Adv. Cardiol., vol. 20, pp.llO-116 (Karger, Basel 1977)
Persistent Abnormalities after Repair of Congenital Heart Defects Ventricular Septal Defect and Tetralogy of Fallot
ALDO R. CASTANEDA and AMNON ROSENTHAL Children's Hospital Medical Center, Boston, Mass.
The objective of surgery in infants and children with congenital heart disease (CHD) is not only to repair the anatomic and hemodynamic abnormalities at a low operative mortality and morbidity, but also to thwart the adverse effects of CHD on the physical, psychological, and intellectual development of the child. Currently, approximately 80% of all patients with CHD can be treated surgically. Despite impressive progress in the surgical management of CHD over the last two decades, we must conclude that only a few operations are, in a strict sense, curative. In most patients a quasinormal anatomy or circulation is restored after surgery. Despite dramatic relief from symptoms, some patients remain with significant anatomic or hemodynamic defects. Minor residual abnormalities are common. A comprehensive review of postoperative residua, sequelae, and complications of the many types of CHD has recently been published [I] and is beyond the scope of this presentation. We, therefore, elected to discuss late postoperative events seen after repair of two of the most common defects: ventricular septal defect (VSD) and tetralogy of Fallot (TOF).
In a recent review of 586 autopsies on patients dying from CHD from 1966 to 1974 at the Children's Hospital Medical Center, VAN PRAAGH and VISNER [2] found that 41 % of the CHD autopsies were medical patients, and 59% were surgical patients. Of the postoperative CHD autopsies, 92% (320 cases) were related to hospital mortality, while only 8% (28 cases) occurred late. Of 348 patients who had operations for TOF during that same period
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 2:53:10 AM
Late Deaths
CASTANEDA/RoSENTHAL
111
oftime, 10 died from 4 months to 19 years after surgery. Six ofthese patients died after palliative surgery, and 4 after repair. Two patients died from malfunctioning pacemakers, and 2 from recurrent ventricular tachycardia. Reoperation for significant VSD and/or pulmonary stenosis, sudden death in association with conduction abnormalities, and even constrictive pericarditis have all been reported. Causes for late death after surgical closure of VSD include permanent complete heart block, reoperation for residual VSD, and tricuspid regurgitation [3]. With improvement in surgical techniques, the number of deaths related to these three causes has now been greatly reduced. Progressive pulmonary vascular obstructive disease, ventricular arrhythmias, and infective endocarditis may also result in late death. The autopsy survey indicates that death from surgery for CHD is related principally to hospital mortality rather than to late mortality.
Growth disturbance is a major consequence of CHD, particularly early in life [4]. Severity of growth inhibition is dependent upon the type of cardiac lesion and its hemodynamic effects. The undernutrition may partially be due to inadequate intake and, more importantly, to large substrate requirements associated with increased cardiopulmonary work. Early correction of the hemodynamic abnormality by surgery usually results in acceleration of growth rate, catch-up growth, and subsequent return to normal dimensions, while prolonged duration of failure to thrive or failure to correct the underlying cause in the first few years may lead to permanent and significant growth retardation. In our experience, the most startling increase in weight and height postoperatively is seen in infants with chronic congestive heart failure and VSD. Patients with TOF usually have only a moderate growth disturbance. Chronic prolonged hypoxemia has been shown to be associated with a lower perceptual and intelligence quotient, and improvement in psychological performance has been demonstrated after surgery [5]. FYLER et al. [6] recently reported on 126 children with CHD who were hospitalized during the first year oflife. Surgery was performed on 85 of the 126 patients. VSD was present in 23 and TOF in 18. The cumulative distribution of median IQ ofthe entire group was similar to popUlation norm. These data suggest that patients treated by present methods may be expected to have, on the average, a normal IQ. Concern has been expressed about the possible effect of deep hypothermia and circulatory arrest on the central nervous system. The limited post opera-
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 2:53:10 AM
Growth and Development
CASTANEDA/RoSENTHAL
112
tive studies available have shown that significant neurological damage or deficit is uncommon [7].
Ventricular Septal Defect The functional results in the great majority of patients after successful closure of a VSD are excellent. However, a small number of patients remain with anatomic, hemodynamic, and electrophysiologic impairments. The most vexing problem is presented by patients with VSD who preoperatively have elevated pulmonary artery pressures and resistances, and in whom the pulmonary artery pressure and resistance falls only slightly after closure of VSD, remains unchanged, or indeed continues to rise in the presence of a closed VSD. In general, normal postoperative pulmonary resistances are almost uniformly seen in patients less than 3 years of age, indicating a greater reversibility of pulmonary arteriolar changes early in life, while pulmonary resistance tends to remain high in patients older than 3 years at the time of surgery [8]. Pulmonary artery banding, in the past, has effectively decreased pulmonary pressure and pulmonary vascular resistance. Because of the mortality and complications inherent in pulmonary artery banding, and also because of the considerations outlined in the seotion on growth and development, we now recommend primary transatrial closure in infants with VSD and intractable congestive heart failure, persistent pulmonary hypertension, or both, irrespective of age or weight [9]. So far, 42 infants ranging in age from 14 to 364 days have been operated upon utilizing deep hypothermic circulatory arrest. The 3 operative deaths (7%) occurred in infants whose weight was less than 2.5 kg at the time of surgery. Only 1 of9 infants catheterized 1 year or more after surgery had a residual VSD. Pulmonary pressure, pulmonary vascular resistance, and LV function were normal in all the patients. Of interest is our recent awareness that a variety of cardiac abnormali-
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 2:53:10 AM
Infective Endocarditis Infective endocarditis may develop in children with CHD after procedures which commonly lead to bacteremia. Palliative cardiovascular surgery does not diminish the susceptibility to infective endocarditis. However, few data are available on endocarditis after successful surgical repair. We have, to date, not encountered any cases of endocarditis beyond the first 6 months after surgery in patients with completely closed VSD or good repair of TOF. It would seem advisable, however, until more information becomes available, to administer antimycrobial prophylaxis at times of predictable risk to all postoperative patients with VSD or TOF.
Persistent Abnormalities after Repair of Congenital Heart Defects
113
Tetralogy of Fallot Follow-up studies show that a majority of patients are relieved of their symptoms and enjoy general good health with educational and occupational opportunities equal to those of the general population. Some patients remain symptomatic and are found to have dysrhythmias or hemodynamically significant residual abnormalities. JORENSEN and MOLLER [10] in studying 120 survivors after corrective operation for cyanotic TOF found that 13 patients had significant residual shunts. Resting RV systolic pressure was less than 60 mm Hg in 88 patients, while in the remaining 32 patients R V systolic pressure ranged to 250 mm Hg. 34 patients were sUbjected to both rest and supine exercise testing. Only 4 of these 34 patients had resting R V systolic pressures greater than 60 mm Hg. 24 showed a normal response to exercise when changes in stroke index were related to right ventricular end-diastolic pressure. R Vend-diastolic pressure rose during exercise in 10 patients, 7 of whom had RV systolic pressure greater than 60 mm Hg. In 4 of these 10 patients the stroke index fell during exercise, indicating an abnormal ventricular response. The presence of mild to moderate impairment of LV function has been reported in TOF patients who had corrective surgery in late childhood or early adulthood. At our institution [11] LV function was assessed at cardiac catheterization in 41 postoperative patients with TOF aged 4-26 years, from 1 to 12 years after repair. 20 patients with good repair (pulmonary to systemic flow ratio 1. 5, RV peak pressure less than 2/3 systemic) had normal LV-end-diastolic volumes, ejection fractions, and muscle mass. By contrast, the patients with residual VSD and RV pressures greater than 2/3 systemic had significantly increased LV end-diastolic volumes and muscle mass, and
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 2:53:10 AM
ties may develop or become apparent subsequent to repair of VSD during infancy or early childhood. These include subaortic stenosis, coarctation of the aorta, aortic regurgitation, and anomalous muscle bundle of the right ventricle. With increase in survival of infants with repaired VSDs, it is likely that other hemodynamic abnormalities will be recognized. Right bundle branch block commonly develops after closure of a VSD. The coexistence of a left anterior hemiblock in approximately 5-15% of patients is worrisome, but its clinical significance remains uncertain. Death due to acute complete heart block following late involvement of the remaining posterior fasciculus has been reported [3]. Permanent postoperative complete heart block is now fortunately rare. In fact, of our last 150 consecutive patients with VSDs none developed permanent complete heart block after surgical closure.
114
decreased ejection fractions. SUNDERLAND et al. [12] have also shown normal values for LV function in 17 postoperative patients with TOF who had repair before 2 years of age. These studies suggest that suboptimal overall cardiac performance may be found in some patients after successful surgery, especially when surgery is performed at an older age. LV function is likely to be normal or minimally altered after successful repair in infancy or childhood. The most common residual abnormalities after repair of TO F include: intracardiac or extracardiac shunts, residual RV outflow gradients (RVOG) which are usually due to inadequate mobilization ofthe parietal band and/or perhaps more commonly due to inadequate judgment concerning the need for an outflow patch across the RV outflow tract [13]. In reviewing 55 patients who required reoperation after previous 'repair' for TOF, the most common lesion was both a residual VSD and an RVOG. Six patients had aneurysms of the RV outflow tract, all of whom had RVOG in excess of 60 mm Hg. Three of the 55 reoperated patients (7%) died in the hospital. Of the 52 surviving patients, 50 are asymptomatic. Pulmonary regurgitation is common (75-90%) after operation for TOF, particularly so after the use of outflow patches. Although, so far, we have not seen long-term ill-effects from pulmonary insufficiency, one cannot be complacent about these results since pulmonary hypertension and other hemodynamic alterations have been reported after long-standing pulmonary regurgitation. Surgery for TOF is required within the first year oflife in close to 60% of critically ill infants. Disenchanted with the high mortality and/or morbidity of palliative surgery, we are now correcting TOF in the symptomatic infants irrespective of weight or age [9]. To date, we have operated on 26 infants (age range 12 days to 11 months) under deep hypothermia with 2 deaths. Ten of these infants have had postoperative catheterization studies. None has a residual VSD, while 3 patients had a residual outflow gradient exceeding 50 mm Hg. In fact, 2 of these patients developed RV outflow tract aneurysms and required reoperation and placement of a transannular patch. Although more extensive long-term follow-up is not yet available, it is hoped that this one-stage repair of TOF in infants will not only avoid the undesirable complications of palliative shunt operations, the psychologic trauma, the financial burden, and the risk of a second operation, but will also lead to normal myocardial performance. In the repair of severe forms of TOF conduits, either homografts, aortic valves with segments of ascending aorta, or dacron tubes with a porcine aortic valve (Hancock) are now widely used to establish continuity between
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 2:53:10 AM
CASTA!'IEDA/RoSENTHAL
Persistent Abnormalities after Repair of Congenital Heart Defects
115
the R V and the pulmonary arteries. We recently reviewed our experience with 16 patients 1 month to 5 years after surgery [14]. All patients with an aortic homograft had severe obstruction and calcification of the graft. In 9 of the 12 patients with a Hancock conduit, there was only mild to moderate obstruction to pulmonary flow, while 3 patients had severe obstruction. Proximal obstruction to the conduit may be eliminated by preventing kinking of the dacron conduit as it exits from the RV. Distal obstruction, due to a narrowed or deformed main or right pulmonary artery by a previous systemic-to-pulmonary artery shunt, may also be prevented by an appropriate anastomosis or angioplasty. More difficult to overcome will be obstruction due to very small or hypoplastic pulmonary artery branches, or to those obstructions developing at the porcine valve level. Over the last few years, the decrease in operating mortality and morbidity following repair of congenital heart defects has been significant. Because of improvements in technique, the incidence of residual defects has also become progressively lower. We hope that the more recent emphasis on early onestage repair may further reduce the occurrence of late residua or sequelae.
References
2 3 4 5 6 7
8
9
SONNENBLICK, F.H. and LESCH, H. (eds): Symp. Postoperative Congenital Heart Disease. Prog. cardiovasc. Dis. 58: 1 (1975). VAN PRAAGH, R. and VISNER, M. S.: The patient with congenital heart disease after surgery: findings of cardiac pathology. Am. J. Cardiol. (in press.) ALLEN, H.D.; ANDERSON, R.C.; NOREN, G.R., and MOLLER, J.H.: Postoperative follow-up of patients with ventricular septal defect. Circulation 50: 465 (1974). ROSENTHAL, A. and CASTANEDA, A. R. : Growth and development after cardiovascular surgery in infants and children. Prog. cardiovasc. Dis. 58: 27 (1975). SILBERT, S.; WOLFF, P.H.; MAYER, B.; ROSENTHAL, A., and NADAS, A.S.: Cyanotic heart disease and psychological development. Pediatrics 43: 192 (1969). FYLER, D.C.; SILBERT, A.R., and ROTHMAN, K.J.: Five-year follow-up of infant cardiacs: intelligence quotient. Symp. Congenital Heart Disease, Toronto 1975. STEVENSON, G.J.; STOVE, E.F.; DILLARD, D.H., and MORGAN, B.: Intellectual development of children subjected to prolonged circulatory arrest during hypothermic heart surgery in infancy. Circulation 50: suppl. II, p.54 (1974). CASTANEDA, A.R.; ZAMORA, R.; NICOLOFF, D.M.; MOLLER, J.H.; HUNT, C.E., and LUCAS, R. V., jr.: High-pressure, high resistance ventricular septal defect: surgical results of closure through right atrium. Ann. thorac. Surg. 12: 1 (1971). CASTANEDA, A.R.; LAMBERTI, J.; SADE, R.M.; WILLIAMS, R.C., and NADAS, A.S.: Open-heart surgery during the first three months of life. J. thorac. cardiovasc. Surg. 68: 5 (1974).
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 2:53:10 AM
2
CASTANEDA/RoSENTHAL
II
12
I3 14
JORENSEN, J.A. and MOLLER, J.H.: Postoperative hemodynamic studies in tetralogy of Fallot. Circulation 40: 803 (1969). ROCCHrNI, A.P.; KEANE, J.F.; FREED, M.D.; CASTANEDA, A.R., and NADAS, A.S.: Left ventricular function after surgical repair of tetralogy of Fallot (abstr.). Am. J. Cardiol. (in press, 1976). SUNDERLAND, C.O.; MATARAZZO, R.G. ; LIES, M . H., et al.: Total correction of tetralogy of Fallot in infancy: postoperative hemodynamic evaluation. Circulation 18: 398 (1973). CASTANEDA, A. R.; SADE, R. A.; LAMBERTI, J., and NICOLOFF, D. M.: Residual defects after repair of tetralogy of Fallot. Surgery, St Louis 76: 1010 (1974). ROCCHINI, A. P.; ROSENTHAL, A.; KEANE, J.F.; CASTANEDA, A.R., and NADAS, A.S.: Hemodynamics after surgical repair with right ventricle to pulmonary artery conduit. Circulation 52: suppl.lI, p.102 (1975).
A. CASTANEDA, MD, Cardiovascular Surgeon-in-Chief, Children's Hospital Medical Center, 300 Longwood Ave., Boston, MA 02115 (USA)
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10
116
Persistent Abnormalities after Repair of Congenital Heart Defects Ventricular Septal Defect and Tetralogy of Fallot
ALDO R. CASTANEDA and AMNON ROSENTHAL Children's Hospital Medical Center, Boston, Mass.
The objective of surgery in infants and children with congenital heart disease (CHD) is not only to repair the anatomic and hemodynamic abnormalities at a low operative mortality and morbidity, but also to thwart the adverse effects of CHD on the physical, psychological, and intellectual development of the child. Currently, approximately 80% of all patients with CHD can be treated surgically. Despite impressive progress in the surgical management of CHD over the last two decades, we must conclude that only a few operations are, in a strict sense, curative. In most patients a quasinormal anatomy or circulation is restored after surgery. Despite dramatic relief from symptoms, some patients remain with significant anatomic or hemodynamic defects. Minor residual abnormalities are common. A comprehensive review of postoperative residua, sequelae, and complications of the many types of CHD has recently been published [I] and is beyond the scope of this presentation. We, therefore, elected to discuss late postoperative events seen after repair of two of the most common defects: ventricular septal defect (VSD) and tetralogy of Fallot (TOF).
In a recent review of 586 autopsies on patients dying from CHD from 1966 to 1974 at the Children's Hospital Medical Center, VAN PRAAGH and VISNER [2] found that 41 % of the CHD autopsies were medical patients, and 59% were surgical patients. Of the postoperative CHD autopsies, 92% (320 cases) were related to hospital mortality, while only 8% (28 cases) occurred late. Of 348 patients who had operations for TOF during that same period
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 2:53:10 AM
Late Deaths
CASTANEDA/RoSENTHAL
111
oftime, 10 died from 4 months to 19 years after surgery. Six ofthese patients died after palliative surgery, and 4 after repair. Two patients died from malfunctioning pacemakers, and 2 from recurrent ventricular tachycardia. Reoperation for significant VSD and/or pulmonary stenosis, sudden death in association with conduction abnormalities, and even constrictive pericarditis have all been reported. Causes for late death after surgical closure of VSD include permanent complete heart block, reoperation for residual VSD, and tricuspid regurgitation [3]. With improvement in surgical techniques, the number of deaths related to these three causes has now been greatly reduced. Progressive pulmonary vascular obstructive disease, ventricular arrhythmias, and infective endocarditis may also result in late death. The autopsy survey indicates that death from surgery for CHD is related principally to hospital mortality rather than to late mortality.
Growth disturbance is a major consequence of CHD, particularly early in life [4]. Severity of growth inhibition is dependent upon the type of cardiac lesion and its hemodynamic effects. The undernutrition may partially be due to inadequate intake and, more importantly, to large substrate requirements associated with increased cardiopulmonary work. Early correction of the hemodynamic abnormality by surgery usually results in acceleration of growth rate, catch-up growth, and subsequent return to normal dimensions, while prolonged duration of failure to thrive or failure to correct the underlying cause in the first few years may lead to permanent and significant growth retardation. In our experience, the most startling increase in weight and height postoperatively is seen in infants with chronic congestive heart failure and VSD. Patients with TOF usually have only a moderate growth disturbance. Chronic prolonged hypoxemia has been shown to be associated with a lower perceptual and intelligence quotient, and improvement in psychological performance has been demonstrated after surgery [5]. FYLER et al. [6] recently reported on 126 children with CHD who were hospitalized during the first year oflife. Surgery was performed on 85 of the 126 patients. VSD was present in 23 and TOF in 18. The cumulative distribution of median IQ ofthe entire group was similar to popUlation norm. These data suggest that patients treated by present methods may be expected to have, on the average, a normal IQ. Concern has been expressed about the possible effect of deep hypothermia and circulatory arrest on the central nervous system. The limited post opera-
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 2:53:10 AM
Growth and Development
CASTANEDA/RoSENTHAL
112
tive studies available have shown that significant neurological damage or deficit is uncommon [7].
Ventricular Septal Defect The functional results in the great majority of patients after successful closure of a VSD are excellent. However, a small number of patients remain with anatomic, hemodynamic, and electrophysiologic impairments. The most vexing problem is presented by patients with VSD who preoperatively have elevated pulmonary artery pressures and resistances, and in whom the pulmonary artery pressure and resistance falls only slightly after closure of VSD, remains unchanged, or indeed continues to rise in the presence of a closed VSD. In general, normal postoperative pulmonary resistances are almost uniformly seen in patients less than 3 years of age, indicating a greater reversibility of pulmonary arteriolar changes early in life, while pulmonary resistance tends to remain high in patients older than 3 years at the time of surgery [8]. Pulmonary artery banding, in the past, has effectively decreased pulmonary pressure and pulmonary vascular resistance. Because of the mortality and complications inherent in pulmonary artery banding, and also because of the considerations outlined in the seotion on growth and development, we now recommend primary transatrial closure in infants with VSD and intractable congestive heart failure, persistent pulmonary hypertension, or both, irrespective of age or weight [9]. So far, 42 infants ranging in age from 14 to 364 days have been operated upon utilizing deep hypothermic circulatory arrest. The 3 operative deaths (7%) occurred in infants whose weight was less than 2.5 kg at the time of surgery. Only 1 of9 infants catheterized 1 year or more after surgery had a residual VSD. Pulmonary pressure, pulmonary vascular resistance, and LV function were normal in all the patients. Of interest is our recent awareness that a variety of cardiac abnormali-
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 2:53:10 AM
Infective Endocarditis Infective endocarditis may develop in children with CHD after procedures which commonly lead to bacteremia. Palliative cardiovascular surgery does not diminish the susceptibility to infective endocarditis. However, few data are available on endocarditis after successful surgical repair. We have, to date, not encountered any cases of endocarditis beyond the first 6 months after surgery in patients with completely closed VSD or good repair of TOF. It would seem advisable, however, until more information becomes available, to administer antimycrobial prophylaxis at times of predictable risk to all postoperative patients with VSD or TOF.
Persistent Abnormalities after Repair of Congenital Heart Defects
113
Tetralogy of Fallot Follow-up studies show that a majority of patients are relieved of their symptoms and enjoy general good health with educational and occupational opportunities equal to those of the general population. Some patients remain symptomatic and are found to have dysrhythmias or hemodynamically significant residual abnormalities. JORENSEN and MOLLER [10] in studying 120 survivors after corrective operation for cyanotic TOF found that 13 patients had significant residual shunts. Resting RV systolic pressure was less than 60 mm Hg in 88 patients, while in the remaining 32 patients R V systolic pressure ranged to 250 mm Hg. 34 patients were sUbjected to both rest and supine exercise testing. Only 4 of these 34 patients had resting R V systolic pressures greater than 60 mm Hg. 24 showed a normal response to exercise when changes in stroke index were related to right ventricular end-diastolic pressure. R Vend-diastolic pressure rose during exercise in 10 patients, 7 of whom had RV systolic pressure greater than 60 mm Hg. In 4 of these 10 patients the stroke index fell during exercise, indicating an abnormal ventricular response. The presence of mild to moderate impairment of LV function has been reported in TOF patients who had corrective surgery in late childhood or early adulthood. At our institution [11] LV function was assessed at cardiac catheterization in 41 postoperative patients with TOF aged 4-26 years, from 1 to 12 years after repair. 20 patients with good repair (pulmonary to systemic flow ratio 1. 5, RV peak pressure less than 2/3 systemic) had normal LV-end-diastolic volumes, ejection fractions, and muscle mass. By contrast, the patients with residual VSD and RV pressures greater than 2/3 systemic had significantly increased LV end-diastolic volumes and muscle mass, and
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 2:53:10 AM
ties may develop or become apparent subsequent to repair of VSD during infancy or early childhood. These include subaortic stenosis, coarctation of the aorta, aortic regurgitation, and anomalous muscle bundle of the right ventricle. With increase in survival of infants with repaired VSDs, it is likely that other hemodynamic abnormalities will be recognized. Right bundle branch block commonly develops after closure of a VSD. The coexistence of a left anterior hemiblock in approximately 5-15% of patients is worrisome, but its clinical significance remains uncertain. Death due to acute complete heart block following late involvement of the remaining posterior fasciculus has been reported [3]. Permanent postoperative complete heart block is now fortunately rare. In fact, of our last 150 consecutive patients with VSDs none developed permanent complete heart block after surgical closure.
114
decreased ejection fractions. SUNDERLAND et al. [12] have also shown normal values for LV function in 17 postoperative patients with TOF who had repair before 2 years of age. These studies suggest that suboptimal overall cardiac performance may be found in some patients after successful surgery, especially when surgery is performed at an older age. LV function is likely to be normal or minimally altered after successful repair in infancy or childhood. The most common residual abnormalities after repair of TO F include: intracardiac or extracardiac shunts, residual RV outflow gradients (RVOG) which are usually due to inadequate mobilization ofthe parietal band and/or perhaps more commonly due to inadequate judgment concerning the need for an outflow patch across the RV outflow tract [13]. In reviewing 55 patients who required reoperation after previous 'repair' for TOF, the most common lesion was both a residual VSD and an RVOG. Six patients had aneurysms of the RV outflow tract, all of whom had RVOG in excess of 60 mm Hg. Three of the 55 reoperated patients (7%) died in the hospital. Of the 52 surviving patients, 50 are asymptomatic. Pulmonary regurgitation is common (75-90%) after operation for TOF, particularly so after the use of outflow patches. Although, so far, we have not seen long-term ill-effects from pulmonary insufficiency, one cannot be complacent about these results since pulmonary hypertension and other hemodynamic alterations have been reported after long-standing pulmonary regurgitation. Surgery for TOF is required within the first year oflife in close to 60% of critically ill infants. Disenchanted with the high mortality and/or morbidity of palliative surgery, we are now correcting TOF in the symptomatic infants irrespective of weight or age [9]. To date, we have operated on 26 infants (age range 12 days to 11 months) under deep hypothermia with 2 deaths. Ten of these infants have had postoperative catheterization studies. None has a residual VSD, while 3 patients had a residual outflow gradient exceeding 50 mm Hg. In fact, 2 of these patients developed RV outflow tract aneurysms and required reoperation and placement of a transannular patch. Although more extensive long-term follow-up is not yet available, it is hoped that this one-stage repair of TOF in infants will not only avoid the undesirable complications of palliative shunt operations, the psychologic trauma, the financial burden, and the risk of a second operation, but will also lead to normal myocardial performance. In the repair of severe forms of TOF conduits, either homografts, aortic valves with segments of ascending aorta, or dacron tubes with a porcine aortic valve (Hancock) are now widely used to establish continuity between
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 2:53:10 AM
CASTA!'IEDA/RoSENTHAL
Persistent Abnormalities after Repair of Congenital Heart Defects
115
the R V and the pulmonary arteries. We recently reviewed our experience with 16 patients 1 month to 5 years after surgery [14]. All patients with an aortic homograft had severe obstruction and calcification of the graft. In 9 of the 12 patients with a Hancock conduit, there was only mild to moderate obstruction to pulmonary flow, while 3 patients had severe obstruction. Proximal obstruction to the conduit may be eliminated by preventing kinking of the dacron conduit as it exits from the RV. Distal obstruction, due to a narrowed or deformed main or right pulmonary artery by a previous systemic-to-pulmonary artery shunt, may also be prevented by an appropriate anastomosis or angioplasty. More difficult to overcome will be obstruction due to very small or hypoplastic pulmonary artery branches, or to those obstructions developing at the porcine valve level. Over the last few years, the decrease in operating mortality and morbidity following repair of congenital heart defects has been significant. Because of improvements in technique, the incidence of residual defects has also become progressively lower. We hope that the more recent emphasis on early onestage repair may further reduce the occurrence of late residua or sequelae.
References
2 3 4 5 6 7
8
9
SONNENBLICK, F.H. and LESCH, H. (eds): Symp. Postoperative Congenital Heart Disease. Prog. cardiovasc. Dis. 58: 1 (1975). VAN PRAAGH, R. and VISNER, M. S.: The patient with congenital heart disease after surgery: findings of cardiac pathology. Am. J. Cardiol. (in press.) ALLEN, H.D.; ANDERSON, R.C.; NOREN, G.R., and MOLLER, J.H.: Postoperative follow-up of patients with ventricular septal defect. Circulation 50: 465 (1974). ROSENTHAL, A. and CASTANEDA, A. R. : Growth and development after cardiovascular surgery in infants and children. Prog. cardiovasc. Dis. 58: 27 (1975). SILBERT, S.; WOLFF, P.H.; MAYER, B.; ROSENTHAL, A., and NADAS, A.S.: Cyanotic heart disease and psychological development. Pediatrics 43: 192 (1969). FYLER, D.C.; SILBERT, A.R., and ROTHMAN, K.J.: Five-year follow-up of infant cardiacs: intelligence quotient. Symp. Congenital Heart Disease, Toronto 1975. STEVENSON, G.J.; STOVE, E.F.; DILLARD, D.H., and MORGAN, B.: Intellectual development of children subjected to prolonged circulatory arrest during hypothermic heart surgery in infancy. Circulation 50: suppl. II, p.54 (1974). CASTANEDA, A.R.; ZAMORA, R.; NICOLOFF, D.M.; MOLLER, J.H.; HUNT, C.E., and LUCAS, R. V., jr.: High-pressure, high resistance ventricular septal defect: surgical results of closure through right atrium. Ann. thorac. Surg. 12: 1 (1971). CASTANEDA, A.R.; LAMBERTI, J.; SADE, R.M.; WILLIAMS, R.C., and NADAS, A.S.: Open-heart surgery during the first three months of life. J. thorac. cardiovasc. Surg. 68: 5 (1974).
Downloaded by: University of Cambridge 131.111.164.128 - 3/19/2019 2:53:10 AM
2
CASTANEDA/RoSENTHAL
II
12
I3 14
JORENSEN, J.A. and MOLLER, J.H.: Postoperative hemodynamic studies in tetralogy of Fallot. Circulation 40: 803 (1969). ROCCHrNI, A.P.; KEANE, J.F.; FREED, M.D.; CASTANEDA, A.R., and NADAS, A.S.: Left ventricular function after surgical repair of tetralogy of Fallot (abstr.). Am. J. Cardiol. (in press, 1976). SUNDERLAND, C.O.; MATARAZZO, R.G. ; LIES, M . H., et al.: Total correction of tetralogy of Fallot in infancy: postoperative hemodynamic evaluation. Circulation 18: 398 (1973). CASTANEDA, A. R.; SADE, R. A.; LAMBERTI, J., and NICOLOFF, D. M.: Residual defects after repair of tetralogy of Fallot. Surgery, St Louis 76: 1010 (1974). ROCCHINI, A. P.; ROSENTHAL, A.; KEANE, J.F.; CASTANEDA, A.R., and NADAS, A.S.: Hemodynamics after surgical repair with right ventricle to pulmonary artery conduit. Circulation 52: suppl.lI, p.102 (1975).
A. CASTANEDA, MD, Cardiovascular Surgeon-in-Chief, Children's Hospital Medical Center, 300 Longwood Ave., Boston, MA 02115 (USA)
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10
116
