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Correction of Type B interrupted aortic arch with ventricular septal defect in infancy
Correction of Type B interrupted aortic arch with ventricular septal defect in infancy The successful correction of a Type B interrupted aortic arch (fAA) with simultaneous closure of a ventricular septal defect (VSD) in a 6-month-old infant is described . With the use of profound hypothermia and limited cardiopulmonary bypass. a flap of pulmonary artery. together with the persistent ductus arteriosus (PDA ). was dissected fr ee and sutured so as to fo rm a tube which then was anastomosed to the ascending aorta . The ventricular septal defect and the pulmonary arteriotomy then were closed. Cardiac catheterization 5 months later demonstrated a satisf actory reconstruction with no gradient between the ascending and descending aorta.
J. L. Monro , F.R.C.S., W. Brawn, F.R.C.S ., and N. Conway, F.R.C .P ., Southampton , England
Because of the high early mortality rate in patients with interrupted aortic arch (IAA) ,l operation usually must be undertaken in infancy. Although early partial correction , with palliation and later complete correction, still is being practiced," it would seem that if total correction is feasible in infancy, this is likely to be more satisfactory. To our knowledge only three previous successful total corrections have been performed in infancy, and in two of them" : 4 grafts were used which almost certainl y will need replacing later. Trusler and Izukawa" successfully performed direct anastomosis of the ascending to the descending aorta and closed the ventricular septic defect (YSD) in a l3-day-old infant , but this may not always be possible without tension. To overcome the problem we have used a technique not described previously, in which a flap of pulmonary artery, together with the persistent ductus arteriosus (PDA), is used to form a tube , which then can be anastomosed to the ascending aorta without tension and thereby form a new aortic arch. Case report A 5-month-old infant presented with failure to thrive. She had been the term product of a normal deli very , and the From The Wessex Cardiac and Thoracic Centre, Southampton, En-
gland. Received for publication March 22, 1977. Accepted for publication May 18, 1977. Address for reprints: J. L. Monro, F.R.C.S., The Wessex Cardiac and Thoracic Centre, Southampton Western Hospital, Oakley Rd., Southampton S09 4WQ, England.
618
Fig. 1. A right ventricular inje ct ion shows filling of a very large pulmonary artery , the de scending thoracic aorta , and the left subclavian artery (arrow). mother's anten atal history had been uneventful. The infant' s birth weight was 2.9 kilograms, and at 5 month s it was 3.9 kilograms. No cyanoti c attacks had been noticed . On examination she was an undernourished , tachypneic infant with normal color, without clubbing, and with no evidence of differential cyanosis . She was in heart failure with moderate hepatomegaly . There was a right ventricular praecordial lift . On auscultation an ejection systolic murmur was heard at the left stern al edge , unaccompanied by a thrill. The pulmonary second sound moved normall y and was accentuated . In the mitral area, a low-pitched, rumbling , delayed-onset d iastolic murmur was audible .
Volume 74 Number 4 October,19n
Interrupted aortic arch with VSD
6 I9
Fig. 2. A, An aortogram shows a Type B interruption of the aortic arch in this early filling phase . B, A later phase of the same injection shows retrograde filling of the left subclavian artery by the vertebral artery (arrow) .
Fig. 3. This photograph shows the preoperative situation. See Fig. 5, A for comparison . Chest radiograms revealed an enlarged heart with marked pulmonary plethora . The electrocardiogram showed sinus rhythm at a rate of 150 beats per minute, with P pulmonale, biventricular hypertrophy, and a mean frontal axis of minus 115 degrees . Blood biochemistry was unremarkable and hemoglobin was 13.4 Gm. per 100 ml. On admission she was being treated with Lanoxin (0.03 mg. twice daily) and furosemide (5 mg. daily). On March 20, 1976, with the use of general anesthesia and intermittent positive-pressure respiration , right heart catheterization was performed via the right saphenous vein . The pulmonary artery and PDA were entered and, through the duct, the descending aorta. The left atrium was reached via a patent foramen ovale, but the left ventricle was not entered.
There was a left-to-right shunt at the ventricular level with a pulmonary-to-systemic flow ratio of 2.3 : I, and the pulmonary artery pressure was at systemic level. Right ventricular angiograms showed an enlarged pulmonary artery, and a through duct to the descending aorta and the left subclavian artery was seen to fill from this injection (Fig. I). An aortogram (Fig. 2, A) showed complete interrupt ion of the aortic arch between the left carotid and left subclavian arteries (Type 8) .6 The left vertebral artery was seen filling retrogradely from above, and the left subclavian artery also was demonstrated from this route (Fig . 2, B) . Following cardiac catheterization, she remained in left ventricular failure, but operation was delayed because of gastroenteritis. Therefore, on April 27, 1976, total correction
The Journal of Thoracic and Cardiovascular Surgery
620 Monro, Brawn, Conway
40 38 36
.." c
~
34
°c
Nasopharyngeal Temperature
32 30
28 26 24 22 20 18 16 14 12 10
tst Period Total Arrest (71mins)
2
3
4
5
6
7
Time in Hours
Fig. 4. This graph shows the surface cooling, two periods of circulatory arrest, and bypass rewarming during the operation. was performed with the infant under profound hypothermia. At operation (Fig. 3) the diameter of the ascending aorta was 6 mm., the pulmonary artery 18 mm., the duct 6 mm., and the VSD II mm. The patient was surface cooled to 25° C. (Fig. 4), the sternum opened, and cardiac bypass commenced at 23° C. for 3 minutes, taking the temperature down to 17° C. The ascending aorta then was cross-clamped distal to the aortic cannula, and when the heart was empty, the superior and inferior venae cavae were snugged. The innominate artery, the left carotid artery, and the duct were cross-clamped. The duct, together with an anterior flap of pulmonary artery wall approximately 15 mm. in length, was resected from the remainder of the pulmonary artery. The new aortic arch then was fashioned by sewing the pulmonary artery tissue attached to the duct inferiorly so as to form a tube (Fig. 5). After the defect in the pulmonary artery had been closed by direct suture, the artery appeared adequate, with a diameter of 10 mm. An incision was made in the left side of the proximal aorta extending into the left common carotid artery, and to this the new aortic arch was anastomosed with 6-0 Prolene (Fig. 6). Following a further period of bypass, the patent foramen ovale was closed, and the ventricular septal defect was repaired through a right ventriculotomy with a Dacron patch. After air had been removed, bypass was recommenced and the baby was rewarmed to 32° C. at which time the heart took over easily in sinus rhythm, without the need for supportive therapy. Following the correction the systolic pressures in the ascending and descending aorta were equal at 100 mm. Hg. She was extubated half an hour after returning to the intensive care unit and was in sinus rhythm with a rate of 150 beats per minute. Seventeen hours later, a short burst of ventricular tachycardia developed, which responded immediately to lidocaine given intravenously. For several days she remained in mild left ventricular failure, for which she received Lasix
and Lanoxin. However, she was pink, active and feeding well. On May 8, she was discharged home but was readmitted urgently 2 days later with a supraventricular tachycardia of 300 beats per minute. This was difficult to control initially, but finally it responded to increased doses of Lanoxin and propranolol, and subsequently she was discharged on May 21. When reviewed on July 6, she was well, in sinus rhythm at 100 beats per minute, and weighed 6.6 kilograms. However, on auscultation a systolic murmur was noted at the left sternal edge. A possible diagnosis of a residual VSD was made, and therefore she was reinvestigated on Oct. 12, 1976. Cardiac catheterization demonstrated no evidence of a residual VSD, but there was a stenosis of the distal main pulmonary artery with a gradient of 53 mm. Hg across it. The systolic pressure in the proximal pulmonary artery was 68 mm. Hg, as compared with 80 mm. Hg in the aorta. The new aortic arch was of satisfactory caliber (Fig. 7, A and B), including that portion composed entirely of ductus tissue. A doubtfully significant gradient of 5 mm. Hg was seen on withdrawal from the ascending to descending aorta.
Discussion Although IAA is rare, it is one of the few conditions grouped under the name "hypoplastic left heart syndrome" in which surgery can be helpful. The three main types described by Celona and Patton" are shown in Fig. 8, together with the relative frequency of each type. I There are almost always associated anomalies of which PDA and VSD are the commonest; however, very rarely these can be absent.': 7 There is a very high early mortality rate in this condition, and about 80 percent of the patients die in the first month of life," with only 10 percent surviving the first year." Therefore, it is obvious that early operation is required if the natural history of the disease is to be altered, and perhaps we were fortunate that our patient had lived to 5 months of age before presenting for operation. Several authors have reported palliative and partially corrective surgical procedures.v 9 but if total correction is feasible, it would seem to be preferable. The first successful correction of IAA in infancy was reported by Barratt-Boyes and associates" in 1972. This was a Type A IAA, and a Dacron graft was used to restore aortic continuity, together with closure of the VSD and correction of intracardiac total anomalous pulmonary venous drainage. The patient was only 8 days old but probably will need a further operation to replace the Dacron graft. Murphy and associates" reported the first successful correction of Type B IAA in a 3-day-old patient. They used homograft vein from the patient's father to restore aortic continuity, but again this probably will need replacing later. Trusler and Izukawa" performed the only other previously reported
Volume 74 Number 4
Interrupted aortic arch with VSD
October, 1977
LeA
(b)
Fig. 5. This shows the stages in the fashioning of a new aortic arch. A, The preoperative situation (compare with Fig. 3). B, The single aortic and venous cannulas are in position and a flap of anterior main pulmonary artery wall has been dissected free so that the persistent ductus arteriosus (PDA) and descending aorta are free. C, The flap of pulmonary artery is then sewn into a tube and the pulmonary artery closed by direct suture. D, The final situation shows the new "aorta" sutured to the ascending aorta at the base of the left carotid artery and the right ventriculotomy. SVC, Superior vena cava. AO, Aorta. Inom, Innominate artery. LCA, Left carotid artery. PA, Main pulmonary artery. LA, Left atrial appendage. RA, Right atrium. RV, Right ventricle. PDA, Persistent ductus arteriosus. RPA, Right pulmonary artery. LPA, Left pulmonary artery.
Fig. 6. Postoperative appearance (compare Fig. 5, D). The forceps are pointing to the suture line in the pulmonary artery.
621
622
Monro, Brawn, Conway
The Journal of Thoracic and Cardiovascular Surgery
Fig. 7. The aortogram 5 months after operation shows a rather acute angle at the anastomosis but no significant narrowing. A, Anteroposterior view. B, Lateral view also shows the left subclavianartery arising distal to the site of the duct. without any need for later surgery, we formed a tube out of a flap of pulmonary artery and the PDA . This allowed a tension-free anastomosis across which there was no gradient at operation and an insignificant one at subsequent cardiac catheterization. Angiographic studies did not suggest any narrowing at the site of the duct , which suggests that its use for this purpose is satisfactory. Unfortunately, direct suture of the pulmonary arteriotomy subsequently proved to have caused a narrowing at the level of the distal main pulmonary artery, but this could have been avoided easily by insertion of a patch into the pulmonary artery, had the pulAscending monary artery not seemed adequate. Resection of this aorta lesion probably will be needed in later life. In the three previously reported cases just described, and in our own, profound hypothermia with circulatory Fig. 8. This diagram shows the different types of interrupted arrest was used and would seem to be the method of aortic arch described by Celoria and Patton," and their relachoice .:" However, it is unnecessary to use a separate tive frequency according to Van Praagh and co-workers. I arterial input for the ascending and descending aortas, as reported by Trusler and Izukawa." successful total correction in infancy . In their l3 -dayThis condition remains one of the most challenging old patient with Type B IAA, they detached the deto be encountered by the surgeon, but early total correcscending aorta from the pulmonary artery and, having tion can be achieved successfully, and the formation of a new aortic arch by using the PDA and a flap of the mobilized it, anastomosed it directly to the ascending aorta. It was hoped that this procedure would avoid any enlarged pulmonary artery is a previously unreported further operation, but reinvestigation demonstrated a method of producing a tension-free reconstruction that residual gradient of 20 mm . Hg across this anastomosis should grow with the patient. 5 months after operation, and it remains to be seen We are grateful to Mrs. S. Jacobs of the Department of whether further surgery will be required. Teaching Media, Southampton University, for her help with the ill ustrations. In an endeavor to perform a complete correction
Volume 74 Number 4 October, 1977
Interrupted aortic arch with VSD
REFERENCES
2
3
4
5
Van Praagh, R., Bernhard, W. F., Rosenthal, A., Parisi, L. F., and Fyler, D. c.: Interrupted Aortic Arch: Surgical Treatment, Am. J. Cardio!. 27: 200, 197I. Ventemiglia, R., Oglietti, J., Wukasch, D. c., Hallman, G. L., and Cooley, D. A.: Interruption of the Aortic Arch: Surgical Considerations, J. THORAC. CARDIOVASC. SURG. 72: 235, 1976. 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: Total Correction in an 8-Day-Old Infant by Means of Profound Hypothermia and Limited Cardiopulmonary Bypass, 1. THORAC. CARDIOVASC. SURG. 63: 367,1972. Murphy, D. A., Lemire, G. G., Tessler, I., and Dunn, G. L.: Correction of Type B Aortic Arch Interruption With Ventricular and Atrial Septal Defects in a Three-Day-Old Infant, J. THoRAc. CARDIOVASC. SURG. 65: 882, 1973. Trusler, G. A., and Izukawa, T.: Interrupted Aortic Arch
6 7
8
9
IO
623
and Ventricular Septal Defect: Direct Repair Through a Median Sternotomy Incision in a 13-Day-Old Infant, J. THoRAc. CARDIOVASC. SURG. 69: 126, 1975. Celoria, G. C., and Patton, R. B.: Congenital Absence of the Aortic Arch, Am. Heart J. 58: 407, 1959. Sharratt, G. P., Carson, P., and Sanderson, J. M.: Complete Interruption of Aortic Arch, Without Persistent Ductus Arteriosus in an Adult, Br. Heart J. 37: 221, 1975. Roberts, W. C., Morrow, A. G., and Braunwald, E.: Complete Interruption of the Aortic Arch, Circulation 26: 39, 1962. Murphy, A., Collins, G., and Dobell, A. R. c. Surgical Correction of Type A Congenital Aortic Arch Interruption, Ann. Thorae. Surg. 11: 593, 1971. Barratt-Boyes, B. G., Simpson, M., and Neutze, J. M.: Intracardiac Surgery in Neonates and Infants Using Deep Hypothermia With Surface Cooling and Limited Cardiopulmonary Bypass, Circulation 43: 25, 1971 (Supp!. I) .
J. L. Monro , F.R.C.S., W. Brawn, F.R.C.S ., and N. Conway, F.R.C .P ., Southampton , England
Because of the high early mortality rate in patients with interrupted aortic arch (IAA) ,l operation usually must be undertaken in infancy. Although early partial correction , with palliation and later complete correction, still is being practiced," it would seem that if total correction is feasible in infancy, this is likely to be more satisfactory. To our knowledge only three previous successful total corrections have been performed in infancy, and in two of them" : 4 grafts were used which almost certainl y will need replacing later. Trusler and Izukawa" successfully performed direct anastomosis of the ascending to the descending aorta and closed the ventricular septic defect (YSD) in a l3-day-old infant , but this may not always be possible without tension. To overcome the problem we have used a technique not described previously, in which a flap of pulmonary artery, together with the persistent ductus arteriosus (PDA), is used to form a tube , which then can be anastomosed to the ascending aorta without tension and thereby form a new aortic arch. Case report A 5-month-old infant presented with failure to thrive. She had been the term product of a normal deli very , and the From The Wessex Cardiac and Thoracic Centre, Southampton, En-
gland. Received for publication March 22, 1977. Accepted for publication May 18, 1977. Address for reprints: J. L. Monro, F.R.C.S., The Wessex Cardiac and Thoracic Centre, Southampton Western Hospital, Oakley Rd., Southampton S09 4WQ, England.
618
Fig. 1. A right ventricular inje ct ion shows filling of a very large pulmonary artery , the de scending thoracic aorta , and the left subclavian artery (arrow). mother's anten atal history had been uneventful. The infant' s birth weight was 2.9 kilograms, and at 5 month s it was 3.9 kilograms. No cyanoti c attacks had been noticed . On examination she was an undernourished , tachypneic infant with normal color, without clubbing, and with no evidence of differential cyanosis . She was in heart failure with moderate hepatomegaly . There was a right ventricular praecordial lift . On auscultation an ejection systolic murmur was heard at the left stern al edge , unaccompanied by a thrill. The pulmonary second sound moved normall y and was accentuated . In the mitral area, a low-pitched, rumbling , delayed-onset d iastolic murmur was audible .
Volume 74 Number 4 October,19n
Interrupted aortic arch with VSD
6 I9
Fig. 2. A, An aortogram shows a Type B interruption of the aortic arch in this early filling phase . B, A later phase of the same injection shows retrograde filling of the left subclavian artery by the vertebral artery (arrow) .
Fig. 3. This photograph shows the preoperative situation. See Fig. 5, A for comparison . Chest radiograms revealed an enlarged heart with marked pulmonary plethora . The electrocardiogram showed sinus rhythm at a rate of 150 beats per minute, with P pulmonale, biventricular hypertrophy, and a mean frontal axis of minus 115 degrees . Blood biochemistry was unremarkable and hemoglobin was 13.4 Gm. per 100 ml. On admission she was being treated with Lanoxin (0.03 mg. twice daily) and furosemide (5 mg. daily). On March 20, 1976, with the use of general anesthesia and intermittent positive-pressure respiration , right heart catheterization was performed via the right saphenous vein . The pulmonary artery and PDA were entered and, through the duct, the descending aorta. The left atrium was reached via a patent foramen ovale, but the left ventricle was not entered.
There was a left-to-right shunt at the ventricular level with a pulmonary-to-systemic flow ratio of 2.3 : I, and the pulmonary artery pressure was at systemic level. Right ventricular angiograms showed an enlarged pulmonary artery, and a through duct to the descending aorta and the left subclavian artery was seen to fill from this injection (Fig. I). An aortogram (Fig. 2, A) showed complete interrupt ion of the aortic arch between the left carotid and left subclavian arteries (Type 8) .6 The left vertebral artery was seen filling retrogradely from above, and the left subclavian artery also was demonstrated from this route (Fig . 2, B) . Following cardiac catheterization, she remained in left ventricular failure, but operation was delayed because of gastroenteritis. Therefore, on April 27, 1976, total correction
The Journal of Thoracic and Cardiovascular Surgery
620 Monro, Brawn, Conway
40 38 36
.." c
~
34
°c
Nasopharyngeal Temperature
32 30
28 26 24 22 20 18 16 14 12 10
tst Period Total Arrest (71mins)
2
3
4
5
6
7
Time in Hours
Fig. 4. This graph shows the surface cooling, two periods of circulatory arrest, and bypass rewarming during the operation. was performed with the infant under profound hypothermia. At operation (Fig. 3) the diameter of the ascending aorta was 6 mm., the pulmonary artery 18 mm., the duct 6 mm., and the VSD II mm. The patient was surface cooled to 25° C. (Fig. 4), the sternum opened, and cardiac bypass commenced at 23° C. for 3 minutes, taking the temperature down to 17° C. The ascending aorta then was cross-clamped distal to the aortic cannula, and when the heart was empty, the superior and inferior venae cavae were snugged. The innominate artery, the left carotid artery, and the duct were cross-clamped. The duct, together with an anterior flap of pulmonary artery wall approximately 15 mm. in length, was resected from the remainder of the pulmonary artery. The new aortic arch then was fashioned by sewing the pulmonary artery tissue attached to the duct inferiorly so as to form a tube (Fig. 5). After the defect in the pulmonary artery had been closed by direct suture, the artery appeared adequate, with a diameter of 10 mm. An incision was made in the left side of the proximal aorta extending into the left common carotid artery, and to this the new aortic arch was anastomosed with 6-0 Prolene (Fig. 6). Following a further period of bypass, the patent foramen ovale was closed, and the ventricular septal defect was repaired through a right ventriculotomy with a Dacron patch. After air had been removed, bypass was recommenced and the baby was rewarmed to 32° C. at which time the heart took over easily in sinus rhythm, without the need for supportive therapy. Following the correction the systolic pressures in the ascending and descending aorta were equal at 100 mm. Hg. She was extubated half an hour after returning to the intensive care unit and was in sinus rhythm with a rate of 150 beats per minute. Seventeen hours later, a short burst of ventricular tachycardia developed, which responded immediately to lidocaine given intravenously. For several days she remained in mild left ventricular failure, for which she received Lasix
and Lanoxin. However, she was pink, active and feeding well. On May 8, she was discharged home but was readmitted urgently 2 days later with a supraventricular tachycardia of 300 beats per minute. This was difficult to control initially, but finally it responded to increased doses of Lanoxin and propranolol, and subsequently she was discharged on May 21. When reviewed on July 6, she was well, in sinus rhythm at 100 beats per minute, and weighed 6.6 kilograms. However, on auscultation a systolic murmur was noted at the left sternal edge. A possible diagnosis of a residual VSD was made, and therefore she was reinvestigated on Oct. 12, 1976. Cardiac catheterization demonstrated no evidence of a residual VSD, but there was a stenosis of the distal main pulmonary artery with a gradient of 53 mm. Hg across it. The systolic pressure in the proximal pulmonary artery was 68 mm. Hg, as compared with 80 mm. Hg in the aorta. The new aortic arch was of satisfactory caliber (Fig. 7, A and B), including that portion composed entirely of ductus tissue. A doubtfully significant gradient of 5 mm. Hg was seen on withdrawal from the ascending to descending aorta.
Discussion Although IAA is rare, it is one of the few conditions grouped under the name "hypoplastic left heart syndrome" in which surgery can be helpful. The three main types described by Celona and Patton" are shown in Fig. 8, together with the relative frequency of each type. I There are almost always associated anomalies of which PDA and VSD are the commonest; however, very rarely these can be absent.': 7 There is a very high early mortality rate in this condition, and about 80 percent of the patients die in the first month of life," with only 10 percent surviving the first year." Therefore, it is obvious that early operation is required if the natural history of the disease is to be altered, and perhaps we were fortunate that our patient had lived to 5 months of age before presenting for operation. Several authors have reported palliative and partially corrective surgical procedures.v 9 but if total correction is feasible, it would seem to be preferable. The first successful correction of IAA in infancy was reported by Barratt-Boyes and associates" in 1972. This was a Type A IAA, and a Dacron graft was used to restore aortic continuity, together with closure of the VSD and correction of intracardiac total anomalous pulmonary venous drainage. The patient was only 8 days old but probably will need a further operation to replace the Dacron graft. Murphy and associates" reported the first successful correction of Type B IAA in a 3-day-old patient. They used homograft vein from the patient's father to restore aortic continuity, but again this probably will need replacing later. Trusler and Izukawa" performed the only other previously reported
Volume 74 Number 4
Interrupted aortic arch with VSD
October, 1977
LeA
(b)
Fig. 5. This shows the stages in the fashioning of a new aortic arch. A, The preoperative situation (compare with Fig. 3). B, The single aortic and venous cannulas are in position and a flap of anterior main pulmonary artery wall has been dissected free so that the persistent ductus arteriosus (PDA) and descending aorta are free. C, The flap of pulmonary artery is then sewn into a tube and the pulmonary artery closed by direct suture. D, The final situation shows the new "aorta" sutured to the ascending aorta at the base of the left carotid artery and the right ventriculotomy. SVC, Superior vena cava. AO, Aorta. Inom, Innominate artery. LCA, Left carotid artery. PA, Main pulmonary artery. LA, Left atrial appendage. RA, Right atrium. RV, Right ventricle. PDA, Persistent ductus arteriosus. RPA, Right pulmonary artery. LPA, Left pulmonary artery.
Fig. 6. Postoperative appearance (compare Fig. 5, D). The forceps are pointing to the suture line in the pulmonary artery.
621
622
Monro, Brawn, Conway
The Journal of Thoracic and Cardiovascular Surgery
Fig. 7. The aortogram 5 months after operation shows a rather acute angle at the anastomosis but no significant narrowing. A, Anteroposterior view. B, Lateral view also shows the left subclavianartery arising distal to the site of the duct. without any need for later surgery, we formed a tube out of a flap of pulmonary artery and the PDA . This allowed a tension-free anastomosis across which there was no gradient at operation and an insignificant one at subsequent cardiac catheterization. Angiographic studies did not suggest any narrowing at the site of the duct , which suggests that its use for this purpose is satisfactory. Unfortunately, direct suture of the pulmonary arteriotomy subsequently proved to have caused a narrowing at the level of the distal main pulmonary artery, but this could have been avoided easily by insertion of a patch into the pulmonary artery, had the pulAscending monary artery not seemed adequate. Resection of this aorta lesion probably will be needed in later life. In the three previously reported cases just described, and in our own, profound hypothermia with circulatory Fig. 8. This diagram shows the different types of interrupted arrest was used and would seem to be the method of aortic arch described by Celoria and Patton," and their relachoice .:" However, it is unnecessary to use a separate tive frequency according to Van Praagh and co-workers. I arterial input for the ascending and descending aortas, as reported by Trusler and Izukawa." successful total correction in infancy . In their l3 -dayThis condition remains one of the most challenging old patient with Type B IAA, they detached the deto be encountered by the surgeon, but early total correcscending aorta from the pulmonary artery and, having tion can be achieved successfully, and the formation of a new aortic arch by using the PDA and a flap of the mobilized it, anastomosed it directly to the ascending aorta. It was hoped that this procedure would avoid any enlarged pulmonary artery is a previously unreported further operation, but reinvestigation demonstrated a method of producing a tension-free reconstruction that residual gradient of 20 mm . Hg across this anastomosis should grow with the patient. 5 months after operation, and it remains to be seen We are grateful to Mrs. S. Jacobs of the Department of whether further surgery will be required. Teaching Media, Southampton University, for her help with the ill ustrations. In an endeavor to perform a complete correction
Volume 74 Number 4 October, 1977
Interrupted aortic arch with VSD
REFERENCES
2
3
4
5
Van Praagh, R., Bernhard, W. F., Rosenthal, A., Parisi, L. F., and Fyler, D. c.: Interrupted Aortic Arch: Surgical Treatment, Am. J. Cardio!. 27: 200, 197I. Ventemiglia, R., Oglietti, J., Wukasch, D. c., Hallman, G. L., and Cooley, D. A.: Interruption of the Aortic Arch: Surgical Considerations, J. THORAC. CARDIOVASC. SURG. 72: 235, 1976. 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: Total Correction in an 8-Day-Old Infant by Means of Profound Hypothermia and Limited Cardiopulmonary Bypass, 1. THORAC. CARDIOVASC. SURG. 63: 367,1972. Murphy, D. A., Lemire, G. G., Tessler, I., and Dunn, G. L.: Correction of Type B Aortic Arch Interruption With Ventricular and Atrial Septal Defects in a Three-Day-Old Infant, J. THoRAc. CARDIOVASC. SURG. 65: 882, 1973. Trusler, G. A., and Izukawa, T.: Interrupted Aortic Arch
6 7
8
9
IO
623
and Ventricular Septal Defect: Direct Repair Through a Median Sternotomy Incision in a 13-Day-Old Infant, J. THoRAc. CARDIOVASC. SURG. 69: 126, 1975. Celoria, G. C., and Patton, R. B.: Congenital Absence of the Aortic Arch, Am. Heart J. 58: 407, 1959. Sharratt, G. P., Carson, P., and Sanderson, J. M.: Complete Interruption of Aortic Arch, Without Persistent Ductus Arteriosus in an Adult, Br. Heart J. 37: 221, 1975. Roberts, W. C., Morrow, A. G., and Braunwald, E.: Complete Interruption of the Aortic Arch, Circulation 26: 39, 1962. Murphy, A., Collins, G., and Dobell, A. R. c. Surgical Correction of Type A Congenital Aortic Arch Interruption, Ann. Thorae. Surg. 11: 593, 1971. Barratt-Boyes, B. G., Simpson, M., and Neutze, J. M.: Intracardiac Surgery in Neonates and Infants Using Deep Hypothermia With Surface Cooling and Limited Cardiopulmonary Bypass, Circulation 43: 25, 1971 (Supp!. I) .