventricular septal defect, and patent ductus arteriosus is presented. Continuity of the aorta was established by end-to-end anastomosis of the subclavian artery and descending aorta. Progress was satisfactory. The fact that the patient lived to the age of six without serious symptoms is worth emphasizing, as is also the surgical technique used.
4
5
6
7
Barratt-Boyes, B. G., Nicholls, T. T., Brandt, P. W. T., and Neutze, J. M.: Aortic Arch Interruption Associated With Patent Ductus Arteriosus, Ventricular Septal Defect, and Total Anomalous Pulmonary Venous Connection, J. THoRAc. CARDIOVASC. SURG. 63: 367, 1972. Blake, H. A., Manion, W. c., and Spencer, F. C.: Atresia or Absence of the Aortic Isthmus, J. THoRAc. CARDIOVASC. SURG. 43: 607, 1962. Celoria, G. c., and Patton, R. B.: Congenital Absence of the Aortic Arch, Am. Heart J. 58: 407, 1959. Litwin, S. B., Van Praagh, R., and Bernhard, W. F.: A Palliative Operation for Certain Infants With Aortic Arch Interruption, Ann. Thorac. Surg. 14: 369, 1972. Pillsbury, R. C., Lower, R. R., and Shumway, N. E.: Atresia of the Aortic Arch, Circulation 30: 749, 1972. Rochette, M., Stanley, P., Ethier, M. F., and Davignon, A.: Complete Interruption of the Aortic Arch in Infancy, Can. Med. Assoc. J. 98: 131, 1968. Tawes, R. L., Jr., Panagopoulos, P., Aberdeen, E., Waterston, D. J., Bonham-Carter, R. E.,
Volume 66 Number 4
Correction of interrupted aortic arch
657
October, 1973
8
9
10
11
and Stephens, H. B.: Aortic Arch Atresia and Interruption of the Aortic Arch, J. THORAC. CARDIOVASC. SURG. 58: 492, 1969. Tyson, K. R. T., Harris, L. C., and Nghiern, Q. X.: Repair of the Aortic Arch Interruption in the Neonate, Surgery 67: 1006, 1970. Van Praagh, R., Bernhard, W. F., Rosenthal, A., Parisi, L., and Fyler, D. c.: Interrupted Aotic Arch; Surgical Treatment, Am. J. Cardial. 27: 201,1971. Kinsley, R. H., Utian, N. C., Fuller, D. N., and Marchand, P. E.: Interruption of the Aortic Arch, Thorax 27: 93, 1972. Evans, W.: Congenital Stenosis (Coarctation),
Atresia and Interruption of the Aortic Arch (a Study of 28 Cases), Quart. J. Med. 26: 1, 1933. 12 Abbott, M. E.: Atlas of Congenital Cardiac Diseases, New York, 1936, American Heart Association, Publisher, Chart I. 12 Hamburger, L. P.: Congenital Cardiac Malformation Presenting Complete Interruption of the Isthmus Aortas With Transposition of the Great Arteries, Bull. Johns Hopkins Hosp. 61: 421, 1937. 14 Sewart, M.: Congenital Interruption of the Aortic Arch, Arch. Dis. Child. 76: 326, 1948.
Icongenital nterruption of the aortic arch is a rare anomaly (0.4 per cent) ."
11-11
There is a high mortality rate (approximately 80 per cent) n, " during the first year of life. There have been 200 known cases of interrupted aortic arch"?" up to now but only 15 of them were successfully treated surgically."?" The present case (Type A, Celoria and Patton's" classification) is reported because of the technical difficulties in surgical management. End-to-end anastomosis of the subclavian artery to the descending aorta was done to re-establish continuity of the aorta. Case report M. S. F., a 6-year-old Brazilian girl, was first seen in June, 1967, at the age of 15 months. At the time a history of frequent episodes of bronchitis was presented. Clinical, laboratory, and hemodynamic studies were done and the diagnosis of intraventricular From the Heart Institute of the University of Sao Paulo Medical School, Sao Paulo, Brazil. Address for reprints: Heart Institute, Caixa Postal 8091, Sao Paulo, Brazil. Received for publication May 25, 1973. • Assistant Professor, Department of Surgery. •• Fellow of Cardiac Surgery. ••• Assistant, Department of Medical Clinic. •••• Assistant Professor, Department of Medical Clinic. *****Professor of Surgery, Honorary Member of The American Association for Thoracic Surgery.
septal defect (IYSD) with patent ductus arteriosus and pulmonary hypertension was made. She underwent surgical Ireatment approximately three years later. The operation was limited to thoracotomy because the canal could not be closed. The aortic arch between the pulmonary artery and the descending aorta was completely interrupted. The case was reviewed and reoperation was proposed. At this time physical examination showed the child to be underdeveloped, acyanotic, without dyspnea, and in rather good general condition. The blood pressure in the upper limbs was 180/100 mm. Hg and in the lower limbs it was 120/60 mm. Hg. The heart rate was 92 beats per minute. On auscultation, the second sound in the pulmonary area was hyperphonetic, with the second component louder than first. An intense systolic murmur of regurgitation irradiating throughout the precordium and dorsum was audible in the mesocardium. The preoperative electrocardiogram (ECG) is shown in Fig. 1. The x-ray (Fig. 2) showed increased pulmonary vasculature, especially in the hilum. It was more developed in the apexes than in the bases. The cardiac area was increased at the expense of the right atrium and both ventricles. The pulmonary artery was obviously dilated. The results of the hemodynamic studies are shown in Table I. The IYSD, patent ductus arteriosus, and interrupted aortic arch were shown by cineangiocardiography. Fig. 3 shows diagrammatically the interruption of the aotic arch corresponding to Type A (Celoria and Patton") . The patient was operated upon in August, 1972. By means of a left posterolateral thoracotomy approach, the descending aorta, ductus, left sub-
653
65 4
Barbero Marcial et al.
The Journol of Thoracic and Card iovascular
Surg ery
Fig. 1. Preoperative ECG showing signs of biventricular overload.
Fig. 2. Preoperative chest x-ray show ing increased pulmonary circulation. ma inly hilar, a nd increased ca rdiac area due to left he art enl ar gement. The pulmonar y artery is also visibly enlarged . cla vian a rtery. and proxim al ao rta were dissected . In view of the impossibility of re-establi shin g co ntinui ty of the aorta by end-to-end anasto mosis, a nd becau se the left subcla vian showed a good ca liber, a n end -to-end ana stom osis between the latte r a nd the descending aorta was perf ormed. Th e ca na l was sectioned and sutured . The postoperative course was very sati sfactory and the patient is presently asymptomatic a nd in good genera l health. On ausc ultation, the second sound continues hyperphonetic in the pulmonary area with the second co mponent louder than the first. A systolic mu rmu r of med ium intensity in the mesocardium a nd an intense aspira ting diasto lic murm ur, mo re clea rly a udible in the seco nd left intercosta l spac e, a re presen t. The ECG in Fig. 4 sho ws o nly a decrease in the right ventricular o verlo ad . The x-ray (Fig. 5 ) shows a significant decr ease of the hea rt size. Th e results of the hem odyn am ic study a re show n in T abl e II . Lef t cinea ngioca rdio gra phy ( Fig. 6) shows the existenc e of a n IV defect with left-to-right shunt and with good contras t of the pulm on ary a rtery. I n Fig. 7 the end -to-e nd ana stomosis bet ween the left subclavia n and the descend ing ao rta is clearly visible.
Fig. 3. Schematic drawing showing an aortic arch inte rru ption of T ype A. The descendin g thoracic ao rta is communica ting with the pu lmonary arte ry through the patent d uctu s.
Discussion This is a very serious congenital anomaly; the average life span is 10 days, with a mortality rate of 80 per cent during the first month after birth." On the other hand. according to Van Praagh and associates ," 31 per cent of the deaths during the first month of life due to congenital card iovascular anom alies are caused by this type of malformaiton . The most common causes of dea th arc heart failure and pulmonary hypertension. In the present case , the fact that the pat ient lived to the age of six without serious symptoms attracted our attention. We know of only 15 patients successfull y operated upon.' :" It is hop ed that , as the diagnosis and technique improve, prognosis will be better. The surgical technique used may be pal-
Volume 66 Number 4 October. 1973
Correction of interrupted aortic arch
655
Fig. 4. Postoperative ECG showing diminished right ventricular overload.
Fig. 6. Left heart cineangiocardiography showing a termino-terrninal anastomosis between the left subclavian artery and the thoracic descending aorta.
Fig 5. Postoperative chest roentgenogram showing diminished cardiac area
liative or corrective. Palliation consists of placing a graft between the pulmonary artery and the descending aorta. Th is technique is used mainly in children in whom the ductus is closing and a graft cannot be placed in the ascending aorta. Corrective technique consists of placing the graft between the ascending and descending aorta; rarely is direct anastomosis between the two stumps possible. Finally, if the caliber of the subclavian artery is good , it may be anastomosed to the descending aorta. In the case presented, the latter technique was
Fig. 7. Left heart cineangiocardiography show ing a high ventricular septal defect and a good filling of the pulmonary artery.
used because of the long distance between the aortic stumps and the difficulty of getting close to the ascending aorta through the left posterolateral thoracotomy approach. The hemod ynamic study done three months
656
The Journal of Thorocic ond Cardiovascular Surgery
Barbero Marcial et al.
Table I. Preoperative catheterization»
Chambers Right ventricle Pulmonary artery Left ventricle Aorta (asc.) Aorta (desc.)
Systolic pressure
Initial diastolic pressure
End-diastolic pressure
Mean pressure
(mm, Hg]
(mm. Hg)
(mm, Hg)
(mm. Hg)
67 57 100 110 50
2.5 42 3.5 66 41
9 11
43 52.5 94 94 49
'Conclusion: Pulmonary artery hypertension; significant gradient, ascending aorta-descending aorta.
Table II. Postoperative catheterization"
Chambers Right atrium Right ventricle Pulmonary artery Wedge pressure Left ventricle Aorta (asc.) Aorta (desc.)
Systolic pressure
Initial diastolic pressure
End-diastolic pressure
Mean pressure
(mm. Hg)
(mm, Hg)
(mm. Hg)
(mm. Hg)
4.2 123 123
-10 95
9.8
137 137 89.4
-10 58.8 60.2
9.8
70 12 102.2 79.8
*Conc1usion: Right ventricular hypertension; pulmonary artery hypertension; significant gradient. ascending aorta-descending aorta.
postoperatively showed the presence of an A-V shunt (Op/Os = 2: 1) because of the IV defect, moderate pulmonary hyperresistance (Rp/Rs = 0.27) (Ni = 0.08), and moderate gradient across the subclavian artery. The good postoperative result was confirmed by the improvement of the ECG and x-ray. The IV defect will be corrected at the time of six-month follow-up.
REFERENCES
2
3
Summary
The case of a 6-year-old girl with interruption of the aortic arch (Type A, Celoria and Patton classification), ventricular septal defect, and patent ductus arteriosus is presented. Continuity of the aorta was established by end-to-end anastomosis of the subclavian artery and descending aorta. Progress was satisfactory. The fact that the patient lived to the age of six without serious symptoms is worth emphasizing, as is also the surgical technique used.
4
5
6
7
Barratt-Boyes, B. G., Nicholls, T. T., Brandt, P. W. T., and Neutze, J. M.: Aortic Arch Interruption Associated With Patent Ductus Arteriosus, Ventricular Septal Defect, and Total Anomalous Pulmonary Venous Connection, J. THoRAc. CARDIOVASC. SURG. 63: 367, 1972. Blake, H. A., Manion, W. c., and Spencer, F. C.: Atresia or Absence of the Aortic Isthmus, J. THoRAc. CARDIOVASC. SURG. 43: 607, 1962. Celoria, G. c., and Patton, R. B.: Congenital Absence of the Aortic Arch, Am. Heart J. 58: 407, 1959. Litwin, S. B., Van Praagh, R., and Bernhard, W. F.: A Palliative Operation for Certain Infants With Aortic Arch Interruption, Ann. Thorac. Surg. 14: 369, 1972. Pillsbury, R. C., Lower, R. R., and Shumway, N. E.: Atresia of the Aortic Arch, Circulation 30: 749, 1972. Rochette, M., Stanley, P., Ethier, M. F., and Davignon, A.: Complete Interruption of the Aortic Arch in Infancy, Can. Med. Assoc. J. 98: 131, 1968. Tawes, R. L., Jr., Panagopoulos, P., Aberdeen, E., Waterston, D. J., Bonham-Carter, R. E.,
Volume 66 Number 4
Correction of interrupted aortic arch
657
October, 1973
8
9
10
11
and Stephens, H. B.: Aortic Arch Atresia and Interruption of the Aortic Arch, J. THORAC. CARDIOVASC. SURG. 58: 492, 1969. Tyson, K. R. T., Harris, L. C., and Nghiern, Q. X.: Repair of the Aortic Arch Interruption in the Neonate, Surgery 67: 1006, 1970. Van Praagh, R., Bernhard, W. F., Rosenthal, A., Parisi, L., and Fyler, D. c.: Interrupted Aotic Arch; Surgical Treatment, Am. J. Cardial. 27: 201,1971. Kinsley, R. H., Utian, N. C., Fuller, D. N., and Marchand, P. E.: Interruption of the Aortic Arch, Thorax 27: 93, 1972. Evans, W.: Congenital Stenosis (Coarctation),
Atresia and Interruption of the Aortic Arch (a Study of 28 Cases), Quart. J. Med. 26: 1, 1933. 12 Abbott, M. E.: Atlas of Congenital Cardiac Diseases, New York, 1936, American Heart Association, Publisher, Chart I. 12 Hamburger, L. P.: Congenital Cardiac Malformation Presenting Complete Interruption of the Isthmus Aortas With Transposition of the Great Arteries, Bull. Johns Hopkins Hosp. 61: 421, 1937. 14 Sewart, M.: Congenital Interruption of the Aortic Arch, Arch. Dis. Child. 76: 326, 1948.