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Aortic arch anomalies in children and infants
Aortic arch anomalies in children and infants The authors report their experience in 150 cases of aortic arch anomalies treated surgically in children and infants. The progress achieved over the past few years through improved diagnosis and resuscitative care has made it possible to obtain good results in the vast majority of cases. J. P. Binet and J. Langlois, Paris, France
-L he first surgical intervention for a developmental anomaly of an aortic arch was performed by Gross 8,9 in 1946. Thirty years later it has become possible to establish well-defined indications and surgical techniques. In this paper, we report our experience with surgery in 150 children, the largest series published to date. Aortic arch anomalies may occur as isolated malformations but in many cases they are associated with other cardiovascular or even visceral deformities (curable or not). The major factor underlying the simplicity and success of surgery is precise preoperative evaluation of the malformations to be treated. It should not be forgotten that the surgeon's view of the mediastinum is only a restricted and partial one and that the operative approach allows him to explore only a limited region. In this respect, the anatomicopathological, embryological, and clinical works of Edwards,6' 7 with his threegroup classification, and Corone and associates,4 with their recapitulative and explicative schema, have been of invaluable assistance to the surgeon. Not only have these works provided an understanding of the malformations resulting from anomalies of the fourth pair of branchial arches, but they have also made it possible to imagine other possible malformations, some of which have not yet been described. Most of all, they provide precise foreknowledge of the anomalies to be treated through simple radiocinematographic examination of the esophagus, angiography often being a "luxury." Methods Successive atresia of certain segments of the embryo's aortic arches and maintenance of patency of From Centre Chirurgical Marie Lannelongue, Paris, France. Received for publication Aug. 9, 1976. Accepted for publication Aug. 26, 1976. Address for reprints: J. P. Binet, Centre Chirurgical Marie Lannelongue, 129 rue de Tolbiac, 75013, Paris, France. 248
others result not only in the normal anatomy of the aortic system but also in most of the known malformations of the aorta and its branches. The schema proposed by Corone is at present the most convenient "method" for understanding both the various malformations and their radiologic interpretation from examination of the barium-filled esophagus. Reproduced many times, this schema has become classical today. It has the advantage of "providing" for the future since the five possibilities described may be completed by five other varieties which are mirror images of the previous ones with the descending aorta and ligamentum arteriosum on the right; not all of these varieties have yet been described. With regard to the so-called classical forms, it may be said that the complete double aortic arch is the most typical form, with, in the majority of cases, a left anterior arch narrower than the posterior arch and the descending aorta on the left. An anomalous retroesophageal right subclavian artery is undoubtedly the most common form, but is usually asymptomatic. Neuhauser's anomaly is a particularly constrictive malformation for the trachea. Clinical findings The clinical patterns to which these malformations give rise are well known to all; the initial symptoms usually occur during the first days of life. In some cases, however, the first manifestations of disease become apparent around the age of 50, when atheroma reveals a malformation hitherto well tolerated. The severity of the clinical picture is extremely variable and all degrees may be encountered from the almost asymptomatic form to the dramatic situation which is rapidly fatal if surgery is not undertaken. Respiratory and digestive symptoms are largely predominant in the clinical picture. Stridor is the major respiratory manifestation. It is a raucous and sometimes wheezing sound heard during
Volume 73 Number 2 February, 1977
both inspiration and expiration. The asphyxial phenomena to which it gives rise occur on exertion and during feeding. These respiratory symptoms are attended by gastrointestinal symptoms. In newborn infants, feeding is difficult, long, and interspersed with bouts of coughing and cyanosis. Acute respiratory manifestations which can lead to death occur when an attempt is made to feed the child. Five of our most recent surgical patients were emergency admissions who arrived with an endotracheal tube. These patients were referred from a resuscitation unit (Drs. Huault and Mathieu) where the diagnosis had been established by examination of the esophagogram performed under assisted ventilation. Examination of the barium-filled esophagus (on standard films or preferably by radiocinematography) is sufficient to enable precise diagnosis of the malformation.2' 13 Barium is preferable to all other contrast media. In almost all of our cases, this investigation technique was sufficient not only to detect the presence of a malformation but also to specify the type of anomaly we were dealing with. In this regard, the enormous work carried out by the radiology department of the EnfantsMalades hospital, and in particular by Sauvegrain and Fortier-Beaulieu, has been of invaluable assistance. Let us review the signs observed on examination of the barium-filled esophagus. The complete double aortic arch is reflected by the following characteristic aspects. On anteroposterior films, the esophagus is indented at two levels: (1) a deep indentation on the right esophageal border at the level of the third thoracic vertebra and (2) a lower, more shallow indentation on the left border. On lateral films, a constant, clear-cut indentation may be seen on the posterior aspect of the esophagus. When an anomalous retroesophageal right subclavian artery is present, an indentation is seen only on lateral films. With an anomalous left subclavian artery, the existence of a retroaortic diverticulum enlarges the picture of posterior compression whereas the ligamentum arteriosum sometimes indents the left border of the esophagus. Anomalies of the Neuhauser type produce a characteristic double indentation on anteroposterior films: (1) a wide indentation on the right border, reflecting a right aortic arch; (2) a sharp indentation situated lower down which corresponds to the ligamentum arteriosum. Sauvegrain clearly showed the importance of concluding the investigation by looking for an associated tracheoesophageal fistula.
Aortic arch anomalies
249
Techniques Standard chest films (anteroposterior and lateral) and tomographies do not provide any direct information in the majority of cases except when a compressive brachiocephalic trunk is present. They are, however, of great importance in our opinion since they enable the diameter of the trachea to be estimated (on lateral films), thereby allowing evaluation of the risk of associated tracheomalacia. Other investigation methods are usually of little use. A recently introduced technique10, 14 which is usually safe and provides very well-defined views is aortography. However, this technique is not indispensable to diagnosis and does not always reveal the anomaly distinctly. This is clearly illustrated by one of our cases of a double aortic arch in which the diagnosis was rejected on angiographic findings although examination of the barium-filled esophagus showed evident signs! Some cases, however, warrant investigation by this method, which requires a highly qualified radiologist. Bronchoscopy is, in our opinion, of considerable value. It visualizes the tracheal stenosis, shows it to be of extrinsic origin, and "reflects" the anatomy of the compressed vessel by suppressing the pulse in the corresponding territory when the impression made by the vessel is effaced by the bronchoscope (sign of Waterston, Aberdeen, and Mustard). A thorough, detailed, general examination of the infant is indispensable (cardiovascular and general) to detect other possible malformation, since associated deformities are far from exceptional. Once the diagnosis has been made, surgical intervention is mandatory. In the opinion of Dupuis,5 surgery may be postponed until the age of 2 years in certain forms which are well tolerated and correctly followed up. We feel that surgery should be performed as early as possible (except perhaps in cases of a retroesophageal subclavian artery) since there is a definite risk of sudden death. Early intervention is, however, subject to the possibility of prolonged maintenance of an endotracheal tube in these children during the postoperative period. Left lateral thoracotomy is the operative approach of choice. When there has been adequate preoperative evaluation, the surgeon knows exactly what type of malformation to expect. He should cut the stenosing vessel and aerate the mediastinum (systematic section of the ductus arteriosus, partial freeing of the esophagus and trachea). Indeed, although the surgical technique itself is simple, the same is not true of the immediate postoperative period. None of the complications we have observed
The Journal o) Thoracic and Cardiovascular Surgery
2 5 0 Binet and Langlois
Table I. Isolated aortic arch anomalies Total Complete double arch: Small right arch Small left arch
11 47
Retro-esoph. subclavian A: Right Left
22 1
Neuhauser's anomaly Brachiocephalic trunk Anomalous left pulmonary artery Total
14 20 3 118
>2yr. of age
<2 yr. of age
f
52 10/1* 42/3*
5
6
2 2
—_ 5 2
— 15
21 20/1* 1/1* 9/0 18/0 3/1* 103/7*
*The seven deaths in this series occurred before 1969, and before pediatric resuscitative care: hospital infections, 2; nonintubated bronchomalacia, 4; digitalis intoxication, 1. Since 1969, 72 patients have undergone surgery without a single death.
(ventilation disturbances, chylothorax) raise any serious problems in postoperative management apart from tracheomalacia. This tracheal lesion is almost constant when stricture exceeds a certain degree. Moreover, in many cases tracheomalacia is latent and is disclosed only after extubation. A demonstrative illustration of this situation is provided by a bitter experience which occurred many years ago in 1958 at a time when one of us was operating in the department of Prof. Laurence at the Bretonneau Hospital. In this unfortunate patient, extubation proved to be impossible. Application of "glue" on the intubated trachea has not given us satisfactory results. In fact, the only therapy is prolonged postoperative endotracheal intubation via the nose. As a rule, it is necessary for 3 to 4 days but in one case an endotracheal tube had to be maintained for 21 days. In serious forms, high-dose steroid therapy (up to 5 or 6 mg. per kilogram per 24 hours) is routinely prescribed. In any case, owing to the risk of aspiration of food into the tracheobroncial tree, feeding should be resumed late and postoperative supervision should be prolonged. We recommend leaving these postoperative patients in a pediatrics unit with a resuscitation unit close at hand for at least 1 month after the operation so that endotracheal reintubation may be performed if necessary. Vascular anomalies Among the now classical isolated aortic arch malformations, two vascular anomalies warrant individualization. Right brachiocephalic trunk (RBCT) compressing the trachea (20 cases). This anomaly is a cause of respiratory accidents and sometimes even asphyxia in infants (three of our patients arrived with an endotra-
cheal tube and two had mouth-to-mouth resuscitation). A brachiocephalic trunk originating in the midline or to the left of the trachea has been considered as abnormal. Recent anatomic investigations appear to contradict this concept and some investigators are of the opinion that a brachiocephalic trunk originating on the left of the midline in children should be considered as normal. One definitely established fact is that in many cases the brachiocephalic trunk, flattened against the thymus and sternum, crushes the anterior aspect of the trachea. This "emergency" diagnosis should be suggested when examination of the barium-filled esophagus reveals none of the signs of a classical aortic arch anomaly (double aortic arch of Neuhauser's anomaly) in a child with severe respiratory distress. Every effort should be made to demonstrate the characteristic sign of an anomalous brachiocephalic trunk on standard lateral chest roentgenograms or better, on tomographies: indentation of the anterior aspect of the trachea at the level of its passage from the neck into the thorax. In most of our cases, angiography was performed but we feel that it is no longer indispensable. Midline sternotomy should be performed in these children: suspension of the brachiocephalic trunk from the posterior aspect of the sternum (Gross) no longer appears to be the ideal operation since its results are irregular despite combined thymectomy. In our opinion, section of this vessel (five cases) or better, its reimplantation onto the ascending aorta (15 cases) using a wide aortic annulus, is a far more preferable procedure," Anomalous left pulmonary artery (ALPA) (3 cases). An ALPA is a rare malformation. It gives rise to dramatic episodes of asphyxia. Here again, the diagnosis should be considered in the absence of roentgenographic signs suggestive of a double aortic arch or Neuhauser's anomaly, The most reliable diagnostic sign is an anterior indentation of the esophagus which expands at each systole at the level of the fourth or fifth thoracic vertebra. This sign can be seen on a lateral view of the barium-filled esophagus during radiocinematography, Angiography, performed with a correct view to separate the pulmonary trunk and its branches, clearly discloses the abnormal trajectory of the left pulmonary artery. This is the only indication for adjunctive procedures such as extracorporeal circulation or profound hypothermia. Although theoretically useless, such procedures are necessary in practice to separate the pulmonary artery from the posterior aspect of the trachea and to reimplant it on the pulmonary trunk.12 We have had three such cases including one death due to tracheomalacia.
Volume 73 Number 2 February, 1977
Discussion This is the present status (April, 1975) of our experience, which goes back over 15 years. Table I shows our experience of 118 isolated aortic arch anomalies (i.e., no other associated cardiac or visceral malformations). It can be seen that surgical cure of these malformations has become safe and that the mortality rate should approach that observed for the simplest congenital heart disease (patent ductus arteriosus) on the fundamental condition that after surgery the patient be given resuscitative care worthy of the name. The problem of aortic anomalies combined with other lesions is very different. Indeed, many cardiac malformations are attended by an aortic arch anomaly. These include Fallot's tetralogy, ventricular septal defect, coarctation, and even patent ductus arteriosus. Each of these raises the theoretical problem of both diagnosis and cure of the dual malformation. In practice, a decision has to be made as to which of the two anomalies, (the cardiac malformation or the vascular anomaly), is the most severe and should be first surgically repaired. Table II summarizes our experience with combined malformation. The following points are noteworthy. 1. In Fallot's tetralogy, the presence of an aortic arch anomaly poses a problem when performing systemic anastomosis in cases where it is indicated. In three cases, we used one of the arches (the smaller)13 and in eight cases we used the contralateral subclavian artery at the risk of creating an artificial double arch which was, however, always loose and never constricted neighboring structures.1 2. The problem of an anomalous subclavian artery associated with patent ductus arteriosus arose in 10 cases. In most instances, treatment consisted in amputating the anomalous artery; in rarer instances, it was reimplanted. 3. One patient with patent ductus arteriosus without a pressure gradient and therefore permanent pulmonary hypertension, associated with an anomalous subclavian artery, did not survive the postoperative period. 4. The presence of a ventricular septal defect sometimes raises the problem of a combined cerclage of the pulmonary artery. It is now possible to repair both malformations simultaneously in infants. This has been done three times, and in particular in a 6-month-old baby who had an anomalous left pulmonary artery and a ventricular septal defect. 5. Malformations of the trachea and/or the esophagus raise two problems, (a) The high incidence of combined vascular/tracheoesophageal malformations is unquestionable (esophageal atresia, with or without a tracheoesophageal fistula associated with a compress-
25 1
Aortic arch anomalies
Table II. Aortic arch anomalies and associated malformations No. of cases
Hospital deaths
11 3 8
2
V.S.D. ± patent D.A.: Double arch Subclavian artery A.L.P.A.
3 1 1 1
—
Coarctation ± patent D.A.: Subclavian artery B.C. trunk
9 8 1
—
Coarctation + V.S.D. +Patent D.A.: Subclavian artery
3 2
—
Patent D.A. + permanent pulm. HT: Subclavian artery
1 1
—
Tracheoesophageal fistula (simultaneous intervention): Subclavian artery
2
—
Fallot's tetralogy: Double arch Subclavian artery
Repaired esophageal atresia: Subclavian artery B.C. trunk Total
2 3 0 3 32
—
y
ive brachiocephalic trunk). As a rule, the esophageal malformation is treated before the vascular anomaly. The only death (1 year after surgery), following section or reimplantation of the brachiocephalic trunk, was caused by repermeation of a tracheoesophageal fistula (Mendelsohn's syndrome) repaired surgically very early in life, (b) A tracheoesophageal fistula, diagnosed and surgically confirmed in two cases, was treated at the same time as the vascular anomaly in children 3 and 5 years of age by a cervical approach in one case and by thoracotomy in the other.3 There is no doubt, however, that the low mortality rate recorded by us in these cases of combined malformations does not adequately reflect the greater severity of this situation. Indeed, the initial operation is usually a palliative one often followed (namely in Fallot's tetralogy) by surgical repair of the vascular anomaly, which carries a risk of its own. Conclusion The surgical repair of aortic arch anomalies has made such advances that it has become simple and virtually devoid of risk. This being so, whatever the patient's age, the indication for surgery is immediate whenever threatening symptoms are present. The only
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questionable indication is a retroesophageal subclavian artery associated with respiratory disturbances. Many surgeons do not operate in this case, but resort to regular follow-up. This is also our tendency at the present time. Long-term results (70 per cent of our patients have been followed) have shown that all symptoms subside after a variable period of time, which may be as long as 1 year. This is the time it takes for tracheomalacia to disappear—a condition in which the surgeon is helpless. REFERENCES 1 Binet, J. P., Carpentier, A., Pottemain, M., and Langlois, J.: Double Arc Aorta Associated With Tetralogy of Fallot in Infants, J. THORAC. CARDIOVASC. SURG. 51:
114, 1966. 2 Binet, J. P., and Langlois, J.: Les anomalies des arcs aortiques chez l'enfant, J. Agre'ge's 5: 403, 1972. 3 Binet, J. P., Langlois, J., Conso, J. F., andPlanche, Cl.: A propos de 3 cas de fistule conge'nitale oeso-trache'obronchique, Ann. Chir. Thorac. Cardiovasc. 14: 33,1975. 4 Corone, P., Nouaile, J., Schweisguth, O., Mathey, J., et Binet, J. P.: Les anomalies des arcs aortiques chez le nourrisson, Arch. France Pediatr. 12: 830, 1955. 5 Dupuis, Cl.: Personal communication.
The Journal of Thoracic and Cardiovascular Surgery
6 Edwards, J. E.: Malformations of the Aortic Arch System Manifested as "Vascular Rings," Lab. Invest. 2: 56, 1953. 7 Edwards, J. E.: Pathology of Anomalies of Thoracic Aorta, Am. J. Clin. Pathol. 23: 1240, 1953. 8 Groos, R. E.: Surgical Relief for Tracheal Obstruction From a Vascular Ring, N. Engl. J. Med. 233: 586, 1945. 9 Groos, R. E.: Compression of the Trachea or Esophagus by Vascular Anomalies, Pediatrics 7: 69, 1951. 10 Jaubert de Beaulieu, Campo-Paysaa, and Kormaz, F.: Les anomalies des arc aortiques, Lyon Chir. 19: 78, 1972. 11 Langlois, J., Binet, J. P., Germain, M., Cloup, M. et I., Fourner, J. P., et Pottemain, M.: La compression de la trache'e par le tronc arte'riel brachio-ce'phalique, chez le nourrisson, Ann. Chir. Thorac. Cardiovasc. 10: 181, 1971. 12 Langlois, J., Germain, M., Gabilan, J. Cl., Pottemain, M., Belloy, A., Conso, J. F., Cloup, M., FortierBeaulieu, M., Watchi, M., and Binet, J. P.: L'artere pulmonaire gauche aberrante. A propos d'un nouveau cas ope're, Ann. Chir. Thorac. Cardiovasc. 9: 311, 1970. 13 Mathey, J., Binet, J. P., and Denis, B.: Anomalies et de'veloppement des arc aortiques, J. Chir. (Paris) 5: 505, 1959. 14 Wade, H., Shuford, M. D., Robert, G., and Sybers: The Aortic Arch and Its Malformations, Springfield, 111, 1974, Charles C Thomas Publisher.
-L he first surgical intervention for a developmental anomaly of an aortic arch was performed by Gross 8,9 in 1946. Thirty years later it has become possible to establish well-defined indications and surgical techniques. In this paper, we report our experience with surgery in 150 children, the largest series published to date. Aortic arch anomalies may occur as isolated malformations but in many cases they are associated with other cardiovascular or even visceral deformities (curable or not). The major factor underlying the simplicity and success of surgery is precise preoperative evaluation of the malformations to be treated. It should not be forgotten that the surgeon's view of the mediastinum is only a restricted and partial one and that the operative approach allows him to explore only a limited region. In this respect, the anatomicopathological, embryological, and clinical works of Edwards,6' 7 with his threegroup classification, and Corone and associates,4 with their recapitulative and explicative schema, have been of invaluable assistance to the surgeon. Not only have these works provided an understanding of the malformations resulting from anomalies of the fourth pair of branchial arches, but they have also made it possible to imagine other possible malformations, some of which have not yet been described. Most of all, they provide precise foreknowledge of the anomalies to be treated through simple radiocinematographic examination of the esophagus, angiography often being a "luxury." Methods Successive atresia of certain segments of the embryo's aortic arches and maintenance of patency of From Centre Chirurgical Marie Lannelongue, Paris, France. Received for publication Aug. 9, 1976. Accepted for publication Aug. 26, 1976. Address for reprints: J. P. Binet, Centre Chirurgical Marie Lannelongue, 129 rue de Tolbiac, 75013, Paris, France. 248
others result not only in the normal anatomy of the aortic system but also in most of the known malformations of the aorta and its branches. The schema proposed by Corone is at present the most convenient "method" for understanding both the various malformations and their radiologic interpretation from examination of the barium-filled esophagus. Reproduced many times, this schema has become classical today. It has the advantage of "providing" for the future since the five possibilities described may be completed by five other varieties which are mirror images of the previous ones with the descending aorta and ligamentum arteriosum on the right; not all of these varieties have yet been described. With regard to the so-called classical forms, it may be said that the complete double aortic arch is the most typical form, with, in the majority of cases, a left anterior arch narrower than the posterior arch and the descending aorta on the left. An anomalous retroesophageal right subclavian artery is undoubtedly the most common form, but is usually asymptomatic. Neuhauser's anomaly is a particularly constrictive malformation for the trachea. Clinical findings The clinical patterns to which these malformations give rise are well known to all; the initial symptoms usually occur during the first days of life. In some cases, however, the first manifestations of disease become apparent around the age of 50, when atheroma reveals a malformation hitherto well tolerated. The severity of the clinical picture is extremely variable and all degrees may be encountered from the almost asymptomatic form to the dramatic situation which is rapidly fatal if surgery is not undertaken. Respiratory and digestive symptoms are largely predominant in the clinical picture. Stridor is the major respiratory manifestation. It is a raucous and sometimes wheezing sound heard during
Volume 73 Number 2 February, 1977
both inspiration and expiration. The asphyxial phenomena to which it gives rise occur on exertion and during feeding. These respiratory symptoms are attended by gastrointestinal symptoms. In newborn infants, feeding is difficult, long, and interspersed with bouts of coughing and cyanosis. Acute respiratory manifestations which can lead to death occur when an attempt is made to feed the child. Five of our most recent surgical patients were emergency admissions who arrived with an endotracheal tube. These patients were referred from a resuscitation unit (Drs. Huault and Mathieu) where the diagnosis had been established by examination of the esophagogram performed under assisted ventilation. Examination of the barium-filled esophagus (on standard films or preferably by radiocinematography) is sufficient to enable precise diagnosis of the malformation.2' 13 Barium is preferable to all other contrast media. In almost all of our cases, this investigation technique was sufficient not only to detect the presence of a malformation but also to specify the type of anomaly we were dealing with. In this regard, the enormous work carried out by the radiology department of the EnfantsMalades hospital, and in particular by Sauvegrain and Fortier-Beaulieu, has been of invaluable assistance. Let us review the signs observed on examination of the barium-filled esophagus. The complete double aortic arch is reflected by the following characteristic aspects. On anteroposterior films, the esophagus is indented at two levels: (1) a deep indentation on the right esophageal border at the level of the third thoracic vertebra and (2) a lower, more shallow indentation on the left border. On lateral films, a constant, clear-cut indentation may be seen on the posterior aspect of the esophagus. When an anomalous retroesophageal right subclavian artery is present, an indentation is seen only on lateral films. With an anomalous left subclavian artery, the existence of a retroaortic diverticulum enlarges the picture of posterior compression whereas the ligamentum arteriosum sometimes indents the left border of the esophagus. Anomalies of the Neuhauser type produce a characteristic double indentation on anteroposterior films: (1) a wide indentation on the right border, reflecting a right aortic arch; (2) a sharp indentation situated lower down which corresponds to the ligamentum arteriosum. Sauvegrain clearly showed the importance of concluding the investigation by looking for an associated tracheoesophageal fistula.
Aortic arch anomalies
249
Techniques Standard chest films (anteroposterior and lateral) and tomographies do not provide any direct information in the majority of cases except when a compressive brachiocephalic trunk is present. They are, however, of great importance in our opinion since they enable the diameter of the trachea to be estimated (on lateral films), thereby allowing evaluation of the risk of associated tracheomalacia. Other investigation methods are usually of little use. A recently introduced technique10, 14 which is usually safe and provides very well-defined views is aortography. However, this technique is not indispensable to diagnosis and does not always reveal the anomaly distinctly. This is clearly illustrated by one of our cases of a double aortic arch in which the diagnosis was rejected on angiographic findings although examination of the barium-filled esophagus showed evident signs! Some cases, however, warrant investigation by this method, which requires a highly qualified radiologist. Bronchoscopy is, in our opinion, of considerable value. It visualizes the tracheal stenosis, shows it to be of extrinsic origin, and "reflects" the anatomy of the compressed vessel by suppressing the pulse in the corresponding territory when the impression made by the vessel is effaced by the bronchoscope (sign of Waterston, Aberdeen, and Mustard). A thorough, detailed, general examination of the infant is indispensable (cardiovascular and general) to detect other possible malformation, since associated deformities are far from exceptional. Once the diagnosis has been made, surgical intervention is mandatory. In the opinion of Dupuis,5 surgery may be postponed until the age of 2 years in certain forms which are well tolerated and correctly followed up. We feel that surgery should be performed as early as possible (except perhaps in cases of a retroesophageal subclavian artery) since there is a definite risk of sudden death. Early intervention is, however, subject to the possibility of prolonged maintenance of an endotracheal tube in these children during the postoperative period. Left lateral thoracotomy is the operative approach of choice. When there has been adequate preoperative evaluation, the surgeon knows exactly what type of malformation to expect. He should cut the stenosing vessel and aerate the mediastinum (systematic section of the ductus arteriosus, partial freeing of the esophagus and trachea). Indeed, although the surgical technique itself is simple, the same is not true of the immediate postoperative period. None of the complications we have observed
The Journal o) Thoracic and Cardiovascular Surgery
2 5 0 Binet and Langlois
Table I. Isolated aortic arch anomalies Total Complete double arch: Small right arch Small left arch
11 47
Retro-esoph. subclavian A: Right Left
22 1
Neuhauser's anomaly Brachiocephalic trunk Anomalous left pulmonary artery Total
14 20 3 118
>2yr. of age
<2 yr. of age
f
52 10/1* 42/3*
5
6
2 2
—_ 5 2
— 15
21 20/1* 1/1* 9/0 18/0 3/1* 103/7*
*The seven deaths in this series occurred before 1969, and before pediatric resuscitative care: hospital infections, 2; nonintubated bronchomalacia, 4; digitalis intoxication, 1. Since 1969, 72 patients have undergone surgery without a single death.
(ventilation disturbances, chylothorax) raise any serious problems in postoperative management apart from tracheomalacia. This tracheal lesion is almost constant when stricture exceeds a certain degree. Moreover, in many cases tracheomalacia is latent and is disclosed only after extubation. A demonstrative illustration of this situation is provided by a bitter experience which occurred many years ago in 1958 at a time when one of us was operating in the department of Prof. Laurence at the Bretonneau Hospital. In this unfortunate patient, extubation proved to be impossible. Application of "glue" on the intubated trachea has not given us satisfactory results. In fact, the only therapy is prolonged postoperative endotracheal intubation via the nose. As a rule, it is necessary for 3 to 4 days but in one case an endotracheal tube had to be maintained for 21 days. In serious forms, high-dose steroid therapy (up to 5 or 6 mg. per kilogram per 24 hours) is routinely prescribed. In any case, owing to the risk of aspiration of food into the tracheobroncial tree, feeding should be resumed late and postoperative supervision should be prolonged. We recommend leaving these postoperative patients in a pediatrics unit with a resuscitation unit close at hand for at least 1 month after the operation so that endotracheal reintubation may be performed if necessary. Vascular anomalies Among the now classical isolated aortic arch malformations, two vascular anomalies warrant individualization. Right brachiocephalic trunk (RBCT) compressing the trachea (20 cases). This anomaly is a cause of respiratory accidents and sometimes even asphyxia in infants (three of our patients arrived with an endotra-
cheal tube and two had mouth-to-mouth resuscitation). A brachiocephalic trunk originating in the midline or to the left of the trachea has been considered as abnormal. Recent anatomic investigations appear to contradict this concept and some investigators are of the opinion that a brachiocephalic trunk originating on the left of the midline in children should be considered as normal. One definitely established fact is that in many cases the brachiocephalic trunk, flattened against the thymus and sternum, crushes the anterior aspect of the trachea. This "emergency" diagnosis should be suggested when examination of the barium-filled esophagus reveals none of the signs of a classical aortic arch anomaly (double aortic arch of Neuhauser's anomaly) in a child with severe respiratory distress. Every effort should be made to demonstrate the characteristic sign of an anomalous brachiocephalic trunk on standard lateral chest roentgenograms or better, on tomographies: indentation of the anterior aspect of the trachea at the level of its passage from the neck into the thorax. In most of our cases, angiography was performed but we feel that it is no longer indispensable. Midline sternotomy should be performed in these children: suspension of the brachiocephalic trunk from the posterior aspect of the sternum (Gross) no longer appears to be the ideal operation since its results are irregular despite combined thymectomy. In our opinion, section of this vessel (five cases) or better, its reimplantation onto the ascending aorta (15 cases) using a wide aortic annulus, is a far more preferable procedure," Anomalous left pulmonary artery (ALPA) (3 cases). An ALPA is a rare malformation. It gives rise to dramatic episodes of asphyxia. Here again, the diagnosis should be considered in the absence of roentgenographic signs suggestive of a double aortic arch or Neuhauser's anomaly, The most reliable diagnostic sign is an anterior indentation of the esophagus which expands at each systole at the level of the fourth or fifth thoracic vertebra. This sign can be seen on a lateral view of the barium-filled esophagus during radiocinematography, Angiography, performed with a correct view to separate the pulmonary trunk and its branches, clearly discloses the abnormal trajectory of the left pulmonary artery. This is the only indication for adjunctive procedures such as extracorporeal circulation or profound hypothermia. Although theoretically useless, such procedures are necessary in practice to separate the pulmonary artery from the posterior aspect of the trachea and to reimplant it on the pulmonary trunk.12 We have had three such cases including one death due to tracheomalacia.
Volume 73 Number 2 February, 1977
Discussion This is the present status (April, 1975) of our experience, which goes back over 15 years. Table I shows our experience of 118 isolated aortic arch anomalies (i.e., no other associated cardiac or visceral malformations). It can be seen that surgical cure of these malformations has become safe and that the mortality rate should approach that observed for the simplest congenital heart disease (patent ductus arteriosus) on the fundamental condition that after surgery the patient be given resuscitative care worthy of the name. The problem of aortic anomalies combined with other lesions is very different. Indeed, many cardiac malformations are attended by an aortic arch anomaly. These include Fallot's tetralogy, ventricular septal defect, coarctation, and even patent ductus arteriosus. Each of these raises the theoretical problem of both diagnosis and cure of the dual malformation. In practice, a decision has to be made as to which of the two anomalies, (the cardiac malformation or the vascular anomaly), is the most severe and should be first surgically repaired. Table II summarizes our experience with combined malformation. The following points are noteworthy. 1. In Fallot's tetralogy, the presence of an aortic arch anomaly poses a problem when performing systemic anastomosis in cases where it is indicated. In three cases, we used one of the arches (the smaller)13 and in eight cases we used the contralateral subclavian artery at the risk of creating an artificial double arch which was, however, always loose and never constricted neighboring structures.1 2. The problem of an anomalous subclavian artery associated with patent ductus arteriosus arose in 10 cases. In most instances, treatment consisted in amputating the anomalous artery; in rarer instances, it was reimplanted. 3. One patient with patent ductus arteriosus without a pressure gradient and therefore permanent pulmonary hypertension, associated with an anomalous subclavian artery, did not survive the postoperative period. 4. The presence of a ventricular septal defect sometimes raises the problem of a combined cerclage of the pulmonary artery. It is now possible to repair both malformations simultaneously in infants. This has been done three times, and in particular in a 6-month-old baby who had an anomalous left pulmonary artery and a ventricular septal defect. 5. Malformations of the trachea and/or the esophagus raise two problems, (a) The high incidence of combined vascular/tracheoesophageal malformations is unquestionable (esophageal atresia, with or without a tracheoesophageal fistula associated with a compress-
25 1
Aortic arch anomalies
Table II. Aortic arch anomalies and associated malformations No. of cases
Hospital deaths
11 3 8
2
V.S.D. ± patent D.A.: Double arch Subclavian artery A.L.P.A.
3 1 1 1
—
Coarctation ± patent D.A.: Subclavian artery B.C. trunk
9 8 1
—
Coarctation + V.S.D. +Patent D.A.: Subclavian artery
3 2
—
Patent D.A. + permanent pulm. HT: Subclavian artery
1 1
—
Tracheoesophageal fistula (simultaneous intervention): Subclavian artery
2
—
Fallot's tetralogy: Double arch Subclavian artery
Repaired esophageal atresia: Subclavian artery B.C. trunk Total
2 3 0 3 32
—
y
ive brachiocephalic trunk). As a rule, the esophageal malformation is treated before the vascular anomaly. The only death (1 year after surgery), following section or reimplantation of the brachiocephalic trunk, was caused by repermeation of a tracheoesophageal fistula (Mendelsohn's syndrome) repaired surgically very early in life, (b) A tracheoesophageal fistula, diagnosed and surgically confirmed in two cases, was treated at the same time as the vascular anomaly in children 3 and 5 years of age by a cervical approach in one case and by thoracotomy in the other.3 There is no doubt, however, that the low mortality rate recorded by us in these cases of combined malformations does not adequately reflect the greater severity of this situation. Indeed, the initial operation is usually a palliative one often followed (namely in Fallot's tetralogy) by surgical repair of the vascular anomaly, which carries a risk of its own. Conclusion The surgical repair of aortic arch anomalies has made such advances that it has become simple and virtually devoid of risk. This being so, whatever the patient's age, the indication for surgery is immediate whenever threatening symptoms are present. The only
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questionable indication is a retroesophageal subclavian artery associated with respiratory disturbances. Many surgeons do not operate in this case, but resort to regular follow-up. This is also our tendency at the present time. Long-term results (70 per cent of our patients have been followed) have shown that all symptoms subside after a variable period of time, which may be as long as 1 year. This is the time it takes for tracheomalacia to disappear—a condition in which the surgeon is helpless. REFERENCES 1 Binet, J. P., Carpentier, A., Pottemain, M., and Langlois, J.: Double Arc Aorta Associated With Tetralogy of Fallot in Infants, J. THORAC. CARDIOVASC. SURG. 51:
114, 1966. 2 Binet, J. P., and Langlois, J.: Les anomalies des arcs aortiques chez l'enfant, J. Agre'ge's 5: 403, 1972. 3 Binet, J. P., Langlois, J., Conso, J. F., andPlanche, Cl.: A propos de 3 cas de fistule conge'nitale oeso-trache'obronchique, Ann. Chir. Thorac. Cardiovasc. 14: 33,1975. 4 Corone, P., Nouaile, J., Schweisguth, O., Mathey, J., et Binet, J. P.: Les anomalies des arcs aortiques chez le nourrisson, Arch. France Pediatr. 12: 830, 1955. 5 Dupuis, Cl.: Personal communication.
The Journal of Thoracic and Cardiovascular Surgery
6 Edwards, J. E.: Malformations of the Aortic Arch System Manifested as "Vascular Rings," Lab. Invest. 2: 56, 1953. 7 Edwards, J. E.: Pathology of Anomalies of Thoracic Aorta, Am. J. Clin. Pathol. 23: 1240, 1953. 8 Groos, R. E.: Surgical Relief for Tracheal Obstruction From a Vascular Ring, N. Engl. J. Med. 233: 586, 1945. 9 Groos, R. E.: Compression of the Trachea or Esophagus by Vascular Anomalies, Pediatrics 7: 69, 1951. 10 Jaubert de Beaulieu, Campo-Paysaa, and Kormaz, F.: Les anomalies des arc aortiques, Lyon Chir. 19: 78, 1972. 11 Langlois, J., Binet, J. P., Germain, M., Cloup, M. et I., Fourner, J. P., et Pottemain, M.: La compression de la trache'e par le tronc arte'riel brachio-ce'phalique, chez le nourrisson, Ann. Chir. Thorac. Cardiovasc. 10: 181, 1971. 12 Langlois, J., Germain, M., Gabilan, J. Cl., Pottemain, M., Belloy, A., Conso, J. F., Cloup, M., FortierBeaulieu, M., Watchi, M., and Binet, J. P.: L'artere pulmonaire gauche aberrante. A propos d'un nouveau cas ope're, Ann. Chir. Thorac. Cardiovasc. 9: 311, 1970. 13 Mathey, J., Binet, J. P., and Denis, B.: Anomalies et de'veloppement des arc aortiques, J. Chir. (Paris) 5: 505, 1959. 14 Wade, H., Shuford, M. D., Robert, G., and Sybers: The Aortic Arch and Its Malformations, Springfield, 111, 1974, Charles C Thomas Publisher.