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Total one-stage repair of interrupted aortic arch associated with aortic septal defect and patent ductus arteriosus
Total one-stage repair of interrupted aortic arch associated with aortic septal defect and patent ductus arteriosus Successful total repair in one stage was performed in a 3-year-old girl who had interrupted aortic arch associated with aortic septal defect and patent ductus arteriosus. Surface-induced deep hypothermia and interrupted perfusion were used. The results of postoperative catheterization and angiocardiographic studies are analyzed, and the literature and results of previous surgical attempts at correction are reviewed.
Kenji Ito, M.D., Naoaki Kohguchi, M.D., Yasunori Ohkawa, M.D., Tadayoshi Akasaka, M.D., Hiroshi Ohara, M.D., MasashiTakarada, M.D., Hiroshi Aoki, M.D., Minoru Ogata, M.D., Makoto Nishibatake, M.D., Osamu Fukatsu, M.D., and Kazuo Matsushima, M.D.,
Yokohama, Japan
T
he complex malformation of interrupted aortic arch associated with aortic septal defect and patent ductus arteriosus is a rare condition that seldom has been repaired successfully .t-3 After having repaired this anomaly in a 3-year-old girl, we reviewed the literature on this defect and evaluated recent progress in treatment.
Case report F. S., a Japanese girl, was first seen at our medical center at the age of I month. Relevant features of her past history were that she was born by cesarean section at the gestational age of 38 weeks. Her birth weight was 2,300 grams. At I month of age, she was found to have a cardiac murmur. During infancy her ability to suck milk was poor. Cardiac catheterization when she was I month old led to a diagnosis of interruption of the aorta with a patent ductus supplying the descending aorta and the probability of a ventricular septal defect (Table I). Her growth rate lagged noticeably, and her exercise tolerance was limited severely. At the second evaluation, when she was 3 years old, the patient was thin and small for her age. The pulse rate was 90 beats per minute. The peripheral pulse was not bounding. Blood pressure was 110 mm. Hg both in the upper and the lower extremities. There was no From Kanagawa Children's Medical Center, Yokohama, Japan. Received for publication Jan. 19, 1977. Accepted for publication March 21, 1977. Address for reprints: Kenji Ito, M.D., Kanagawa Children's Medical Center, Mutsukawa 2-138-4, Minami-Ku, Yokohama, Japan.
differential cyanosis. A systolic murmur with a thrill was present along the upper right sternal border. There was increased splitting of the second sound. The liver was palpable two fingerbredths below the costal margin. Laboratory studies revealed no abnormalities. Chest roentgenograms showed moderate cardiomegaly and moderately increased pulmonary vascular markings. The electrocardiogram demonstrated regular sinus rhythm, normal electrical axis, counterclockwise rotation, and biventricular hypertrophy. Repeat cardiac catheterization revealed pulmonary hypertension (pulmonary pressure being almost equal to systemic pressure), a left-to-right shunt at the ventricular level, and a right-to-Ieft shunt from the main pulmonary artery to the descending aorta through the patent ductus. There was a persistent left superior vena cava (Table I). Angiocardiographic studies demonstrated Type A interruption of the aortic arch, (Celoria and Patton's" categorization), a large patent ductus arteriosus supplying the descending aorta, and an aortic septal defect (Figs. I and 2). Operation. The child was cooled to a esophageal temperature of 31 C. by immersion in ice water. A thoracotomy was performed through the third intercostal space on the left, and the anatomy of the arch interruption was confirmed. The ductus and descending aorta were dissected and encircled with a tape, and then the left side of the thorax was closed. The child was turned on her back and cooled further to an esophageal temperature of 21 C. The heart was exposed through a sternum-splitting incision. The 15 mm. aortic septal defect was closed with direct continuous over-and-over sutures through the aortotomy with the aid of circulatory arrest. After the aortotomy was closed, the ascending aorta, right external iliac artery, and right atrial appendage were 0
0
9I3
9 14
The Journal of Thoracic and Cardiovascular Surgery
Ito et al ,
Fig. 1. Preoperative angiocardiogram in the lateral projection shows large patent ductus arteriosus supplying the descending aorta. cannulated . The descending aorta was temporarily occluded after air was evacuated from the aorta, and perfusion was commenced after a 60 minute period of circulatory arrest. Following 20 minutes of perfusion, which lowered the temperature to 19° c., the perfusion was stopped for 33 minutes. The proximal end of a 10 mm. woven Dacron graft was anastomosed to a vertical incision in the ascending aorta, extending to the origin of innominate artery. Then air was evacuated from the aorta, and rewarming perfusion was begun . Sinus rhythm returned spontaneously, and the heart maintained an adequate circulation after 53 minutes of perfusion . Again the left side of the thorax was opened, and the graft was passed downward through a hole in the pericardium. The ductus was divided and its pulmonary end was oversewn. The end-to-end anastomosis between the distal end of the graft and descending aorta was performed, and aortic reconstruction was completed . In the postoperative period, the child required the infusion of isoproterenol, dopamine, and/or epinephrine for 7 days. Excess postoperative bleeding was controlled by conservative measures . The patient also required 7 days of respiratory assistance with nasotracheal intubation and finally was extubated on the eleventh postoperative day. She had two episodes of seizures. During the second seizure, she had
Fig. 2. Preoperative aortogram in the frontal projection reveals that the ascending aorta and the main pulmonary artery are opacified simultaneously, which indicates the presence of an aortic septal defect. The descending aorta is not opacified, which indicates that the aortic arch is interrupted just beyond the origin of the left subclavian artery . marked bradycardia, hypotension, and impending cardiac arrest, but she was resuscitated successfully after a short period of external cardiac massage. One month postoperatively, cardiac catheterization and angiocardiographic studies were performed . There were no shunts . The pulmonary artery pressure had decreased, but a slight pressure gradient between the main pulmonary artery and right ventricle was detected (Table I). Patency of the graft was confirmed (Fig. 3). Blood pressure in the upper and lower extremities was 128 and 114 mm. Hg, respectively . The child was discharged 34 days after operation .
Discussion Almost any cardiovascular malformation can coexist with interruption of the aortic arch ." Ventricular septal defect is very common although not always present. 1-3. 6. 7. 10-12. 14 The association of aortopulmonary fenestration is uncommon'<'- 6 . 8-14 but, when present, the communication can be very large owing to total absence of the aortopulmonary septum ." Without surgery, the mortality rate from the presence of an interrupted aortic arch in the neonatal period is more than 90 percent. 5 Natural survival be-
Volume 74
Interrupted aortic arch
Number 6 December, 1977
9I5
Table I. Cardiac catheterization data Preoperative (3/16/73) Site
Pressure (mm. Hg)
Lsve Rsve
(%)
Pressure (mm. Hg)
I
Oxygen sat. (%)
Postoperative (11/5/76) Pressure (mm. Hg)
51.8
rvc
RA RV MPA LPA LA PDA Desc. Ao. FA
I
Oxygen sat.
Preoperative (9/1 /76)
(4)* 70/0 70/20(40) 10/2 (7) 70/35 70/35 (SO)
46.0 66.4 68.4 71.4
(4) 83/10 85/63 (73)
65.2 63.6 57.2 64.1 75.9
(4) 60/2 43/22 45/20(25)
I
Oxygen sat. (%)
54.6 58.4 66.3 61.2 59.0 54.6 54.6
89.7 76.4 74.2
95.7
Legend: LSye, Left superior vena cava. RSye, Right superior vena cava. lye, Inferior vena cava. RA, Right atrium. RY, Right ventricle. MPA, Main pulmonary
artery. LPA, Left pulmonary artery. LA, Left atrium. PDA, Patent ductus arteriosus. Desc. Ao., Descending aorta. FA, Femoral artery. 'Figures in parentheses are mean values.
yond infancy is related to the extent of the obstruction to blood flow through a segment of the aorta and associated anomalies. When aortic arch interruption is associated with aortic septal defect, the prognosis is thought to be somewhat more favorable than when it is associated with ventricular septal defect. To date, 14 cases of aortic arch interruption associated with aortic septal defect have been reported. In four of these 14 cases, total repair was attempted: twostage repair in two': 2 and in one-stage repair in two." All four of the patients were 2 years of age or older at the time of operation and all survived (Table II). The ideal management of an interrupted aortic arch consists of one-stage correction of the arch anomaly and the associated cardiac defects. This is now possible with the use of cardiopulmonary bypass, profound hypothermia, and circulatory arrest. However, whether one-stage repair or staged correction of the arch and associated anomalies is preferrable remains to be shown by results in a large number of cases. The question of which method of aortic arch reconstruction is preferable has caused a great deal of controversy. A direct anastomosis between the aortic segments or between the aorta and its major brachiocephalic vessels is more likely to result in a satisfactory lumen over the long term than is reconstruction of the arch with prosthetic material or a vascular homograft, because there is a chance for the channel to increase in size with the patient's growth. t5 However, direct anastomosis in interruption of the aorta is much more difficult technically, because the distance between the proximal and distal aortic segments is
Fig. 3. Postoperative angiocardiogram in the frontal projection demonstrates that the reconstructed aortic arch is patent. There is no reopacification of the main pulmonary artery.
usually great. On the other hand, when the left subclavian artery or the left common carotid artery is used to bridge the gap between the aortic segments, the lumen is not wide enough, and division or ligation of the ductus arteriosus results in left heart failure and unfavorable results. 16 The prognosis depends on the size of the conduit that is constructed. Therefore, the conduit should measure at least 4 mm.P In the infant, a prosthetic graft is usually required to bridge, since adequate autogenous tissue is not available. Future replacement of the bypass graft probably will be required as the child grows.
9 16
The Journal of Thoracic and Cardiovascular Surgery
Ito et al.
Table II. Reported cases of successful total repair of interrupted aortic arch associated with aortic septal defect Type of Ref. No.
1969
15
interruption"
Surgical procedures
Type A
First stage: Division of ductus; recon-
M
2
1975
18
M
Type A
3
1975
2
M
Type A
3
1976
8
M
Type A
Present case
1976
3
F
Type A
Another problem is that, in infants and small children, the ascending aorta and abbreviated aortic arch are very small, often too small to permit the side clamping necessary for direct anastomosis? or graft reconstruction. These procedures may be accomplished easily and safely by use of circulatory arrest with deep hypothermia. The technical problems with one-stage total repair in this anomaly are as follows: (1) how to control the large aortopulmonary shunt to protect the lungs before instituting cardiopulmonary bypass and (2) how to protect the organs supplied by the descending aorta, especially the kidney, during reconstruction of aortic continuity. To overcome these difficulties, we used surface-induced deep hypothermia for closure of the aortic septal defect. We then used limited cardiopulmonary bypass, with split inflow lines (i.e., ascending aorta and external iliac artery), for reconstruction of aortic continuity. The reversibility of pulmonary hypertension after total repair of this complex anomaly remains uncertain, since there have been no successful repairs during infancy. Marked residual pulmonary hypertension, with the pressure almost at the systemic level, was detected in all surviving patients who were more than 2 years of age" 3 except for our own patient. Much longer follow-up periods and much larger series are necessary to allow definite conclusions.
struction of aortic arch with prosthetic graft Second stage: Repairof aortic septal defect First stage: Division of ductus; reconstruction of aortic arch with prosthetic graft Second stage: Repair of aortopulmonary septal absence One-stage total repair: Repair of aortic septal defect; Blalock-Park anastomosis; division of ductus One-stage total repair: Repair of aortic septal defect; reconstruction of aortic arch with prosthetic graft; division of ductus One-stage total repair: Repair of aortic septal defect; reconstruction of aortic arch with prosthetic graft; division of ductus
REFERENCES
2
3 4 5
6
7
8 9 10
II
Sissman, N.: Personal communication, 1969. Cited from Reference 5. Daily, P.O., Sissman, N. J., Lipton, M. J., and Shumway, N. E.: Correction of Absence of the Aortopulmonary Septum by Creation of Concentric Great Vessels, Ann. Thorac. Surg. 19: 180, 1975. Ninomiya, 1., Tsunemoto, M., et al.: Personal communication, 1976. Celoria, G. C.; and Patton, R. B.: Congenital Absenceof the Aortic Arch, Am. Heart J. 58: 407, 1959. Van Praagh, R., Bernhard, F., Rosenthal, A., Parisi, L. F., and Fyler, D. c.: Interrupted Aortic Arch: Surgical Treatment, Am. 1. Cardiol. 27: 200, 1971. Blake, H. A., Manion, W. C.; and Spencer, F. C.; Atresia or Absence of the Aortic Isthmus, J. THORAC. CARDIOVASC. SURG. 43: 607, 1962. . Rochette, M., Stanley, P., Fethier, M., and Davignon, A.: Complete Interruption of the Aortic Arch in Infancy, Can. Med. Assoc. J. 98: 131, 1968. Mehrizi, A., and Morrish, H. F.: Interruption of the Aortic Arch, John Hopkins Med. J. Ill: 127, 1962. Watson, H.: Paediatric Cardiology, St. Louis, 1968, The C. V. Mosby Company, p. 224. Ohmori, K., Osano, M., Yashiro, K., et aI.: Four autopsy Cases of Interrupted Aortic Arch, Shohnika Rinsho. 22: 775, 1969 (in Japanese). Chiemmongkoltip, P., Moulder, P. U., and Cassels, D. E.: Interruption of the Aortic Arch With Aorticopulmonary Septal Defect amd Intact Ventricular Septum in a Teenage Girl, Chest 60: 324, 1971.
Volume 74 Number 6 December, 1977
12 Blieden, L. c., and Moller, J. H.: Aorticopulmonary Septal Defect: An Experience With 17 Patients, Br. Heart J. 36: 630, 1974. 13 Rosenquist, G. L., Taylor, J. F. N., and Stark, J.: Aortopulmonary Fenestration and Aortic Atresia: Report of an Infant With Ventricular Septal Defect, Persistent Ductus Arteriosus, and Interrupted Aortic Arch, Br. Heart J. 36: 1146, 1974. 14 Freedom, R. M., Bain, H., and Rowe, R. D.: A Morphologic Study of Ventricular Septal Defect (VSD) and Subaortic Stenosis in Interruption of Aortic Arch (lAA), Circulation 56: 170, 1976 (Supp!. II).
Interrupted aortic arch
9 17
15 Tawes, R. L., Jr., Aberdeen, E., and Berry, C. L.: The Growth of an Aortic Anastomosis: An Experimental Study in Piglets, Surgery 64: 1122, 1968. 16 Singh, M. P., Bentall, H. H., and Oakley, C. M.: Successful Total Correction of Congenital Interruption of the Aortic Arch and Ventricular Septal Defect, Thorax 25: 615, 1970. 17 Fishman, N. H., Bronstein, M. H., Berman, W., Jr., Roe, B. B., Edmunds, L. H., Jr., Robinson, S. J., and Rudolph, A. M.: Surgical Management of Severe Aortic Coarctation and Interrupted Aortic Arch in Neonates, J. THORAC. CARDIOVASC. SURG. 71: 35, 1976.
Kenji Ito, M.D., Naoaki Kohguchi, M.D., Yasunori Ohkawa, M.D., Tadayoshi Akasaka, M.D., Hiroshi Ohara, M.D., MasashiTakarada, M.D., Hiroshi Aoki, M.D., Minoru Ogata, M.D., Makoto Nishibatake, M.D., Osamu Fukatsu, M.D., and Kazuo Matsushima, M.D.,
Yokohama, Japan
T
he complex malformation of interrupted aortic arch associated with aortic septal defect and patent ductus arteriosus is a rare condition that seldom has been repaired successfully .t-3 After having repaired this anomaly in a 3-year-old girl, we reviewed the literature on this defect and evaluated recent progress in treatment.
Case report F. S., a Japanese girl, was first seen at our medical center at the age of I month. Relevant features of her past history were that she was born by cesarean section at the gestational age of 38 weeks. Her birth weight was 2,300 grams. At I month of age, she was found to have a cardiac murmur. During infancy her ability to suck milk was poor. Cardiac catheterization when she was I month old led to a diagnosis of interruption of the aorta with a patent ductus supplying the descending aorta and the probability of a ventricular septal defect (Table I). Her growth rate lagged noticeably, and her exercise tolerance was limited severely. At the second evaluation, when she was 3 years old, the patient was thin and small for her age. The pulse rate was 90 beats per minute. The peripheral pulse was not bounding. Blood pressure was 110 mm. Hg both in the upper and the lower extremities. There was no From Kanagawa Children's Medical Center, Yokohama, Japan. Received for publication Jan. 19, 1977. Accepted for publication March 21, 1977. Address for reprints: Kenji Ito, M.D., Kanagawa Children's Medical Center, Mutsukawa 2-138-4, Minami-Ku, Yokohama, Japan.
differential cyanosis. A systolic murmur with a thrill was present along the upper right sternal border. There was increased splitting of the second sound. The liver was palpable two fingerbredths below the costal margin. Laboratory studies revealed no abnormalities. Chest roentgenograms showed moderate cardiomegaly and moderately increased pulmonary vascular markings. The electrocardiogram demonstrated regular sinus rhythm, normal electrical axis, counterclockwise rotation, and biventricular hypertrophy. Repeat cardiac catheterization revealed pulmonary hypertension (pulmonary pressure being almost equal to systemic pressure), a left-to-right shunt at the ventricular level, and a right-to-Ieft shunt from the main pulmonary artery to the descending aorta through the patent ductus. There was a persistent left superior vena cava (Table I). Angiocardiographic studies demonstrated Type A interruption of the aortic arch, (Celoria and Patton's" categorization), a large patent ductus arteriosus supplying the descending aorta, and an aortic septal defect (Figs. I and 2). Operation. The child was cooled to a esophageal temperature of 31 C. by immersion in ice water. A thoracotomy was performed through the third intercostal space on the left, and the anatomy of the arch interruption was confirmed. The ductus and descending aorta were dissected and encircled with a tape, and then the left side of the thorax was closed. The child was turned on her back and cooled further to an esophageal temperature of 21 C. The heart was exposed through a sternum-splitting incision. The 15 mm. aortic septal defect was closed with direct continuous over-and-over sutures through the aortotomy with the aid of circulatory arrest. After the aortotomy was closed, the ascending aorta, right external iliac artery, and right atrial appendage were 0
0
9I3
9 14
The Journal of Thoracic and Cardiovascular Surgery
Ito et al ,
Fig. 1. Preoperative angiocardiogram in the lateral projection shows large patent ductus arteriosus supplying the descending aorta. cannulated . The descending aorta was temporarily occluded after air was evacuated from the aorta, and perfusion was commenced after a 60 minute period of circulatory arrest. Following 20 minutes of perfusion, which lowered the temperature to 19° c., the perfusion was stopped for 33 minutes. The proximal end of a 10 mm. woven Dacron graft was anastomosed to a vertical incision in the ascending aorta, extending to the origin of innominate artery. Then air was evacuated from the aorta, and rewarming perfusion was begun . Sinus rhythm returned spontaneously, and the heart maintained an adequate circulation after 53 minutes of perfusion . Again the left side of the thorax was opened, and the graft was passed downward through a hole in the pericardium. The ductus was divided and its pulmonary end was oversewn. The end-to-end anastomosis between the distal end of the graft and descending aorta was performed, and aortic reconstruction was completed . In the postoperative period, the child required the infusion of isoproterenol, dopamine, and/or epinephrine for 7 days. Excess postoperative bleeding was controlled by conservative measures . The patient also required 7 days of respiratory assistance with nasotracheal intubation and finally was extubated on the eleventh postoperative day. She had two episodes of seizures. During the second seizure, she had
Fig. 2. Preoperative aortogram in the frontal projection reveals that the ascending aorta and the main pulmonary artery are opacified simultaneously, which indicates the presence of an aortic septal defect. The descending aorta is not opacified, which indicates that the aortic arch is interrupted just beyond the origin of the left subclavian artery . marked bradycardia, hypotension, and impending cardiac arrest, but she was resuscitated successfully after a short period of external cardiac massage. One month postoperatively, cardiac catheterization and angiocardiographic studies were performed . There were no shunts . The pulmonary artery pressure had decreased, but a slight pressure gradient between the main pulmonary artery and right ventricle was detected (Table I). Patency of the graft was confirmed (Fig. 3). Blood pressure in the upper and lower extremities was 128 and 114 mm. Hg, respectively . The child was discharged 34 days after operation .
Discussion Almost any cardiovascular malformation can coexist with interruption of the aortic arch ." Ventricular septal defect is very common although not always present. 1-3. 6. 7. 10-12. 14 The association of aortopulmonary fenestration is uncommon'<'- 6 . 8-14 but, when present, the communication can be very large owing to total absence of the aortopulmonary septum ." Without surgery, the mortality rate from the presence of an interrupted aortic arch in the neonatal period is more than 90 percent. 5 Natural survival be-
Volume 74
Interrupted aortic arch
Number 6 December, 1977
9I5
Table I. Cardiac catheterization data Preoperative (3/16/73) Site
Pressure (mm. Hg)
Lsve Rsve
(%)
Pressure (mm. Hg)
I
Oxygen sat. (%)
Postoperative (11/5/76) Pressure (mm. Hg)
51.8
rvc
RA RV MPA LPA LA PDA Desc. Ao. FA
I
Oxygen sat.
Preoperative (9/1 /76)
(4)* 70/0 70/20(40) 10/2 (7) 70/35 70/35 (SO)
46.0 66.4 68.4 71.4
(4) 83/10 85/63 (73)
65.2 63.6 57.2 64.1 75.9
(4) 60/2 43/22 45/20(25)
I
Oxygen sat. (%)
54.6 58.4 66.3 61.2 59.0 54.6 54.6
89.7 76.4 74.2
95.7
Legend: LSye, Left superior vena cava. RSye, Right superior vena cava. lye, Inferior vena cava. RA, Right atrium. RY, Right ventricle. MPA, Main pulmonary
artery. LPA, Left pulmonary artery. LA, Left atrium. PDA, Patent ductus arteriosus. Desc. Ao., Descending aorta. FA, Femoral artery. 'Figures in parentheses are mean values.
yond infancy is related to the extent of the obstruction to blood flow through a segment of the aorta and associated anomalies. When aortic arch interruption is associated with aortic septal defect, the prognosis is thought to be somewhat more favorable than when it is associated with ventricular septal defect. To date, 14 cases of aortic arch interruption associated with aortic septal defect have been reported. In four of these 14 cases, total repair was attempted: twostage repair in two': 2 and in one-stage repair in two." All four of the patients were 2 years of age or older at the time of operation and all survived (Table II). The ideal management of an interrupted aortic arch consists of one-stage correction of the arch anomaly and the associated cardiac defects. This is now possible with the use of cardiopulmonary bypass, profound hypothermia, and circulatory arrest. However, whether one-stage repair or staged correction of the arch and associated anomalies is preferrable remains to be shown by results in a large number of cases. The question of which method of aortic arch reconstruction is preferable has caused a great deal of controversy. A direct anastomosis between the aortic segments or between the aorta and its major brachiocephalic vessels is more likely to result in a satisfactory lumen over the long term than is reconstruction of the arch with prosthetic material or a vascular homograft, because there is a chance for the channel to increase in size with the patient's growth. t5 However, direct anastomosis in interruption of the aorta is much more difficult technically, because the distance between the proximal and distal aortic segments is
Fig. 3. Postoperative angiocardiogram in the frontal projection demonstrates that the reconstructed aortic arch is patent. There is no reopacification of the main pulmonary artery.
usually great. On the other hand, when the left subclavian artery or the left common carotid artery is used to bridge the gap between the aortic segments, the lumen is not wide enough, and division or ligation of the ductus arteriosus results in left heart failure and unfavorable results. 16 The prognosis depends on the size of the conduit that is constructed. Therefore, the conduit should measure at least 4 mm.P In the infant, a prosthetic graft is usually required to bridge, since adequate autogenous tissue is not available. Future replacement of the bypass graft probably will be required as the child grows.
9 16
The Journal of Thoracic and Cardiovascular Surgery
Ito et al.
Table II. Reported cases of successful total repair of interrupted aortic arch associated with aortic septal defect Type of Ref. No.
1969
15
interruption"
Surgical procedures
Type A
First stage: Division of ductus; recon-
M
2
1975
18
M
Type A
3
1975
2
M
Type A
3
1976
8
M
Type A
Present case
1976
3
F
Type A
Another problem is that, in infants and small children, the ascending aorta and abbreviated aortic arch are very small, often too small to permit the side clamping necessary for direct anastomosis? or graft reconstruction. These procedures may be accomplished easily and safely by use of circulatory arrest with deep hypothermia. The technical problems with one-stage total repair in this anomaly are as follows: (1) how to control the large aortopulmonary shunt to protect the lungs before instituting cardiopulmonary bypass and (2) how to protect the organs supplied by the descending aorta, especially the kidney, during reconstruction of aortic continuity. To overcome these difficulties, we used surface-induced deep hypothermia for closure of the aortic septal defect. We then used limited cardiopulmonary bypass, with split inflow lines (i.e., ascending aorta and external iliac artery), for reconstruction of aortic continuity. The reversibility of pulmonary hypertension after total repair of this complex anomaly remains uncertain, since there have been no successful repairs during infancy. Marked residual pulmonary hypertension, with the pressure almost at the systemic level, was detected in all surviving patients who were more than 2 years of age" 3 except for our own patient. Much longer follow-up periods and much larger series are necessary to allow definite conclusions.
struction of aortic arch with prosthetic graft Second stage: Repairof aortic septal defect First stage: Division of ductus; reconstruction of aortic arch with prosthetic graft Second stage: Repair of aortopulmonary septal absence One-stage total repair: Repair of aortic septal defect; Blalock-Park anastomosis; division of ductus One-stage total repair: Repair of aortic septal defect; reconstruction of aortic arch with prosthetic graft; division of ductus One-stage total repair: Repair of aortic septal defect; reconstruction of aortic arch with prosthetic graft; division of ductus
REFERENCES
2
3 4 5
6
7
8 9 10
II
Sissman, N.: Personal communication, 1969. Cited from Reference 5. Daily, P.O., Sissman, N. J., Lipton, M. J., and Shumway, N. E.: Correction of Absence of the Aortopulmonary Septum by Creation of Concentric Great Vessels, Ann. Thorac. Surg. 19: 180, 1975. Ninomiya, 1., Tsunemoto, M., et al.: Personal communication, 1976. Celoria, G. C.; and Patton, R. B.: Congenital Absenceof the Aortic Arch, Am. Heart J. 58: 407, 1959. Van Praagh, R., Bernhard, F., Rosenthal, A., Parisi, L. F., and Fyler, D. c.: Interrupted Aortic Arch: Surgical Treatment, Am. 1. Cardiol. 27: 200, 1971. Blake, H. A., Manion, W. C.; and Spencer, F. C.; Atresia or Absence of the Aortic Isthmus, J. THORAC. CARDIOVASC. SURG. 43: 607, 1962. . Rochette, M., Stanley, P., Fethier, M., and Davignon, A.: Complete Interruption of the Aortic Arch in Infancy, Can. Med. Assoc. J. 98: 131, 1968. Mehrizi, A., and Morrish, H. F.: Interruption of the Aortic Arch, John Hopkins Med. J. Ill: 127, 1962. Watson, H.: Paediatric Cardiology, St. Louis, 1968, The C. V. Mosby Company, p. 224. Ohmori, K., Osano, M., Yashiro, K., et aI.: Four autopsy Cases of Interrupted Aortic Arch, Shohnika Rinsho. 22: 775, 1969 (in Japanese). Chiemmongkoltip, P., Moulder, P. U., and Cassels, D. E.: Interruption of the Aortic Arch With Aorticopulmonary Septal Defect amd Intact Ventricular Septum in a Teenage Girl, Chest 60: 324, 1971.
Volume 74 Number 6 December, 1977
12 Blieden, L. c., and Moller, J. H.: Aorticopulmonary Septal Defect: An Experience With 17 Patients, Br. Heart J. 36: 630, 1974. 13 Rosenquist, G. L., Taylor, J. F. N., and Stark, J.: Aortopulmonary Fenestration and Aortic Atresia: Report of an Infant With Ventricular Septal Defect, Persistent Ductus Arteriosus, and Interrupted Aortic Arch, Br. Heart J. 36: 1146, 1974. 14 Freedom, R. M., Bain, H., and Rowe, R. D.: A Morphologic Study of Ventricular Septal Defect (VSD) and Subaortic Stenosis in Interruption of Aortic Arch (lAA), Circulation 56: 170, 1976 (Supp!. II).
Interrupted aortic arch
9 17
15 Tawes, R. L., Jr., Aberdeen, E., and Berry, C. L.: The Growth of an Aortic Anastomosis: An Experimental Study in Piglets, Surgery 64: 1122, 1968. 16 Singh, M. P., Bentall, H. H., and Oakley, C. M.: Successful Total Correction of Congenital Interruption of the Aortic Arch and Ventricular Septal Defect, Thorax 25: 615, 1970. 17 Fishman, N. H., Bronstein, M. H., Berman, W., Jr., Roe, B. B., Edmunds, L. H., Jr., Robinson, S. J., and Rudolph, A. M.: Surgical Management of Severe Aortic Coarctation and Interrupted Aortic Arch in Neonates, J. THORAC. CARDIOVASC. SURG. 71: 35, 1976.