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Results of total correction of tetralogy of fallot performed in adults
UPDATE
As Originally Published in 1988:
Results of Total Correction of Tetralogy of Fallot Performed in Adults P. Presbitero, M.D., D. Demarie, M.D., E. Aruta, M.D., M. Villani, M.D., M. Disumma, M.D., G. M. Ottino, M.D., F. Orzan, M.D., A. Fubini, M.D., M. T. Spinnler, M.D., M. R. Conte, M.D., and M. Morea, M.D.
ABSTRACT Today, total correction of tetralogy of Fallot is rarely performed in adults. In a 10-year period, 40 patients aged 20 to 67 years underwent intracardiac repair in our institution. Twenty-eight of them had had a palliative procedure 11 to 30 years earlier. Preoperatively, 23 patients were in N e w York Heart Association (NYHA) Functional Class II, 14 were in Class lIl, and 3 were in Class IV. Operative mortality was 2.5% (1/40). Follow-up ranged from I year to 11 years (average, 3 years). One patient died of a noncardiac cause 4 years after operation. Residual cardiac defects were observed in 4 patients. Postoperatively,
30 patients were in NYHA Functional Class I, 8 were in Class II, and 1 was in Class III. Major ventricular arrhythmias were recorded in 7 (35%) of 20 patients. Radionuclide angiography demonstrated impaired right ventricular function in 8 patients. Left ventricular impairment was present in 2. Total correction of tetralogy of Fallot can be performed safely in adults with low mortality and good functional improvement. The incidence of residual cardiac defects is low. The long-term importance of impaired ventricular function and arrhythmias remains to be ascertained.
Updated in 1996 by Patrizia Presbitero, MD, Silvia Brach Prever, MD, Igino Contrafatto, MD, and Mario Morea, MD Divisione di Cardiologia, Ospedale G. Bosco, Torino, Italy
lthough most patients with tetralogy of Fallot have radical repair ("total correction") during infancy or childhood, some patients reach adult life without such an operation. This may occur when a previous palliative shunt has been performed, or when there is natural collateral circulation to the lung, or when a mild form of the disease is present. The long-term follow-up for this operation performed in adults is less well-known and may be suspected to differ significantly from those in younger patients. Accordingly, we present the long-term follow-up of a series of 40 patients with tetralogy of Fallot who had radical repair at age 20 years or older. Their clinical outcome and factors affecting their survival are analyzed. In 1988 [1] we reported the immediate and short-term results of the same group of patients.
A
Material and M e t h o d s The original study group consisted of 40 consecutive patients with tetralogy of Fallot who had intracardiac repair, from 1974 to 1985, in the Cardiac Surgery Department, University of Torino. The patients' age at operation ranged from 20 to 67 years (mean, 30 years); 22 were men and 18 were women. A previous palliative operation had Address reprint requests to Dr Presbitero, PiazzaDonatoridel Sangue3, 10154Torino, Italy. © 1996 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
been performed in 28 patients (70%). Preoperative findings, operative procedures, and short-term follow-up have been described in the previous report in 1988 [1]. The hospital mortality was 2.5% (1140), and the 39 survivors were followed by either outpatient clinic visit (22 patients) or telephone conversation with the patient or the attending physician (10 patients). We were unable to determine the current status of 7 patients, including the oldest patient (67 years of age), but the hospital records indicated that they were alive 10 years after the operation. Follow-up in surviving patients ranged from 10 to 20 years (mean, 13 years). Patients who returned for a clinical visit were evaluated with physical examination, resting electrocardiogram, chest roentgenography, and two-dimensional Doppler echocardiography. Fourteen patients had postoperative cardiac catheterization and angiography. Holter monitoring was performed in 20 patients, and ventricular arrhythmias were classified according to the criteria of Lown. Results
Late Deaths Four of the 39 operative survivors had died by the last assessment in June 1995, representing an overall mortalAnn Thorac Surg 1996;61:1870-3 ° 0003-4975/96/$15.00 PII S0003-4975(96)00180-4
A n n Thorac Surg 1996;61:1870-3
ity rate for all causes of 0.01 death/patient-year. One of these patients died of a non-cardiac-related cause 4 years after complete repair, namely, massive hemorrhage in the postpartum period. Three more cardiovascularrelated deaths occurred 9, 10, and 13 years after total surgical repair. Two of the three were sudden: one occurred at home in a 48-year-old patient with a previous brain abscess, 9 years after operation. The second patient died suddenly and unexpectedly at age 36 years, 10 years after complete repair; he had had a previous Blalock shunt at age 10 years, and had residual moderate regurgitation of the aortic and tricuspid valves. His electrocardiogram showed left-axis deviation and right bundlebranch block, and postoperative Holter monitoring revealed ventricular arrhythmias of Lown grade Ill. The third patient died of congestive heart failure at age 45 years, 13 years after complete repair, having had a previous Ports anastomosis at age 7 years (25 years before total surgical correction). Four years after the total repair he had reoperation for severe tricuspid regurgitation, residual atrial septal defect, and signs of heart failure, which did not recover. His electrocardiogram showed atrial fibrillation and right bundle-branch block.
Reoperations and Late Morbidity One patient required a permanent pacemaker for sick sinus syndrome 3 years after complete repair. Another patient had recurrence of a cerebral abscess, which had to be surgically drained. There were three late reoperations (8%). Besides the 1 patient who died later, 1 patient required reoperation because of residual ventricular septal defect and signs of heart failure, 2 years after complete repair. Eight years later she is well in functional class II. Another patient underwent replacement of the ascending aorta and aortic valve with a Dacron prosthetic tube and a mechanical valve (Bentall operation) because of severe aortic regurgitation and aneurysm of the ascending aorta (Fig 1) 10 years after complete repair. He had severe heart failure with a very low left ventrieular ejection fraction (Fig 2). After operation the patient recovered very well, and he is now in functional class II, with a left ventricular ejection fraction of 0.50 without medication (Fig 3). In 12 patients there was residual pulmonary stenosis: mild in 10 and moderate in 2. In 14 patients there was pulmonary valve insufficiency: mild in 13 and moderate in 1. Aortic insufficiency was found in 4 patients: mild in 2 cases, moderate in 1, and severe in 1 (who underwent aortic replacement). In 10 patients there was tricuspid regurgitation: mild in 7, moderate in 1, and severe in 2. One of the 2 patients with severe regurgitation underwent reoperation and another, treated medically, improved and did not need a further operation. Three patients (8%) had residual ventricular septal defect; which was minor in 2 cases but severe in 1, necessitating surgical intervention.
UPDATE PRESBITERO ET AL F O L L O W - U P IN ADULTS WITH TOF
1871
Fig 1. Aortic angiogram of a 34-year-old man, 10 years after total correction of tetralogy of Fallot, showing large aortic root with severe aortic regurgitation,
Arrhythmias and Conduction Defects Bifascicular block (left anterior hemiblock with right bundle-branch block) was detected in 7 patients (18%). One of them died suddenly. Chronic atrial fibrillation was found in 1 patient, and another had a permanent pacemaker for sick sinus syndrome. One patient had symptomatic paroxysmal supraventricular tachycardia. Significant ventricular arrhythmias (Lown class III or more) on ambulatory electrocardiographic monitoring were found in 7 of 20 patients (35%), and 1 of them died suddenly. Comparison of preoperative Holter monitoring with postoperative monitoring showed worsening of arrhythmias in only 4 patients. Late Functional Status The mean age of the 35 late survivors at latest follow-up was 43 years (range, 35 to 58 years). Sixteen patients were in New York Heart Association class I, 18 were in class II, and 1 was in class III. Seven women became pregnant after total surgical correction of tetralogy of Fallot, and delivered 10 babies. Nine of them were in good health, but 1 had a complex congenital heart disease and died a few days after delivery.
Comment This study demonstrates that patients with tetralogy of Fallot who reach adult life without a previous total correction can be operated on with low operative risk and a reasonble morbidity and mortality at long-term follow-
1872
UPDATE PRESB|TERO ET AL F O L L O W - U P IN ADULTS WITH TOF
Ann Thorac Surg 1996;61:1870-3
ejection fraction m a y continue to decrease with time. The i m p a i r m e n t in left ventricular function could be exp l a i n e d by the fibrosis following long-lasting cyanosis, with consequent chronic myocardial hypoxaemia, a n d b y altered contraction of the interventricular s e p t u m due to the presence of a prosthetic patch. Furthermore, the collateral circulation from systemic to p u l m o n a r y arteries, either surgically created or naturally present, causes chronic ventricular overload, particularly in adult patients. Some authors [4-6] have reported an increase in late morbidity a n d mortality in patients who h a d large shunts like Waterston or Ports shunts before their total correction. However, in our series only 1 patient died of congestive heart failure, a n d in another 1 with chronic heart failure i m p r o v e m e n t was achieved by aortic valve replacement, even though most of our patients were palliated with shunts. So, at least for m e d i u m - t e r m follow up, this p r o b l e m does not a p p e a r relevant.
A
B Fig 2. Left ventm'cular cineangiography of the same patient as in Figure 1, with end-diastolic (A) and end-systolic (B) frames. Ejection fraction = 0.30.
up. The postoperative p r o b l e m s in this category of patients are the same as those e n c o u n t e r e d in y o u n g patients, but with some peculiarities related to the longlasting i m p a i r e d h e m o d y n a m i c conditions.
Left Ventricular Function Although left ventricular failure is extremely rare after operation in tetralogy of Fallot, some studies [2] have d e m o n s t r a t e d that left ventricular volume a n d pressure p a r a m e t e r s are i m p a i r e d after total correction in childhood. Long-term trends [3] indicate that left ventricular
Pulmonary Hypertension No p u l m o n a r y vascular disease d e v e l o p e d in our patients, but 2 patients were excluded from this study at the time of operation because of the presence of p u l m o n a r y hypertension. Both these patients had had a Potts anastomosis. Residual Defects Severe residual p u l m o n a r y stenosis, which is the most important cause of reoperation in children, is not frequent in adult patients. Probably this is due to a milder form of right ventricular outflow tract obstruction in the survivors to adult life. P u l m o n a r y regurgitation also is more often mild, due to fewer a n d smaller t r a n s a n n u l a r patches (only 30% of our patients). Residual ventricular septal defects were p r e s e n t in our patients with the same frequency as in other series of patients o p e r a t e d on for tetralogy of Fallot at a y o u n g e r age. The most significant valvular abnormalities are aortic regurgitation, p r e s e n t in 10% of our patients, and tricuspid regurgitation, p r e s e n t in 25% of our population. Aortic regurgitation is mainly due to dilatation of the aortic root, which carries for a long time the cardiac output from both ventricles. It can also be due to infective endocarditis, as h a p p e n e d in I of our patients, who n e e d aortic r e p l a c e m e n t at the time of surgical correction. Aortic valve r e p l a c e m e n t is n e e d e d w h e n the left ventricle starts to fail, because a volume overload is not well tolerated by an already i m p a i r e d left ventricle. Tricuspid valve regurgitation can be the result of a dilated right ventricle because of other residual defects, such as residual p u l m o n a r y stenosis. In this case it is i m p o r t a n t to remove the cause of overload of the right ventricle to improve tricuspid regurgitation. Tricuspid regurgitation can also occur as a result of dilated and hypocontractile right ventricle; in this case, tricuspid annuloplasty often fails to restore a competent valve. Arrhythmias and Sudden Death It is well established that ventricular a r r h y t h m i a s are more frequent in older patients with tetralogy of Fallot.
Ann Thorac Surg 1996;61:1870-3
UPDATE PRESBITEROET AL FOLLOW-UP|N ADULTSWITHTOF
1873
Fig 3. Echocardiographic images of the same patient as in Figures 1 and 2 after Bentall procedure: the septum is fiat (arrow) and the left ventricular wall motion is very good, as can be seen in M-mode, which shows excellent posterior wall motion.
The incidence is about 12% to 17% in the patients operated on at a y o u n g age (1 to 7 years), a n d increases to 39% to 42% in patients whose age at operation was more than 13 years [7, 8], as in our series of older patients. The major concern has been the relation of these arrhythmias with s u d d e n death. However, it has not been proved that s p o n t a n e o u s or i n d u c e d ventricular arrhythmias, after surgical correction, indicate an elevated risk of s u d d e n death or that suppression of these arrhythmias protects patients against s u d d e n death. In our series, 1 of the 2 patients who died s u d d e n l y had significant arrhythmias (Lown > I I I ) , but he had also a very impaired heart performance, with tricuspid and aortic regurgitation. Five other patients who had the same degree of arrhythmias but better h e m o d y n a m i c conditions are still alive (3 treated with sotalol). A comparison of the high incidence of ventricular arrhythmias (40%) and the incidence of late s u d d e n death (1% to 5% [9-11]) implies that this association cannot be strong, and probably arrhythmia is only one of the causes; the other causes must be related to the h e m o d y n a m i c condition.
Conclusions
In conclusion, there is general agreement that the best treatment of patients with tetralogy of Fallot is complete repair in infancy, which may reduce the incidence of late electrophysiologic and hernodynamic problems a n d improve survival. However, advanced age does not appear to carry higher operative risk for total surgical correction, which should be accomplished as soon as possible. After surgical repair there is a marked i m p r o v e m e n t in functional class, a n d the long-term survival is better than in patients with only medical treatment. However, patients should be carefully followed up to diagnose residual
h e m o d y n a m i c abnormalities a n d significant rhythm disturbances. References
1. Presbitero P, Demarie D, Aruta E, et al. Results of total correction of tetralogy of Fallot performed in adults. Ann Thorac Surg 1988;46:297-301. 2. Sandor GGS, Patterson MWH, Tipple M, Ashmore PG, Popov R. Left ventricular systolic and diastolic function after total correction of tetralogy of Fallot. Am J Cardiol 1987;60: 1148-51. 3. Borow KM, Green LH, Castafieda AR, Keane JF. Left ventricular function after repair of tetralogy of Fallot and its relationship to age at surgery. Circulation 1980;61:1150-8. 4. Katz NM, Blackstone EH, Kirklin JW, Pacifico AD, Bargeron LM. Late survival and symptoms after repair of tetralogy of Fallot. Circulation 1982;65:403-10. 5. Fuster V, McGoon DC, Kennedy MA, Ritter DG, Kirklin JW. Long-term evaluation (12 to 22 years) of open heart surgery for tetralogy of Fallot. Am J Cardiol 1980;46:635-42. 6. Murphy JG, Gersh BJ, Mair DD, et al. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med 1993;329:593-9. 7. Sullivan I, Presbitero P, Gooch VM, et al. Is ventricular arrhythmia in repaired tetralogy of Fallot an effect of operation or a consequence of the course of the disease? Br Heart J 1987;58:40-4. 8. Deanfield JE, McKenna WJ, Presbitero P, et al. Ventricular arrhythmia in unrepaired and repaired tetralogy of Fallot. Relation to age, timing of repair, and haemodynamic status. Br Heart J 1984;52:77-81. 9. Cullen S, Celermajer DS, Franklin RCG, Hallidie KA, Deanfield JE. Prognostic significance of ventricular arrhythmia after repair of tetralogy of Fallot: a 12-year prospective study. J Am Coil Cardiol 1994;23:1151-5. 10. Horstkotte D, Paselk C, Bircks W, Loogen F. Clinical longterm results after corrective surgery of tetralogy of Fallot. Z Kardiol 1993;82:552-62. 11. Morris CD, Menashe VD. 25 year mortality after surgical repair of congenital heart defect in childhood. A populationbased cohort study. JAMA 1991;266:3447-52.
As Originally Published in 1988:
Results of Total Correction of Tetralogy of Fallot Performed in Adults P. Presbitero, M.D., D. Demarie, M.D., E. Aruta, M.D., M. Villani, M.D., M. Disumma, M.D., G. M. Ottino, M.D., F. Orzan, M.D., A. Fubini, M.D., M. T. Spinnler, M.D., M. R. Conte, M.D., and M. Morea, M.D.
ABSTRACT Today, total correction of tetralogy of Fallot is rarely performed in adults. In a 10-year period, 40 patients aged 20 to 67 years underwent intracardiac repair in our institution. Twenty-eight of them had had a palliative procedure 11 to 30 years earlier. Preoperatively, 23 patients were in N e w York Heart Association (NYHA) Functional Class II, 14 were in Class lIl, and 3 were in Class IV. Operative mortality was 2.5% (1/40). Follow-up ranged from I year to 11 years (average, 3 years). One patient died of a noncardiac cause 4 years after operation. Residual cardiac defects were observed in 4 patients. Postoperatively,
30 patients were in NYHA Functional Class I, 8 were in Class II, and 1 was in Class III. Major ventricular arrhythmias were recorded in 7 (35%) of 20 patients. Radionuclide angiography demonstrated impaired right ventricular function in 8 patients. Left ventricular impairment was present in 2. Total correction of tetralogy of Fallot can be performed safely in adults with low mortality and good functional improvement. The incidence of residual cardiac defects is low. The long-term importance of impaired ventricular function and arrhythmias remains to be ascertained.
Updated in 1996 by Patrizia Presbitero, MD, Silvia Brach Prever, MD, Igino Contrafatto, MD, and Mario Morea, MD Divisione di Cardiologia, Ospedale G. Bosco, Torino, Italy
lthough most patients with tetralogy of Fallot have radical repair ("total correction") during infancy or childhood, some patients reach adult life without such an operation. This may occur when a previous palliative shunt has been performed, or when there is natural collateral circulation to the lung, or when a mild form of the disease is present. The long-term follow-up for this operation performed in adults is less well-known and may be suspected to differ significantly from those in younger patients. Accordingly, we present the long-term follow-up of a series of 40 patients with tetralogy of Fallot who had radical repair at age 20 years or older. Their clinical outcome and factors affecting their survival are analyzed. In 1988 [1] we reported the immediate and short-term results of the same group of patients.
A
Material and M e t h o d s The original study group consisted of 40 consecutive patients with tetralogy of Fallot who had intracardiac repair, from 1974 to 1985, in the Cardiac Surgery Department, University of Torino. The patients' age at operation ranged from 20 to 67 years (mean, 30 years); 22 were men and 18 were women. A previous palliative operation had Address reprint requests to Dr Presbitero, PiazzaDonatoridel Sangue3, 10154Torino, Italy. © 1996 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
been performed in 28 patients (70%). Preoperative findings, operative procedures, and short-term follow-up have been described in the previous report in 1988 [1]. The hospital mortality was 2.5% (1140), and the 39 survivors were followed by either outpatient clinic visit (22 patients) or telephone conversation with the patient or the attending physician (10 patients). We were unable to determine the current status of 7 patients, including the oldest patient (67 years of age), but the hospital records indicated that they were alive 10 years after the operation. Follow-up in surviving patients ranged from 10 to 20 years (mean, 13 years). Patients who returned for a clinical visit were evaluated with physical examination, resting electrocardiogram, chest roentgenography, and two-dimensional Doppler echocardiography. Fourteen patients had postoperative cardiac catheterization and angiography. Holter monitoring was performed in 20 patients, and ventricular arrhythmias were classified according to the criteria of Lown. Results
Late Deaths Four of the 39 operative survivors had died by the last assessment in June 1995, representing an overall mortalAnn Thorac Surg 1996;61:1870-3 ° 0003-4975/96/$15.00 PII S0003-4975(96)00180-4
A n n Thorac Surg 1996;61:1870-3
ity rate for all causes of 0.01 death/patient-year. One of these patients died of a non-cardiac-related cause 4 years after complete repair, namely, massive hemorrhage in the postpartum period. Three more cardiovascularrelated deaths occurred 9, 10, and 13 years after total surgical repair. Two of the three were sudden: one occurred at home in a 48-year-old patient with a previous brain abscess, 9 years after operation. The second patient died suddenly and unexpectedly at age 36 years, 10 years after complete repair; he had had a previous Blalock shunt at age 10 years, and had residual moderate regurgitation of the aortic and tricuspid valves. His electrocardiogram showed left-axis deviation and right bundlebranch block, and postoperative Holter monitoring revealed ventricular arrhythmias of Lown grade Ill. The third patient died of congestive heart failure at age 45 years, 13 years after complete repair, having had a previous Ports anastomosis at age 7 years (25 years before total surgical correction). Four years after the total repair he had reoperation for severe tricuspid regurgitation, residual atrial septal defect, and signs of heart failure, which did not recover. His electrocardiogram showed atrial fibrillation and right bundle-branch block.
Reoperations and Late Morbidity One patient required a permanent pacemaker for sick sinus syndrome 3 years after complete repair. Another patient had recurrence of a cerebral abscess, which had to be surgically drained. There were three late reoperations (8%). Besides the 1 patient who died later, 1 patient required reoperation because of residual ventricular septal defect and signs of heart failure, 2 years after complete repair. Eight years later she is well in functional class II. Another patient underwent replacement of the ascending aorta and aortic valve with a Dacron prosthetic tube and a mechanical valve (Bentall operation) because of severe aortic regurgitation and aneurysm of the ascending aorta (Fig 1) 10 years after complete repair. He had severe heart failure with a very low left ventrieular ejection fraction (Fig 2). After operation the patient recovered very well, and he is now in functional class II, with a left ventricular ejection fraction of 0.50 without medication (Fig 3). In 12 patients there was residual pulmonary stenosis: mild in 10 and moderate in 2. In 14 patients there was pulmonary valve insufficiency: mild in 13 and moderate in 1. Aortic insufficiency was found in 4 patients: mild in 2 cases, moderate in 1, and severe in 1 (who underwent aortic replacement). In 10 patients there was tricuspid regurgitation: mild in 7, moderate in 1, and severe in 2. One of the 2 patients with severe regurgitation underwent reoperation and another, treated medically, improved and did not need a further operation. Three patients (8%) had residual ventricular septal defect; which was minor in 2 cases but severe in 1, necessitating surgical intervention.
UPDATE PRESBITERO ET AL F O L L O W - U P IN ADULTS WITH TOF
1871
Fig 1. Aortic angiogram of a 34-year-old man, 10 years after total correction of tetralogy of Fallot, showing large aortic root with severe aortic regurgitation,
Arrhythmias and Conduction Defects Bifascicular block (left anterior hemiblock with right bundle-branch block) was detected in 7 patients (18%). One of them died suddenly. Chronic atrial fibrillation was found in 1 patient, and another had a permanent pacemaker for sick sinus syndrome. One patient had symptomatic paroxysmal supraventricular tachycardia. Significant ventricular arrhythmias (Lown class III or more) on ambulatory electrocardiographic monitoring were found in 7 of 20 patients (35%), and 1 of them died suddenly. Comparison of preoperative Holter monitoring with postoperative monitoring showed worsening of arrhythmias in only 4 patients. Late Functional Status The mean age of the 35 late survivors at latest follow-up was 43 years (range, 35 to 58 years). Sixteen patients were in New York Heart Association class I, 18 were in class II, and 1 was in class III. Seven women became pregnant after total surgical correction of tetralogy of Fallot, and delivered 10 babies. Nine of them were in good health, but 1 had a complex congenital heart disease and died a few days after delivery.
Comment This study demonstrates that patients with tetralogy of Fallot who reach adult life without a previous total correction can be operated on with low operative risk and a reasonble morbidity and mortality at long-term follow-
1872
UPDATE PRESB|TERO ET AL F O L L O W - U P IN ADULTS WITH TOF
Ann Thorac Surg 1996;61:1870-3
ejection fraction m a y continue to decrease with time. The i m p a i r m e n t in left ventricular function could be exp l a i n e d by the fibrosis following long-lasting cyanosis, with consequent chronic myocardial hypoxaemia, a n d b y altered contraction of the interventricular s e p t u m due to the presence of a prosthetic patch. Furthermore, the collateral circulation from systemic to p u l m o n a r y arteries, either surgically created or naturally present, causes chronic ventricular overload, particularly in adult patients. Some authors [4-6] have reported an increase in late morbidity a n d mortality in patients who h a d large shunts like Waterston or Ports shunts before their total correction. However, in our series only 1 patient died of congestive heart failure, a n d in another 1 with chronic heart failure i m p r o v e m e n t was achieved by aortic valve replacement, even though most of our patients were palliated with shunts. So, at least for m e d i u m - t e r m follow up, this p r o b l e m does not a p p e a r relevant.
A
B Fig 2. Left ventm'cular cineangiography of the same patient as in Figure 1, with end-diastolic (A) and end-systolic (B) frames. Ejection fraction = 0.30.
up. The postoperative p r o b l e m s in this category of patients are the same as those e n c o u n t e r e d in y o u n g patients, but with some peculiarities related to the longlasting i m p a i r e d h e m o d y n a m i c conditions.
Left Ventricular Function Although left ventricular failure is extremely rare after operation in tetralogy of Fallot, some studies [2] have d e m o n s t r a t e d that left ventricular volume a n d pressure p a r a m e t e r s are i m p a i r e d after total correction in childhood. Long-term trends [3] indicate that left ventricular
Pulmonary Hypertension No p u l m o n a r y vascular disease d e v e l o p e d in our patients, but 2 patients were excluded from this study at the time of operation because of the presence of p u l m o n a r y hypertension. Both these patients had had a Potts anastomosis. Residual Defects Severe residual p u l m o n a r y stenosis, which is the most important cause of reoperation in children, is not frequent in adult patients. Probably this is due to a milder form of right ventricular outflow tract obstruction in the survivors to adult life. P u l m o n a r y regurgitation also is more often mild, due to fewer a n d smaller t r a n s a n n u l a r patches (only 30% of our patients). Residual ventricular septal defects were p r e s e n t in our patients with the same frequency as in other series of patients o p e r a t e d on for tetralogy of Fallot at a y o u n g e r age. The most significant valvular abnormalities are aortic regurgitation, p r e s e n t in 10% of our patients, and tricuspid regurgitation, p r e s e n t in 25% of our population. Aortic regurgitation is mainly due to dilatation of the aortic root, which carries for a long time the cardiac output from both ventricles. It can also be due to infective endocarditis, as h a p p e n e d in I of our patients, who n e e d aortic r e p l a c e m e n t at the time of surgical correction. Aortic valve r e p l a c e m e n t is n e e d e d w h e n the left ventricle starts to fail, because a volume overload is not well tolerated by an already i m p a i r e d left ventricle. Tricuspid valve regurgitation can be the result of a dilated right ventricle because of other residual defects, such as residual p u l m o n a r y stenosis. In this case it is i m p o r t a n t to remove the cause of overload of the right ventricle to improve tricuspid regurgitation. Tricuspid regurgitation can also occur as a result of dilated and hypocontractile right ventricle; in this case, tricuspid annuloplasty often fails to restore a competent valve. Arrhythmias and Sudden Death It is well established that ventricular a r r h y t h m i a s are more frequent in older patients with tetralogy of Fallot.
Ann Thorac Surg 1996;61:1870-3
UPDATE PRESBITEROET AL FOLLOW-UP|N ADULTSWITHTOF
1873
Fig 3. Echocardiographic images of the same patient as in Figures 1 and 2 after Bentall procedure: the septum is fiat (arrow) and the left ventricular wall motion is very good, as can be seen in M-mode, which shows excellent posterior wall motion.
The incidence is about 12% to 17% in the patients operated on at a y o u n g age (1 to 7 years), a n d increases to 39% to 42% in patients whose age at operation was more than 13 years [7, 8], as in our series of older patients. The major concern has been the relation of these arrhythmias with s u d d e n death. However, it has not been proved that s p o n t a n e o u s or i n d u c e d ventricular arrhythmias, after surgical correction, indicate an elevated risk of s u d d e n death or that suppression of these arrhythmias protects patients against s u d d e n death. In our series, 1 of the 2 patients who died s u d d e n l y had significant arrhythmias (Lown > I I I ) , but he had also a very impaired heart performance, with tricuspid and aortic regurgitation. Five other patients who had the same degree of arrhythmias but better h e m o d y n a m i c conditions are still alive (3 treated with sotalol). A comparison of the high incidence of ventricular arrhythmias (40%) and the incidence of late s u d d e n death (1% to 5% [9-11]) implies that this association cannot be strong, and probably arrhythmia is only one of the causes; the other causes must be related to the h e m o d y n a m i c condition.
Conclusions
In conclusion, there is general agreement that the best treatment of patients with tetralogy of Fallot is complete repair in infancy, which may reduce the incidence of late electrophysiologic and hernodynamic problems a n d improve survival. However, advanced age does not appear to carry higher operative risk for total surgical correction, which should be accomplished as soon as possible. After surgical repair there is a marked i m p r o v e m e n t in functional class, a n d the long-term survival is better than in patients with only medical treatment. However, patients should be carefully followed up to diagnose residual
h e m o d y n a m i c abnormalities a n d significant rhythm disturbances. References
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