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Left ventricular outflow tract obstruction with aortic arch anomalies and ventricular septal defect
CORRESPONDENCE
Valve Insufficiency After Atrioventricular Septal Defect Repair: Differences Between Patients With and Without Down’s Syndrome? To the Editor: In a recent issue of The Annals 1 read the interesting paper of Abbruzzese and associates [ 11 on the anatomical and surgical determinants of late mitral valve insufficiency after repair of partial atrioventricular canal. Unfortunately, Abbruzzese and associates did not report any data about the presence of Down’s syndrome in their patients. How many of the 9 patients that underwent reoperation for mitral dysfunction had Down’s syndrome? Was there a difference between children with and without Down’s syndrome in their series? Because increasing attention is being paid to the anatomical [2, 31 and surgical [4, 51 differences between Down’s and non-Down’s populations with atrioventricular canal, 1 believe that these data must be analyzed and described even if not statistically significant.
Bruno Marino, M D Dipart imento Medico-Ch ir u rgico di Cardiolog ia Pedia t rica Ospedale Bambino Gesli Rome, Italy
References 1. Abbruzzese PA, Napoleone A, Bini M, Annecchino FP, Merlo M, Parenzan L. Late left atrioventricular valve insufficiency after repair of partial atrioventricular septal defects: anatomical and surgical determinants. Ann Thorac Surg 1990;49: 1114. 2. Penkoske PA, Neches WH, Anderson RH, Zuberbuhler JR. Further observations on the morphology of atrioventricular septal defects. J Thorac Cardiovasc Surg 1985;90:611-22. 3. De Biase L, Di Ciommo V, Ballerini L, Bevilacqua M, Marcelletti C, Marino B. Prevalence of left-sided obstructive lesions in patients with atrioventricular canal without Down’s syndrome. J Thorac Cardiovasc Surg 1986;91:467-72. 4. Williams WH, Perrella AM, Plauth WH Jr, Hatcher CR Jr, Guyton RA. Survival following repair of complete A-V canal defects associated with the Down syndrome. 1st World Congress of Pediatric Cardiac Surgery, Bergamo, Italy, June 1988:38. 5. Vet TW, Ottenkamp J. Correction of atrioventricular septal defect. Am J Dis Child 1989;143:1361-5.
Reply
To the Editor:
The focus of our paper was on anatomical and surgical factors possibly associated with late left atrioventricular (AV) valve insufficiency after repair of partial AV septal defects. However, we are aware that Dr Marino’s group is investigating the proposition that AV septal defects may be associated with a worse prognosis in children without chromosomal abnormalities, and we will gladly answer his questions. None of the 9 patients who required reoperation in our series had Down’s syndrome. Also, only 1 of the 16 patients (6.25%) in whom late left AV valve insufficiency developed had Down’s syndrome. These figures show a lower incidence than that of Down’s syndrome in our total series of partial AV septal defects (16 of 107 patients = 14.95%). These differences do not reach statistical significance ( p = 0.45 and 0.58, respectively). However, to correctly interpret these data, one must keep in mind that 15 patients, in whom the partial AV septal defect was 0 1990 by The Society of Thoracic Surgeons
part of a more complex malformation, were excluded from our study; none of them had Down‘s syndrome. Interestingly, similar to the findings of the group from the Ospedale Bambino Gesu 111, this series includes 3 patients with aortic coarctation and 2 patients with right ventricular dominance. Moreover, when reviewing our total experience with reoperations for left AV valve repair failure in AV septal defects (all types) we noticed a very low incidence of Down’s syndrome (2 of 22 = 9.1%) as compared with the 49% incidence of Down’s syndrome in the total group of AV septal defects (all types). This difference is highly significant ( p < 0.001). A specific search for differences in the severity of this malformation in patients with and without Down‘s syndrome was not performed in our center as we believe that AV septal defects must be repaired in patients with and without Down’s syndrome, regardless of their extent. More data are needed to confirm the controversial findings of Bull and associates [2] regarding an excellent life expectancy in complete AV septal defects with Down’s syndrome without surgical repair.
Pietro A. Abbruzzese, M D Alrssandra Napoleone, M D R. Margherita Bini, M D F . Paolo Annecchino, M D Maurizio Merlo, M D Lucio Parenzan, M D Divisione di Cardiochirurgia Ospedali Riuniti di Bergamo Bergamo, ltaly
References 1. De Biase L, Di Ciommo V, Ballerini L, Bevilacqua M, Marcelletti C, Marino B. Prevalence of left-sided obstructive lesions in patients with atrioventricular canal without Down’s syndrome. J Thorac Cardiovasc Surg 1986;91:467-72. 2. Bull C, Rigby ML, Shinebourne EA. Should management of complete atrioventricular canal defect be influenced by coexistent Down syndrome? Lancet 1985;1:1147-9.
Left Ventricular Outflow Tract Obstruction With Aortic Arch Anomalies and Ventricular Septal Defect To the Editor: Iwahara and colleagues [ l ] have recently suggested that patients with a ventricular septal defect (VSD) and either interrupted aortic arch or coarctation, who have a left ventricular outflow tract diameter to ascending aortic diameter ratio of less than 0.6, should not undergo a standard reparative procedure of VSD closure and correction of the arch anomaly. They have based their conclusion on 4 patients with interrupted aortic arch and 13 patients with coarctation and VSD. Three of 4 patients with a ratio less than 0.6 died, whereas 2 of 13 patients with a ratio greater than 0.6 died. They conclude that the presence of severe left ventricular outflow tract obstruction (as determined by a left ventricular outflow tract to aortic ratio of less than 0.6) “clearly necessitates modification of present surgical strategies.” Our recent review (21of 37 patients with interrupted aortic arch and VSD as the only associated anomaly has led us to an alternative conclusion regarding the clinical significance of left ventricular outflow tract obstruction. Only 2 of the 37 patients (5%)underwent modification of their initial procedure because of the presence of suspected severe subaortic stenosis. In 8 of the 37 patients, evidence of subaortic stenosis subsequently developed after repair. All cases became apparent within 3 years of repair and have been treated successfully by conventional methods. In Ann Thorac Surg 1990;50:854-60
0003-4975/90/$3.50
CORRESPONDENCE
Ann Thorac Surg 1990;50:854-64
spite of this conservative approach to the management of subaortic stenosis with interrupted aortic arch, there has been a dramatic improvement in survival after one-stage reparative operation, so that by 1986 the probability of death within 2 weeks of repair was 7%. Retrospective echocardiographic analysis of the left ventricular outflow tract diameter did not reveal this variable to be a useful predictor of outcome in our series. Neonates with a left ventricular outflow tract diameter as small as 3 mm have done well, whereas in others with greater diameters left ventricular outflow tract obstruction has subsequently developed. We have speculated that VSD closure results in a shift in interventricular septal position resulting in an increase in the effective diameter of the left ventricular outflow tract, perhaps analagous to the situation with left ventricular outflow tract obstruction and transposition after an arterial switch procedure. One probable predictor of subsequent development of left ventricular outflow tract obstruction was found to be the presence of an aberrant right subclavian artery arising from the descending thoracic aorta in patients with type B interrupted aortic arch [3]. The arterial pH immediately before surgical repair was an important predictor of subsequent outcome in our series. We note that the mean pH of patients in the high mortality group from Iwahara’s series was 7.26 with a base excess of -8.7. We believe that aggressive resuscitation of the neonate with interrupted aortic arch including not only the use of prostaglandin E, to maintain ductal patency but also ventilation with room air to improve systemic perfusion, appropriate use of inotropic agents and antibiotics, and probably most importantly, waiting an appropriate period of time so that all variables have normalized before proceeding to operation, are more likely to improve the outcome of operation rather than complex modifications of various palliative surgical options.
\efrey E . Sell, M D Department of Cardiovascular Surgery Children’s Hospital National Medical Center 111 Michigan Ave, NW Washington, DC 20010 Richard A. Jonas, M D Children‘s Hospital 300 Longwood Ave Boston. M A 02225
References Iwahara M, Ino T, Nishimoto K, et al. Clinical features of aortic arch anomaly with malalignment ventricular septal defect. Ann Thorac Surg 1989;48:693-6. Sell JE, Jonas RA, Mayer JE, Blackstone EH, Kirklin JW, Castaneda AR. The results of a surgical program for interrupted aortic arch. J Thorac Cardiovasc Surg 1988;96:864-77. Jonas RA, Sell JE, Van Praagh R, et al. Left ventricular outflow obstruction in interrupted aortic arch and ventricular septal defect. In: Crupi G, Parenzan L, Anderson RH, eds. Perspectives in pediatric cardiology. New York: Futura, 1989:61-5.
Reply
To the Editor:
The reason why our variable did not fit into their series may be related to the incidence of posterior deviation of infundibular septum as a cause of left ventricular outflow tract obstruction. Sennari [ l ] reported the high incidence of posterior deviation of
855
infundibular septum in Japanese patients with aortic arch anomaly and VSD, whereas Anderson and associates [2]reported a lower incidence of posterior deviation of infundibular septum in their series. In fact, all patients with malalignment VSD in our series had associated posterior deviation of infundibular septum. Because our variable was made based on this patient setting, we believe it fits the patients with posterior deviation of infundibular septum in the series of Sell and associates [3]. The arterial pH indicated in our paper was measured on admission, not immediately before surgical repair. Of course severe acidemia in the high-mortality group was corrected by administration of prostaglandin E,, inotropic agents, diuretics, or mechanical ventilation until surgical repair. We also believe as they do that aggressive resuscitation before operation would improve the outcome of operation. Generally, the mortality rate in patients with aortic arch anomaly and VSD is still high [4], although Sell and associates [3] showed good results and the possibility of one-stage operation to become the best choice for patients with aortic arch anomaly and VSD. One-stage operation may become the best choice as they recommended if reasonable risk can be demonstrated, but the optimal surgical management of severe left ventricular outflow tract obstruction in patients with this anomaly has not been generally agreed on. Some variable to define the severity of left ventricular outflow tract obstruction is still necessary to raise the survival rate. We believe our variable can help in the management of this anomaly until the mortality rate of one-stage operation becomes low at most of the main centers in the world. At least one should consider the severity of left ventricular outflow tract obstruction before operation, whichever operation is chosen.
Masazumi Iwahara, M D Toshihiro [no, M D Keijiro Yabuta, M D Department of Pediatrics Iuntendo University School of Medicine 2-1-1, Hongo, Bunkyo-ku Tokyo 113, Japan
References 1. Sennari E. Morphological study of ventricular septal defect associated with obstruction of aortic arch among Japanese. Jpn Circ J 1985;49:61-7. 2. Anderson RH, Clenox C, Zuberbuhler JR. Morphology of ventricular septal defect associated with coarctation of aorta. Br Heart J 1983;50:176-81. 3. Sell JE, Jonas RA, Mayer JE, Blackstone EH, Kirklin JW, Castaneda AR. The results of a surgical program for interrupted aortic arch. J Thorac Cardiovasc Surg 1988;96:8&77. 4. Trinquet F, Vouhe PR, Vernant F. Coarctation of the aorta in infants: which operation? Ann Thorac Surg 1988;45:186-91.
Diaphragmatic Plication To the Editor: The recent paper by Graham and collaborators [ l ] clearly indicates the interest in diaphragmatic plication for unilateral diaphragmatic paralysis. It should be remembered that paralysis and so-called eventration have the same aspects and provoke the same physiological disturbances, and that it is difficult to distinguish one from the other, even at thoracotomy or on biopsies. This explains controversies on the subject.
Valve Insufficiency After Atrioventricular Septal Defect Repair: Differences Between Patients With and Without Down’s Syndrome? To the Editor: In a recent issue of The Annals 1 read the interesting paper of Abbruzzese and associates [ 11 on the anatomical and surgical determinants of late mitral valve insufficiency after repair of partial atrioventricular canal. Unfortunately, Abbruzzese and associates did not report any data about the presence of Down’s syndrome in their patients. How many of the 9 patients that underwent reoperation for mitral dysfunction had Down’s syndrome? Was there a difference between children with and without Down’s syndrome in their series? Because increasing attention is being paid to the anatomical [2, 31 and surgical [4, 51 differences between Down’s and non-Down’s populations with atrioventricular canal, 1 believe that these data must be analyzed and described even if not statistically significant.
Bruno Marino, M D Dipart imento Medico-Ch ir u rgico di Cardiolog ia Pedia t rica Ospedale Bambino Gesli Rome, Italy
References 1. Abbruzzese PA, Napoleone A, Bini M, Annecchino FP, Merlo M, Parenzan L. Late left atrioventricular valve insufficiency after repair of partial atrioventricular septal defects: anatomical and surgical determinants. Ann Thorac Surg 1990;49: 1114. 2. Penkoske PA, Neches WH, Anderson RH, Zuberbuhler JR. Further observations on the morphology of atrioventricular septal defects. J Thorac Cardiovasc Surg 1985;90:611-22. 3. De Biase L, Di Ciommo V, Ballerini L, Bevilacqua M, Marcelletti C, Marino B. Prevalence of left-sided obstructive lesions in patients with atrioventricular canal without Down’s syndrome. J Thorac Cardiovasc Surg 1986;91:467-72. 4. Williams WH, Perrella AM, Plauth WH Jr, Hatcher CR Jr, Guyton RA. Survival following repair of complete A-V canal defects associated with the Down syndrome. 1st World Congress of Pediatric Cardiac Surgery, Bergamo, Italy, June 1988:38. 5. Vet TW, Ottenkamp J. Correction of atrioventricular septal defect. Am J Dis Child 1989;143:1361-5.
Reply
To the Editor:
The focus of our paper was on anatomical and surgical factors possibly associated with late left atrioventricular (AV) valve insufficiency after repair of partial AV septal defects. However, we are aware that Dr Marino’s group is investigating the proposition that AV septal defects may be associated with a worse prognosis in children without chromosomal abnormalities, and we will gladly answer his questions. None of the 9 patients who required reoperation in our series had Down’s syndrome. Also, only 1 of the 16 patients (6.25%) in whom late left AV valve insufficiency developed had Down’s syndrome. These figures show a lower incidence than that of Down’s syndrome in our total series of partial AV septal defects (16 of 107 patients = 14.95%). These differences do not reach statistical significance ( p = 0.45 and 0.58, respectively). However, to correctly interpret these data, one must keep in mind that 15 patients, in whom the partial AV septal defect was 0 1990 by The Society of Thoracic Surgeons
part of a more complex malformation, were excluded from our study; none of them had Down‘s syndrome. Interestingly, similar to the findings of the group from the Ospedale Bambino Gesu 111, this series includes 3 patients with aortic coarctation and 2 patients with right ventricular dominance. Moreover, when reviewing our total experience with reoperations for left AV valve repair failure in AV septal defects (all types) we noticed a very low incidence of Down’s syndrome (2 of 22 = 9.1%) as compared with the 49% incidence of Down’s syndrome in the total group of AV septal defects (all types). This difference is highly significant ( p < 0.001). A specific search for differences in the severity of this malformation in patients with and without Down‘s syndrome was not performed in our center as we believe that AV septal defects must be repaired in patients with and without Down’s syndrome, regardless of their extent. More data are needed to confirm the controversial findings of Bull and associates [2] regarding an excellent life expectancy in complete AV septal defects with Down’s syndrome without surgical repair.
Pietro A. Abbruzzese, M D Alrssandra Napoleone, M D R. Margherita Bini, M D F . Paolo Annecchino, M D Maurizio Merlo, M D Lucio Parenzan, M D Divisione di Cardiochirurgia Ospedali Riuniti di Bergamo Bergamo, ltaly
References 1. De Biase L, Di Ciommo V, Ballerini L, Bevilacqua M, Marcelletti C, Marino B. Prevalence of left-sided obstructive lesions in patients with atrioventricular canal without Down’s syndrome. J Thorac Cardiovasc Surg 1986;91:467-72. 2. Bull C, Rigby ML, Shinebourne EA. Should management of complete atrioventricular canal defect be influenced by coexistent Down syndrome? Lancet 1985;1:1147-9.
Left Ventricular Outflow Tract Obstruction With Aortic Arch Anomalies and Ventricular Septal Defect To the Editor: Iwahara and colleagues [ l ] have recently suggested that patients with a ventricular septal defect (VSD) and either interrupted aortic arch or coarctation, who have a left ventricular outflow tract diameter to ascending aortic diameter ratio of less than 0.6, should not undergo a standard reparative procedure of VSD closure and correction of the arch anomaly. They have based their conclusion on 4 patients with interrupted aortic arch and 13 patients with coarctation and VSD. Three of 4 patients with a ratio less than 0.6 died, whereas 2 of 13 patients with a ratio greater than 0.6 died. They conclude that the presence of severe left ventricular outflow tract obstruction (as determined by a left ventricular outflow tract to aortic ratio of less than 0.6) “clearly necessitates modification of present surgical strategies.” Our recent review (21of 37 patients with interrupted aortic arch and VSD as the only associated anomaly has led us to an alternative conclusion regarding the clinical significance of left ventricular outflow tract obstruction. Only 2 of the 37 patients (5%)underwent modification of their initial procedure because of the presence of suspected severe subaortic stenosis. In 8 of the 37 patients, evidence of subaortic stenosis subsequently developed after repair. All cases became apparent within 3 years of repair and have been treated successfully by conventional methods. In Ann Thorac Surg 1990;50:854-60
0003-4975/90/$3.50
CORRESPONDENCE
Ann Thorac Surg 1990;50:854-64
spite of this conservative approach to the management of subaortic stenosis with interrupted aortic arch, there has been a dramatic improvement in survival after one-stage reparative operation, so that by 1986 the probability of death within 2 weeks of repair was 7%. Retrospective echocardiographic analysis of the left ventricular outflow tract diameter did not reveal this variable to be a useful predictor of outcome in our series. Neonates with a left ventricular outflow tract diameter as small as 3 mm have done well, whereas in others with greater diameters left ventricular outflow tract obstruction has subsequently developed. We have speculated that VSD closure results in a shift in interventricular septal position resulting in an increase in the effective diameter of the left ventricular outflow tract, perhaps analagous to the situation with left ventricular outflow tract obstruction and transposition after an arterial switch procedure. One probable predictor of subsequent development of left ventricular outflow tract obstruction was found to be the presence of an aberrant right subclavian artery arising from the descending thoracic aorta in patients with type B interrupted aortic arch [3]. The arterial pH immediately before surgical repair was an important predictor of subsequent outcome in our series. We note that the mean pH of patients in the high mortality group from Iwahara’s series was 7.26 with a base excess of -8.7. We believe that aggressive resuscitation of the neonate with interrupted aortic arch including not only the use of prostaglandin E, to maintain ductal patency but also ventilation with room air to improve systemic perfusion, appropriate use of inotropic agents and antibiotics, and probably most importantly, waiting an appropriate period of time so that all variables have normalized before proceeding to operation, are more likely to improve the outcome of operation rather than complex modifications of various palliative surgical options.
\efrey E . Sell, M D Department of Cardiovascular Surgery Children’s Hospital National Medical Center 111 Michigan Ave, NW Washington, DC 20010 Richard A. Jonas, M D Children‘s Hospital 300 Longwood Ave Boston. M A 02225
References Iwahara M, Ino T, Nishimoto K, et al. Clinical features of aortic arch anomaly with malalignment ventricular septal defect. Ann Thorac Surg 1989;48:693-6. Sell JE, Jonas RA, Mayer JE, Blackstone EH, Kirklin JW, Castaneda AR. The results of a surgical program for interrupted aortic arch. J Thorac Cardiovasc Surg 1988;96:864-77. Jonas RA, Sell JE, Van Praagh R, et al. Left ventricular outflow obstruction in interrupted aortic arch and ventricular septal defect. In: Crupi G, Parenzan L, Anderson RH, eds. Perspectives in pediatric cardiology. New York: Futura, 1989:61-5.
Reply
To the Editor:
The reason why our variable did not fit into their series may be related to the incidence of posterior deviation of infundibular septum as a cause of left ventricular outflow tract obstruction. Sennari [ l ] reported the high incidence of posterior deviation of
855
infundibular septum in Japanese patients with aortic arch anomaly and VSD, whereas Anderson and associates [2]reported a lower incidence of posterior deviation of infundibular septum in their series. In fact, all patients with malalignment VSD in our series had associated posterior deviation of infundibular septum. Because our variable was made based on this patient setting, we believe it fits the patients with posterior deviation of infundibular septum in the series of Sell and associates [3]. The arterial pH indicated in our paper was measured on admission, not immediately before surgical repair. Of course severe acidemia in the high-mortality group was corrected by administration of prostaglandin E,, inotropic agents, diuretics, or mechanical ventilation until surgical repair. We also believe as they do that aggressive resuscitation before operation would improve the outcome of operation. Generally, the mortality rate in patients with aortic arch anomaly and VSD is still high [4], although Sell and associates [3] showed good results and the possibility of one-stage operation to become the best choice for patients with aortic arch anomaly and VSD. One-stage operation may become the best choice as they recommended if reasonable risk can be demonstrated, but the optimal surgical management of severe left ventricular outflow tract obstruction in patients with this anomaly has not been generally agreed on. Some variable to define the severity of left ventricular outflow tract obstruction is still necessary to raise the survival rate. We believe our variable can help in the management of this anomaly until the mortality rate of one-stage operation becomes low at most of the main centers in the world. At least one should consider the severity of left ventricular outflow tract obstruction before operation, whichever operation is chosen.
Masazumi Iwahara, M D Toshihiro [no, M D Keijiro Yabuta, M D Department of Pediatrics Iuntendo University School of Medicine 2-1-1, Hongo, Bunkyo-ku Tokyo 113, Japan
References 1. Sennari E. Morphological study of ventricular septal defect associated with obstruction of aortic arch among Japanese. Jpn Circ J 1985;49:61-7. 2. Anderson RH, Clenox C, Zuberbuhler JR. Morphology of ventricular septal defect associated with coarctation of aorta. Br Heart J 1983;50:176-81. 3. Sell JE, Jonas RA, Mayer JE, Blackstone EH, Kirklin JW, Castaneda AR. The results of a surgical program for interrupted aortic arch. J Thorac Cardiovasc Surg 1988;96:8&77. 4. Trinquet F, Vouhe PR, Vernant F. Coarctation of the aorta in infants: which operation? Ann Thorac Surg 1988;45:186-91.
Diaphragmatic Plication To the Editor: The recent paper by Graham and collaborators [ l ] clearly indicates the interest in diaphragmatic plication for unilateral diaphragmatic paralysis. It should be remembered that paralysis and so-called eventration have the same aspects and provoke the same physiological disturbances, and that it is difficult to distinguish one from the other, even at thoracotomy or on biopsies. This explains controversies on the subject.