981
Archives ofDisease in Childhood 1992; 67: 981-984
ARCHIVES OF DISEASE IN CHILDHOOD The Journal of the British
Paedliatric
Association
Annotations Current
status
of definitive
surgery
for congenital heart disease
The past 30 years have witnessed major changes in surgical treatment of congenital heart disease (CHD). The early years of innovation have been superseded by a new era of scientific assessment enabling the surgeon and the cardiologist both to evaluate and develop therapeutic strategies for the different groups of patients with CHD. In considering the current status of surgery for CHD one should be clear that modern surgical strategies may involve staged procedures in order to achieve a definitive long term outcome. Current treatment protocols therefore demand total integration of the services provided by surgeons, paediatricians, and cardiologists and this 'team approach' remains the cornerstone of any successful surgical programme. The past decade has witnessed an increasing tendency to advocate 'definitive' operation where possible in neonatal or infant life. Thus, for instance, infants with ventricular septal defect and intractable cardiac failure can now undergo definitive surgical repair with a mortality approaching zero. In this brief review I will address the current status of 'definitive' surgery in this younger age group. In all instances, it must be remembered that growth of the patient will often necessitate some further surgical intervention, most commonly when artificial conduits are employed as part of the original reconstruction. The term definitive must therefore be used with caution and close cardiological supervision is mandatory for all patients who have undergone surgery for CHD in childhood.
neonates,3 similar to that reported by Brawn et al for this group of patients.4 With increasing experience, particularly in the management of variations in coronary artery anatomy, the current hospital mortality for patients with TGA and intact ventricular septum at our institution is 1 8%, while that of complex TGA, primarily determined by the nature of the associated lesions, is 5-5%. Most recently, increasing confidence in the arterial switch procedure has led to its application in more complex forms of transposition, for instance those associated with atresia of a single atrioventricular valve, in an attempt to avert the morbidity often previously seen after palliative procedures such as pulmonary artery banding. Although follow up of patients in most series is relatively short, it appears that the main postoperative problem in patients undergoing an arterial switch repair is the development of pulmonary artery stenosis. This occurs, in our experience, in 10% of patients and may be treated successfully in most cases by catheter balloon dilatation of the obstruction. In older patients in whom right ventricular dysfunction has developed many years after a previous Senning or Mustard operation, the arterial switch procedure may still be applicable. Before such an operation, however, the left ventricle must be conditioned to develop systemic levels of blood pressure and this may be achieved by pulmonary artery banding. At present the mortality and morbidity of this staged approach remains high.5
Transposition of the great arteries (TGA) Although it has been possible for many years to achieve good early results in surgery for TGA using an atrial correction in which systemic venous blood is directed by means of an intra-atrial baffle to the left ventricle and pulmonary artery (Senning or Mustard operation), the intermediate and long term results have revealed an unacceptable late morbidity. ' 2 The arterial switch operation, in which the aorta and pulmonary arteries are divided and retransposed to the anatomically normal position together with appropriate relocation of the coronary arteries, has thus become preferred treatment of choice for patients with TGA unless there is significant pulmonary or subpulmonary stenosis. Even when TGA is associated with other complex malformations, an arterial switch procedure should normally be undertaken at the time of repair of the associated cardiac or extra cardiac anomalies. Planche et al recorded a hospital mortality of 8-3% in
Atrioventricular septal defect While partial atrioventricular septal defect may be treated in a similar way to atrial septal defect (repair before school age), the treatment of complete atrioventricular septal defect poses a major surgical challenge. The risk of obstructive pulmonary vascular disease secondary to pulmonary hypertension is such that primary repair within the first year of life is advocated by most major centres. Current experience suggests that a hospital mortality of 3-10% should be expected.6 7 The reoperation rate, usually for residual ventricular septal defect or left atrioventricular valve incompetence is between 8 and 16%. These results are encourging when compared with the 20% mortality published in series prior to 1983.
Tetralogy of Fallot Controversy still remains over the optimal timing for
982
definitive surgery in tetralogy of Fallot.8 Both proponents of complete repair in infancy and two stage repair (initial shunt formation followed by later correction) cite a hospital mortality well below 5% with similarly low figures for late mortality. On the other hand, repair in early infancy is undoubtedly associated with a higher incidence of patch enlargement of the right ventricular outflow tract extending across the pulmonary valve annulus. This inevitably produces some degree of pulmonary valve incompetence. The long term sequelae of mild or moderate pulmonary incompetence do not appear to be troublesome at up to 20 years after repair of tetralogy of Fallot. However, the natural history of congenital isolated pulmonary valve incompetence would suggest that important right ventricular dysfunction may be expected after 20-30 years9 and this may be especially true when surgical repair has been performed through a right ventriculotomy incision. It is possible therefore that we may expect to see an increase in the number of young adults who have undergone repair of tetralogy of Fallot and who require later pulmonary valve insertion. Once again this emphasises the importance of adequate long term cardiological follow up of these patients. Pulmonary atresia Pulmonary atresia whether with intact ventricular septum or ventricular septal defect remains a difficult management problem. In patients with pulmonary atresia and intact ventricular septum initial palliation, usually by placement of a patch across the right ventricular outflow or systemic to pulmonary artery shunting, may permit later operative correction resulting in a heart with normal anatomical relationships. In many patients, however, a palliative atriopulmonary connection will remain the only definitive treatment option in the longer term. When pulmonary atresia is associated with ventricular septal defect, the pulmonary blood supply may originate in large part from abnormal major aortopulmonary collateral arteries (MAPCAs). In this situation definitive repair demands initial palliative procedures in which these abnormal MAPCAs are anastomosed together prior to a final biventricular corrective repair. Although the mortality and morbidity of these procedures has been high in the past, current experience suggests that excellent results can be achieved in this difficult group of patients provided they are entered into an appropriate surgical programme early in the course of their disease. 1112 Aortic arch anomalies and aortic coarctation Surgical repair of aortic coarctation is now well established whether this condition presents in neonatal life or beyond. Current experience suggests that, while balloon aortic dilatation may be applied to certain types of native aortic coarctation, the major role of this treatment lies in the treatment of aortic recoarctation. Present concepts favour concomitant primary repair of aortic arch anomalies such as interrupted aortic arch, usually by end to end anastomosis of the affected segments, and the associated intracardiac defects at the time of initial presentation. The hospital mortality in these complex cases still remains high however (10-20%) 13 and residual aortic narrowing or recoarctation will often require treatment by angioplasty.
Sethia
presenting with critical aortic stenosis will progress well after surgical valvotomy, the longer term outlook for some of these patients and for older patients with aortic valve disease remains uncertain. The morbidity attendant upon anticoagulation treatment in patients who undergo aortic valve replacement, with a mechanical prosthesis at a young age, detracts significantly from the merits of such operations. Experience with aortic homografts as valve substitutes in young children has been encouraging but these patients also face potentially hazardous reoperation as they grow older. More recently, the use of the pulmonary valve as an autograft aortic valve replacement has been recommended by Gerosa et al in the hope that this valve substitute will grow with the child and thereby minimise the longer term morbidity.'4 As yet, however, data on the growth of this valve substitute is available in only a very small number of patients and so the management of patients with aortic valve dysfunction remains problematical.
Conclusion It will be clear from these observations on a few selected conditions that definitive surgery for congenital heart disease can be considered for most situations with the aim of achieving normal atrial, ventricular, and great arterial connections and relationships. The team approach to the problems posed by patients with congenital heart disease encourages us to look forward to the next decade with optimism as we seek to develop and evaluate the role of definitive surgery in the palliation of entities such as hypoplastic left heart syndrome and other conditions where restitution of normal cardiac relationships remains an
impossibility. B SETHIA The Children's Hospital, Ladywood Middleway, Ladywood, Birmingham B16 8ET 1 Williams WG, Trusler GA, Kirklin JW, et al. Early and late results of a protocol for simple transposition leading to an atrial switch (Mustard) repair. J Thorac Cardiovasc Surg 1988;95:717-26. 2 Deanfield J, Camm J, Macartney F, et al. Arrhythmia and late mortality after Mustard and Senning operation for transposition of the great arteries.: an eight year prospective study. J Thorac Cardiovasc Surg 1988;96:569-76. 3 Planche C, Bruniaux J, Lacour-Gayet F, et al. Switch operation for transposition of the great arteries in neonates: a study of 120 patients. .7 7horac Cardiovasc Surg 1988;96:354-63. 4 Brawn WJ, Mee RBB. Early results for anatomic correction of transposition of the great arteries and for double outlet right ventricle with subpulmonary ventricular septal defect. J Thorac Cardiovasc Surg 1988;95:230-8. 5 Mee RBB. Severe right ventricular failure after Mustard or Senning operation. Two stage repair: pulmonary artery banding and switch. .7 7horac Cardiovasc Surg 1986;92:385-90. 6 Thies WR, Breymann T, Matthies W, et al. Primary repair of complete atrioventricular septal defect in infancy. Eurj Cardiothorac Surg 1991;5:571-4. 7 Weintraub RJ, Brawn WJ, Venables AW, et al. Patch repair of complete atrioventricular septal defect in the first year of life: results and sequential assessment of atrio-ventricular valve function. J 7horac Cardiovasc Surg 1990;99:
320-6.
8 Castaneda AR. Repair of tetralogy of Fallot. Current 9 10 11
12 13
Aortic valve stenosis This condition remains one of the more difficult management problems in the young patient. While most patients
14
Opiniont in Cardiologv
1987;2:847-53. Shimazaki Y, Blackstone EH, Kirklin JW, et al. The natural historv of isolated congenital pulmonary valve incompetence: surgical implications. 7'horac Cardiovasc Surg 1984;32:257-9. Puga SJ, Leoni FE, Julsrud PR, et al. Complete repair of pulmonarv atresia, ventricular septal defect, and severe peripheral arborisation abnormalities of the central pulmonary arteries: experience with preliminary unifocalisation procedures in 38 patients. J Thorac Cardiovasc Surg 1989;98:1018-29. Sawatari K, Imai Y, Kurosawa H, et al. Staged operation for pulmonary atresia and ventricular septal defect with major aorto-pulmonary collateral arteries. New technique for complete unifocalisation. j Thorac Cardiovasc Surg 1989; 98:738-50. Iyer KS, Mee RBB. Stage repair of pulmonary atresia with ventricular septal defect and major systemic to pulmonary artery collaterals. Ann 7horac Surg 1991 ;51:65-72. Ha;zekamp MG, Quaegebeur JM, Singh 5, et at. One stage repair of aortic arch anomalies and intra-cardiac defects. Eur .7 C'ardiothorac KSurg: 1991 ;5: 283-7. Gerosa G, McKay R, Davis J, et at. Comparison of the aortic homograft and the pulmonary autograft for aortic valve or root replacement in children. ,7 Tlhorac CJardivasc S'urg 19911;102:5 1-61.
Archives ofDisease in Childhood 1992; 67: 981-984
ARCHIVES OF DISEASE IN CHILDHOOD The Journal of the British
Paedliatric
Association
Annotations Current
status
of definitive
surgery
for congenital heart disease
The past 30 years have witnessed major changes in surgical treatment of congenital heart disease (CHD). The early years of innovation have been superseded by a new era of scientific assessment enabling the surgeon and the cardiologist both to evaluate and develop therapeutic strategies for the different groups of patients with CHD. In considering the current status of surgery for CHD one should be clear that modern surgical strategies may involve staged procedures in order to achieve a definitive long term outcome. Current treatment protocols therefore demand total integration of the services provided by surgeons, paediatricians, and cardiologists and this 'team approach' remains the cornerstone of any successful surgical programme. The past decade has witnessed an increasing tendency to advocate 'definitive' operation where possible in neonatal or infant life. Thus, for instance, infants with ventricular septal defect and intractable cardiac failure can now undergo definitive surgical repair with a mortality approaching zero. In this brief review I will address the current status of 'definitive' surgery in this younger age group. In all instances, it must be remembered that growth of the patient will often necessitate some further surgical intervention, most commonly when artificial conduits are employed as part of the original reconstruction. The term definitive must therefore be used with caution and close cardiological supervision is mandatory for all patients who have undergone surgery for CHD in childhood.
neonates,3 similar to that reported by Brawn et al for this group of patients.4 With increasing experience, particularly in the management of variations in coronary artery anatomy, the current hospital mortality for patients with TGA and intact ventricular septum at our institution is 1 8%, while that of complex TGA, primarily determined by the nature of the associated lesions, is 5-5%. Most recently, increasing confidence in the arterial switch procedure has led to its application in more complex forms of transposition, for instance those associated with atresia of a single atrioventricular valve, in an attempt to avert the morbidity often previously seen after palliative procedures such as pulmonary artery banding. Although follow up of patients in most series is relatively short, it appears that the main postoperative problem in patients undergoing an arterial switch repair is the development of pulmonary artery stenosis. This occurs, in our experience, in 10% of patients and may be treated successfully in most cases by catheter balloon dilatation of the obstruction. In older patients in whom right ventricular dysfunction has developed many years after a previous Senning or Mustard operation, the arterial switch procedure may still be applicable. Before such an operation, however, the left ventricle must be conditioned to develop systemic levels of blood pressure and this may be achieved by pulmonary artery banding. At present the mortality and morbidity of this staged approach remains high.5
Transposition of the great arteries (TGA) Although it has been possible for many years to achieve good early results in surgery for TGA using an atrial correction in which systemic venous blood is directed by means of an intra-atrial baffle to the left ventricle and pulmonary artery (Senning or Mustard operation), the intermediate and long term results have revealed an unacceptable late morbidity. ' 2 The arterial switch operation, in which the aorta and pulmonary arteries are divided and retransposed to the anatomically normal position together with appropriate relocation of the coronary arteries, has thus become preferred treatment of choice for patients with TGA unless there is significant pulmonary or subpulmonary stenosis. Even when TGA is associated with other complex malformations, an arterial switch procedure should normally be undertaken at the time of repair of the associated cardiac or extra cardiac anomalies. Planche et al recorded a hospital mortality of 8-3% in
Atrioventricular septal defect While partial atrioventricular septal defect may be treated in a similar way to atrial septal defect (repair before school age), the treatment of complete atrioventricular septal defect poses a major surgical challenge. The risk of obstructive pulmonary vascular disease secondary to pulmonary hypertension is such that primary repair within the first year of life is advocated by most major centres. Current experience suggests that a hospital mortality of 3-10% should be expected.6 7 The reoperation rate, usually for residual ventricular septal defect or left atrioventricular valve incompetence is between 8 and 16%. These results are encourging when compared with the 20% mortality published in series prior to 1983.
Tetralogy of Fallot Controversy still remains over the optimal timing for
982
definitive surgery in tetralogy of Fallot.8 Both proponents of complete repair in infancy and two stage repair (initial shunt formation followed by later correction) cite a hospital mortality well below 5% with similarly low figures for late mortality. On the other hand, repair in early infancy is undoubtedly associated with a higher incidence of patch enlargement of the right ventricular outflow tract extending across the pulmonary valve annulus. This inevitably produces some degree of pulmonary valve incompetence. The long term sequelae of mild or moderate pulmonary incompetence do not appear to be troublesome at up to 20 years after repair of tetralogy of Fallot. However, the natural history of congenital isolated pulmonary valve incompetence would suggest that important right ventricular dysfunction may be expected after 20-30 years9 and this may be especially true when surgical repair has been performed through a right ventriculotomy incision. It is possible therefore that we may expect to see an increase in the number of young adults who have undergone repair of tetralogy of Fallot and who require later pulmonary valve insertion. Once again this emphasises the importance of adequate long term cardiological follow up of these patients. Pulmonary atresia Pulmonary atresia whether with intact ventricular septum or ventricular septal defect remains a difficult management problem. In patients with pulmonary atresia and intact ventricular septum initial palliation, usually by placement of a patch across the right ventricular outflow or systemic to pulmonary artery shunting, may permit later operative correction resulting in a heart with normal anatomical relationships. In many patients, however, a palliative atriopulmonary connection will remain the only definitive treatment option in the longer term. When pulmonary atresia is associated with ventricular septal defect, the pulmonary blood supply may originate in large part from abnormal major aortopulmonary collateral arteries (MAPCAs). In this situation definitive repair demands initial palliative procedures in which these abnormal MAPCAs are anastomosed together prior to a final biventricular corrective repair. Although the mortality and morbidity of these procedures has been high in the past, current experience suggests that excellent results can be achieved in this difficult group of patients provided they are entered into an appropriate surgical programme early in the course of their disease. 1112 Aortic arch anomalies and aortic coarctation Surgical repair of aortic coarctation is now well established whether this condition presents in neonatal life or beyond. Current experience suggests that, while balloon aortic dilatation may be applied to certain types of native aortic coarctation, the major role of this treatment lies in the treatment of aortic recoarctation. Present concepts favour concomitant primary repair of aortic arch anomalies such as interrupted aortic arch, usually by end to end anastomosis of the affected segments, and the associated intracardiac defects at the time of initial presentation. The hospital mortality in these complex cases still remains high however (10-20%) 13 and residual aortic narrowing or recoarctation will often require treatment by angioplasty.
Sethia
presenting with critical aortic stenosis will progress well after surgical valvotomy, the longer term outlook for some of these patients and for older patients with aortic valve disease remains uncertain. The morbidity attendant upon anticoagulation treatment in patients who undergo aortic valve replacement, with a mechanical prosthesis at a young age, detracts significantly from the merits of such operations. Experience with aortic homografts as valve substitutes in young children has been encouraging but these patients also face potentially hazardous reoperation as they grow older. More recently, the use of the pulmonary valve as an autograft aortic valve replacement has been recommended by Gerosa et al in the hope that this valve substitute will grow with the child and thereby minimise the longer term morbidity.'4 As yet, however, data on the growth of this valve substitute is available in only a very small number of patients and so the management of patients with aortic valve dysfunction remains problematical.
Conclusion It will be clear from these observations on a few selected conditions that definitive surgery for congenital heart disease can be considered for most situations with the aim of achieving normal atrial, ventricular, and great arterial connections and relationships. The team approach to the problems posed by patients with congenital heart disease encourages us to look forward to the next decade with optimism as we seek to develop and evaluate the role of definitive surgery in the palliation of entities such as hypoplastic left heart syndrome and other conditions where restitution of normal cardiac relationships remains an
impossibility. B SETHIA The Children's Hospital, Ladywood Middleway, Ladywood, Birmingham B16 8ET 1 Williams WG, Trusler GA, Kirklin JW, et al. Early and late results of a protocol for simple transposition leading to an atrial switch (Mustard) repair. J Thorac Cardiovasc Surg 1988;95:717-26. 2 Deanfield J, Camm J, Macartney F, et al. Arrhythmia and late mortality after Mustard and Senning operation for transposition of the great arteries.: an eight year prospective study. J Thorac Cardiovasc Surg 1988;96:569-76. 3 Planche C, Bruniaux J, Lacour-Gayet F, et al. Switch operation for transposition of the great arteries in neonates: a study of 120 patients. .7 7horac Cardiovasc Surg 1988;96:354-63. 4 Brawn WJ, Mee RBB. Early results for anatomic correction of transposition of the great arteries and for double outlet right ventricle with subpulmonary ventricular septal defect. J Thorac Cardiovasc Surg 1988;95:230-8. 5 Mee RBB. Severe right ventricular failure after Mustard or Senning operation. Two stage repair: pulmonary artery banding and switch. .7 7horac Cardiovasc Surg 1986;92:385-90. 6 Thies WR, Breymann T, Matthies W, et al. Primary repair of complete atrioventricular septal defect in infancy. Eurj Cardiothorac Surg 1991;5:571-4. 7 Weintraub RJ, Brawn WJ, Venables AW, et al. Patch repair of complete atrioventricular septal defect in the first year of life: results and sequential assessment of atrio-ventricular valve function. J 7horac Cardiovasc Surg 1990;99:
320-6.
8 Castaneda AR. Repair of tetralogy of Fallot. Current 9 10 11
12 13
Aortic valve stenosis This condition remains one of the more difficult management problems in the young patient. While most patients
14
Opiniont in Cardiologv
1987;2:847-53. Shimazaki Y, Blackstone EH, Kirklin JW, et al. The natural historv of isolated congenital pulmonary valve incompetence: surgical implications. 7'horac Cardiovasc Surg 1984;32:257-9. Puga SJ, Leoni FE, Julsrud PR, et al. Complete repair of pulmonarv atresia, ventricular septal defect, and severe peripheral arborisation abnormalities of the central pulmonary arteries: experience with preliminary unifocalisation procedures in 38 patients. J Thorac Cardiovasc Surg 1989;98:1018-29. Sawatari K, Imai Y, Kurosawa H, et al. Staged operation for pulmonary atresia and ventricular septal defect with major aorto-pulmonary collateral arteries. New technique for complete unifocalisation. j Thorac Cardiovasc Surg 1989; 98:738-50. Iyer KS, Mee RBB. Stage repair of pulmonary atresia with ventricular septal defect and major systemic to pulmonary artery collaterals. Ann 7horac Surg 1991 ;51:65-72. Ha;zekamp MG, Quaegebeur JM, Singh 5, et at. One stage repair of aortic arch anomalies and intra-cardiac defects. Eur .7 C'ardiothorac KSurg: 1991 ;5: 283-7. Gerosa G, McKay R, Davis J, et at. Comparison of the aortic homograft and the pulmonary autograft for aortic valve or root replacement in children. ,7 Tlhorac CJardivasc S'urg 19911;102:5 1-61.
