- Email: [email protected]
Total correction of tetralogy of fallot in adults-surgical experience
International Journal of Cardiology 63 (1998) 121–128
Total correction of tetralogy of fallot in adults-surgical experience M Rammohan, Balram Airan*, Anil Bhan, Rajesh Sharma, Sushant Srivastava, Anita´ Saxena, Kumar A. Sampath, P. Venugopal Department of Cardiothoracic and Vascular Surgery Cardiothoracic Sciences Centre, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110 029, India Received 16 July 1997; accepted 22 October 1997
Abstract A total of 100 patients of tetralogy of Fallot aged 13 years and over were operated upon at the All India Institute of Medical Sciences, New Delhi, India between January 1991 and December 1996. There were 69 males (69%) and 31 females (31%). Age ranged from 13 years to 43 years (mean 19.66 years). Twenty % of patients had preoperative complications like haemoptysis, cerebrovascular accidents, brain abscess and infective endocarditis. Twenty-two patients had previous palliative shunts. Fifteen patients had coil embolisation of major collaterals prior to surgery. In hospital mortality rate was 4%. Follow-up ranged from 1 month to 5 years (mean 3.4 years). There was one late death due to infective endocarditis. Postoperatively 93.6% patients were in NYHA class I. Significant residual defects warranting re-operation were present in three patients. Total correction of tetralogy of Fallot in older patients can be performed with acceptable results. 1998 Elsevier Science Ireland Ltd. Keywords: Tetralogy of Fallot; Adult patient; Total correction
1. Introduction Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease in which survival into adulthood is possible. Only 66% of patients survive up to 1 year of age without surgery; 49% to 3 years and 24% to 10 years of age [1]. The current trend is to perform total correction as early as possible and preferably during infancy. The results of total correction performed during infancy are superior to correction done later in life [2]. TOF in adults represents a special subset of patients with peculiar problems namely the effects of prolonged cyanosis, polycythemia, coagulation defects, development of collaterals, secondary myocardial dysfunction and *Corresponding author.
morphological and physiological consequences of previous palliative shunts. In our country, patients continue to present late in their life with one or more of the problems addressed to. We present our experience with 100 such patients who had the surgical correction of tetralogy of Fallot in adulthood (13 years and over) from All India Institute of Medical Sciences, New Delhi, India.
2. Materials and methods A total of 100 patients, 13 years of age and over with a diagnosis of tetralogy of Fallot were operated upon at our institute between January 1991 and December 1996. They constituted 8.65% of all total corrections for tetralogy of Fallot and 2.26% of all
0167-5273 / 98 / $19.00 1998 Elsevier Science Ireland Ltd. All rights reserved. PII S0167-5273( 97 )00279-9
122
M. Rammohan et al. / International Journal of Cardiology 63 (1998) 121 – 128
Table 1 Preoperative complications in adult patients with tetralogy of Fallot Haemoptysis Cerebrivascular accidents (brain abcess-4) Infective endocarditis Epistaxis
– –
10 7
– –
2 1
congenital cardiac surgeries performed during the same period. There were 69 males (69%). Ages of the patients ranged from 13 years to 43 years. The mean age at presentation was 19.66 years. At presentation 58 patients (58%) were in NYHA class II and 42 (42%) were in NYHA class III. Ten patients had history of haemoptysis and one had history of epistaxis. Two patients required hospital admission prior to surgery Table 2 Palliative surgeries performed in adult patients with tetralogy of Fallot Classical left BT shunt Right modified BT shunt Left modified BT shunt Central aortopulmonary shunt Pott’s shunt
– – – – –
9 8 4 2 1
because of massive haemoptysis. Seven patients had history of cerebrovascular accidents with residual hemiparesis in five. Of these seven patients, four had documented brain abscesses; two patients required repeated aspirations of brain abscess because of incomplete evacuation at the first sitting. In another three patients the cause was not documented. Two patients had preoperative endocarditis, one of whom had shunt endocarditis. (Table 1). Palliative Surgery: Twenty-two patients underwent 24 palliative systemic to pulmonary artery shunt operations prior to total correction (Table 2) (Fig. 1). Two patients underwent shunt surgeries twice. One of them had a right modified Blalock–Taussing shunt at 13 years of age. Fifteen days later the shunt got blocked and a left modified Blalock–Taussig shunt was performed. The other patient had left classical Blalock–Taussing shunt at 6 years of age. A central aortopulmonary shunt was performed at 28 years of age when the classical shunt was blocked. She underwent successful total correction after another 8 years. The time interval between palliative shunts and total correction varied from 20 months to 23 years
Fig. 1. Aortic arch injection in left anterior oblique view shows Pott’s shunt between descending thoracic aorta and left pulmonary artery (arrow) filling both pulmonary arteries.
M. Rammohan et al. / International Journal of Cardiology 63 (1998) 121 – 128 Table 3 Pulmonary anatomy in adult patients with tetralogy of Fallot Small main pulmonary artery Bifurcational stenosis Ostial stenosis of left pulmonary artery Small right pulmonary artery Small left pulmonary artery
– –
9 7
– – –
4 1 1
(mean 10.6 years). Four patients on angiography before total correction were found to have blocked shunts, three of whom had classical left Blalock]Taussig shunt and one patient had right modified Blalock–Taussig shunt. In only one patient, there was distortion of left pulmonary artery due to the previous left classical Blalock]Taussig shunt. None of the patients developed pulmonary hypertension or pulmonary vascular obstructive disease secondary to shunt including the patient who had Pott’s shunt at the age of 6 years and had total correction at the age of 43 years. Chest X-ray showed cardiac enlargement in 19 patients (19%). Reticular pattern due to well developed bronchopulmonary collaterals was evident in 26 patients (26%). Rib notching, due to long standing
123
Blalock–Taussig (BT) shunt was seen in three patients (3%). All patients underwent echocardiography and complete cardiac catheterisation and angiography. Echocardiography was particularly useful in delineating the aortomitral continuity and additional ventricular septal defects if any. On cardiac catheterisation right ventricular systolic pressures were typically equal to the systemic pressures in all patients. In 69 patients, pulmonary artery could not be entered. None of the previously shunted patients had pulmonary hypertension. Systemic arterial saturation varied from 65% to 89.5% (mean 78.5%). Angiography showed distortion of left pulmonary artery due to the previous left classical Blalock– Taussig shunt in one patient (Table 3). Shunt angiogram in previously palliated patients showed that four shunts were blocked. Angiography was useful in delineating coronary artery anomalies (Fig. 2). In five patients unexpected coronary arterial anomalies were detected in operating room. Aortopulmonary collaterals: Fifteen patients (15%) underwent coil embolization prior to surgery for significant aortopulmonary collaterals. Two patients needed surgical ligation. In one of the two, collateral
Fig. 2. Aortic root angiogram in right anterior oblique view shows common origin of right and left coronary arteries from right coronary sinus (arrow).
M. Rammohan et al. / International Journal of Cardiology 63 (1998) 121 – 128
124
Table 5 Associated procedures performed in adult patients with tetralogy of Fallot
was ligated at the time of making a systemic-pulmonary shunt and in the other patient it had to be ligated postoperatively because of significant left to right shunt. Associated anomalies (Table 4): One patient had aneurysmal right atrium along with cleft in the septal tricuspid leaflet with tricuspid valve prolapse causing severe tricuspid regurgitation. Another patient had a criss-cross pattern of ventricles with dextroposed left atrial appendage. Two patients were found to have healed vegetations in the operating room; in one on the tricuspid valve and in the other on the pulmonary valve. Operative details: Total correction was performed through median sternotomy. Cardiopulmonary bypass was established with standard aortic and bicaval cannulation. All functioning shunts were ligated at this stage. In one patient Pott’s shunt was closed under total circulatory arrest. Myocardial preservation was achieved by moderate hypothermia, multidose antegrade modified cold prime cardioplegia and topical ice slush. Left atrium was routinely vented. Transatrial approach was used in 50 patients (50%). In the other 50 patients transventricular correction was done. Interrupted mattress sutures were used to close the ventricular septal defect in 52 patients; in 30 patients continuous suture was used. Interrupted sutures along the posteroinferior margin of the ventricular septal defect and continuous suture along the rest of the ventricular septal defect margin were used in the rest. Transannular pericardial patch was placed in 60 patients (60%). The patch was extended up to the pulmonary trunk bifurcation or onto the proximal pulmonary artery branches in 13
Closure of previous palliative shunt Patch closure of ASDs a Tricuspid valve repair1right atrial aneurysm repair a
a b
Atrial septal defect. Ventricular septal defect.
– – – –
2 1 1 1
Right aortic arch Left superior vena cava Criss cross ventricles Dextroposed left atrial appendage Double aortic arch Retroaortic innominate vein Left subclavian artery stenosis Left subclavian artery aneurysm
18 2 1
Atrial septal defect
patients. In one patient, right pulmonary artery was augmented with pericardial patch. In four patients, the atrial septal defect was partially left open because of the borderline size of pulmonary arteries. Abnormal coronary arteries: Twelve patients had coronary artery anomalies. In six patients, left anterior descending artery was crossing the right ventricular outflow tract; of these, two patients had single coronary artery arising from right coronary sinus and in two other patients there was additional large left anterior descending artery arising from right coronary artery. In the remaining six patients a large conal branch was crossing the right ventricular outflow tract. Associated procedures: (Table 5). One patient underwent closure of the tricuspid cleft, tricuspid annuloplasty and repair of aneurysmal right atrium. Since 1995 we have been using Aprotonin prophylactically during total correction in adult tetralogy of Fallot patients to reduce the amount of blood loss. Since the same time, these patients were bled (one or two units depending upon the haematocrit) before surgery. The blood was infused postoperatively to control bleeding.
3. Results
3.1. Early results
Table 4 Associated anomalies in adult patients with tetralogy of Fallot Large ostium secundum ASDs a Multiple VSDs b Aneurysmal rightartrium Cleft tricuspid leaflet
– – –
– – –
28 6 1
– –
1 1
–
1
–
1
–
1
Hospital stay of the patients ranged from 6 days to 51 days (mean 13.7 days). Mortality: The early mortality was 4% (Table 6). Two patients died of low cardiac output on the 8th and 9th postoperative days. Echocardiography in both these patients showed poor right ventricular function Table 6 Causes of early mortality following tetralogy of Fallot repair in adults Low cardiac output Intrapulmonary haemorrhage Hepatorenal failure
– – –
2 1 1
M. Rammohan et al. / International Journal of Cardiology 63 (1998) 121 – 128
and there was no demonstrable residual ventricular septal defect or significant right ventricular outflow tract obstruction. One patient developed severe jaundice and acute renal failure on the 2nd postoperative day and died on the 10th postoperative day. One patient had persistent bleeding from the day of surgery and ultimately died of the 22nd postoperative day of massive intrapulmonary haemorrhage. Early complications: (Table 7). Four patients had complete atrioventricular conduction block in the early postoperative period. Three of these reverted spontaneously to normal sinus rhythm before discharge, while one patient needed permanent pacemaker implantation. One patient had generalised tonic-clonic convulsions on the 4th postoperative day. He recovered without any sequelae. One patient, a case of previous pulmonary tuberculosis had recrudescence and required antituberculous treatment.
3.2. Late results Follow-up ranged from 1 month to 5 years (mean 3.4 years). At the latest follow-up 89 patients (93.6%) were in NYHA functional class I and 6 patients (6.31%) in class II. One patient died 2 months after repair due to infective endocarditis. He had right ventricular outflow tract patch dehiscence and underwent repeat surgery for patch reconstruction. He finally succumbed to persistent toxaemia and multiorgan failure. Forty patients, randomly selected, had postoperative echocardiographic examination. Echocardiography showed significant residual ventricular septal defect in three patients, one of whom had significant right ventricular outflow tract obstruction also. One patient had right ventricular diastolic dysfunction and another had moderate tricuspid regurgitation. Four other patients had small residual ventricular septal defects of no haemodynamic significance.
Excessive bleeding Transient complete heart block Pleural effusion Permanent complete heart block Haematemesis Sternal wound infection Recrudescence of pulmonary tuberculosis
– – – – – – –
Cardiac catherisation was performed in three patients which included two patients who had residual ventricular septal defect on echocardiography and one patient who was persistently symptomatic and in whom echocardiographic examination was not conclusive. In this patient, the cardiac catheterisation was also normal. Cardiac catheterisation in the other two patients with significant residual ventricular septal defect showed significant left to right shunt.
3.3. Late complications Table 8 Five patients had congestive cardiac failure at 1, 2.5, 2.5, 3 and 6 months after surgery (mean 3 months). Echocardiography did not show residual surgical lesion in any of these patients. Tow patients had right ventricular dysfunction and one patient had biventricular dysfunction. They were managed conservatively and all of them recovered well. One patient had tuberculous pericardial effusion 2 months postoperatively which was managed with antituberculous treatment and catheter drainage. None of the patients had significant arrhythmias on follow-up. Two patients had persistent atrial bigeminy which responded to discontinuation of digoxin. Patients who had tricuspid regurgitation and right ventricular diastolic dysfunction are asymptomatic on medication presently. None of the patients had left ventricular dysfunction or pulmonary incompetence. Re-operation: Four patients underwent re-operation at 2, 2, 5 and 22 months after total correction. Three patients had closure of residual ventricular septal defects one of whom had residual right ventricular outflow tract obstruction as well. One patient underTable 8 Late complications following tetralogy of Fallot repair in adults Residual VSDs a Tricuspid regurgitation Right ventricular diastolic dysfunction
Table 7 Early complications following tetralogy of Fallot repair in adults 15 3 3 1 1 1 1
125
a
– – –
3 1 1
Congestive cardiac failure Pericardial effusion Arrhythmias Pleural effusion Convulsions RVOT b patch dehiscence
– – – – – –
Ventricular septal defect (one of these patients had associated significant right ventricular outflow tract obstruction). b Right ventricular outflow tract (infective endocarditis was the cause of right ventricular outflow tract patch dehiscence and residual vertricular septal defect in one patient each).
5 2 2 1 1 1
126
M. Rammohan et al. / International Journal of Cardiology 63 (1998) 121 – 128
went right ventricular outflow tract patch reconstruction twice for patch dehiscence secondary to infective endocarditis. He died one month after surgery due to multiorgan failure and toxaemia.
4. Discussion Survival to adulthood despite chronic cyanosis in some patients with tetralogy of Fallot is partly due to the less severe form of the disease and development of collaterals leading to increased pulmonary blood flow [3,4]. Since the first successful surgical correction of tetralogy of Fallot by Lillehei et al., [5] in 1955, there has been tremendous improvement in surgical techniques and methods of myocardial protection. Consequently the morbidity and mortality following repair of tetralogy of Fallot has drastically reduced. The present day consensus is to perform total correction at the earliest preferably during infancy if the anatomy is suitable. Not many series of results of total correction of tetralogy of Fallot in adults are available. In our study, twenty patients had complications of tetralogy of Fallot prior to surgery and most of these were due to the presence of extensive bronchial collaterals (ten patients had haemoptysis). This is comparable with the experience of Presbitero et al., [6] and Stanley John et al., [7]. The incidence of haemorrhagic and neurologic complications in adults is definitely more compared to childhood. In our study, more than half of the patients with bleeding tendencies, namely haemoptysis and epistaxis required repeated hospital admissions prior to total correction. Sizeable collaterals requiring intervention were present in seventeen patients (17%) in our experience. Fifteen of these patients had successful preoperative coil embolisation. Surgical intervention was needed in two cases. In contrast to reports of Abraham et al., [4] and Stanley John et al., [7] who reported 15% incidence of congestive cardiac failure, none of our patients had such complication in the preoperative period. The high incidence of cardiac failure was attributed to long standing pressure overload and the consequent pathological changes in the right ventricle [7]. Twenty-two patients (22%) in our study underwent previous palliative shunt operations prior to total
correction. In the experience of Presbitero et al., [6] and Mattila et al., [8] more than half of the patients received preliminary shunts. Pulmonary artery distortion consequent to such shunts was seen in only one of our patients in whom the left pulmonary artery was distorted due to left classical Blalock–Taussig shunt. None of the patients including the one who had Pott’s shunt developed pulmonary vascular obstructive disease. In contrast to the report of Nandi and coworkers [9] we did not find increased mortality after total correction in patients who underwent palliative shunt operations previously. Excessive bleeding was frequently encountered during the dissection of old shunts which however did not alter the mortality rate in our experience. Most of the early morbidity in our series was due to the excessive bleeding following surgery. Fifteen patients (15%) had such complication. As mentioned earlier, we used Aprotonin and autologous blood in patients where excessive bleeding was anticipated. This resulted in reduced blood loss during and after surgery. Although we do not have controlled study, based on initial experience, we feel that this two options might be effective in reducing blood loss in these patients. Complete heart block is uncommon following total correction. Only 1.3% of patients of all ages in Kirklin’s series [10] had this complication. In our study only one patient required permanent pacemaker implantation. Casteneda [11] and Kirklin et al., [10] in their total experience had a mortality between 2% and 5%. Kirklin et al., [10] found that old age was an incremental risk factor for early mortality following total correction. Our early mortality of 4% is comparable to that of others [6,7,11]. Based on our results, we feel that old age may not be an incremental risk factor with the current mode of intraoperative and postoperative management. Although our follow-up period is not very long, there was only one late death. David Hu et al., [12] reported the results of total correction in 32 patients who were 40 years of age or older at the time of surgery. They followed the patients up to a mean of 110 months. Five patients died of cardiovascular related causes in their series. There were four late deaths out of 39 adults patients who underwent total correction in the study of Presbitero et al., [13]. Both groups proved that total correction in adult patients
M. Rammohan et al. / International Journal of Cardiology 63 (1998) 121 – 128
could be performed with less incidence of early and late mortality. Presbitero et al., [13] found significant arrhythmias in at least nine out of 39 adult patients on follow-up. Bifascicular block was found in 18% of patients one of whom died suddenly. Our results are more encouraging. Only two patients had persistent atrial bigeminy on follow-up which responded to discontinuation of digoxin. The exact incidence of infective endocarditis in adults following total correction is not known. Two patients in our study had infective endocarditis within 6 months of surgery. One of them died following right ventricular outflow tract patch dehiscence. Residual right ventricular outflow tract obstruction following total correction is less common in adults than in children most probably because of milder degrees of right ventricular outflow tract narrowing. Significant right ventricular outflow tract obstruction occurred in one out of 96 late survivors in our series. Although residual ventricular septal defects occur postoperatively, less than 1% of them are significant enough to need re-operation. [14] Mattila et al., [8] reported two cases following total correction in 39 adults who needed re-operation. We had three cases (3.1%) of significant residual ventricular septal defects out of 96 patients, (one of which was due to infective endocarditis) which needed re-operation and had successful outcome. The use of transannular pericardial patch did not result in increased operative mortality or significant pulmonary regurgitation in our patients. This is consistent with the results of Casteneda et al., [11]. We are of the opinion that adequate use of transannular patch should prevent the important complication of residual right ventricular outflow tract obstruction in adults who had already suffering from long standing right ventricular hypertension. We did not find any difference in the outcome after total correction done by transatrial or transventricular route. Karl and Mee et al., [15] advocate transatrial approach because of the late right ventricular dilatation and dysfunction and increased risk of ventricular arrhythmias that might follow ventriculotomy. Although our follow-up period is short, we did not encounter such complications following transventricular approach. 93.6% of patients following surgery in our study
127
are in NYHA class I. Our results are similar to those of Katz et al., [16] and Rosenthal et al., [17] who reported more than 90% improvement in functional status following total correction at all age groups. However Strieder [18] found that functional results were not as good in older patients when assessed by exercise tolerance test. We are yet to follow our patients when exercise tolerance test.
5. Conclusion Total correction of tetralogy of Fallot can be performed in older patients with good results. Functional improvement following surgery is marked and most of the patients can return to normal life. The morbidity and mortality in our experience do not alter the outcome. In our experience the incidence of residual defects is quite low.
References [1] Bertranou EG, Blackstone EH, Hazelrig JB, Turner Jr. ME, Kirklin JW. Life expectancy without surgery in tetralogy of Fallot. Am J Cardiol 1978;42:458–66. [2] Casteneda AR, Freed MD, William RG, Norwood WI. Repair of tetralogy of Fallot in infancy. Early and late results. J Thorac Cardiovasc Surg 1977;74:372–81. [3] Higgins CB, Mulder DG. Tetralogy of Fallot in the adult. Am J Cardiol 1972;29:837–46. [4] Abraham KA, Cherian G, Dayasagar RV, Sukumar IP, Krishnaswami S, Stanley J. Tetralogy of Fallot in adults. A report on 147 patients. Am J Med 1979;66:811–6. [5] Lillehei CW, Cohen M, Warden HE, et al. Direct vision intracardiac surgical correction of tetralogy of Fallot, pentology of Fallot and pulmonary atresia defects, report of first ten cases. Ann Surg 1955; 418–45. [6] 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. [7] Stanley J, Kejriwal NK, Ravikumar E, Bashi VV, Mohanty BB, Sukumar IP. The clinical profile and surgical treatment of tetralogy of Fallot in the adult: results of repair in 200 patients. Ann Thorac Surg 1986;41:502–6. [8] Mattila S, Luosto R, Ketonen P, Nieminen M, Merikallio E, Kyllnen EJ. Total correction of tetralogy of Fallot in adults: Scan. J Thorac Cardiovasc 1984;18:23–7. [9] Nandi PL, Chaung KL, Mok CK. Total correction of tetralogy of Fallot: a review of 280 cases. JR Coll Surg Edinb 1981;26:340–5. [10] Kirklin JW, Blackstone EH, Jonas RA et al. Morphologic and surgical determinants of outcome events after repair of tetralogy of Fallot and pulmonary stenosis. J Thorac Cardiovasc Surg 1992;103:706–23.
128
M. Rammohan et al. / International Journal of Cardiology 63 (1998) 121 – 128
[11] Casteneda AR. Classical repair of tetralogy of Fallot: Timing, technique and results. Semin Thorac Cardiovasc Surg 1990;2:70–9. [12] David HCK, Seward JB, Puga FJ, Fuster V, Tajik AJ. Total correction of tetralogy of Fallot at age 40 years and older: long term followup. J Am Coll Cardiol 1985;1:40–4. [13] Presbitero P, Demarie D, Aruta E et al. Results of total correction of tetralogy of Fallot performed in adults. Updated in 1996. Ann Thorac Surg 1996;61:1870–3. [14] Dobell ARC, Charrette EP, Chughtai MS. Correction of tetralogy of Fallot in the young child. J Thorac Cardiovasc Surg 1968;55:70–9. [15] Karl TR, Sano S, Pornviliwan S, Mee RBB. Tetralogy of Fallot: favourable outcome of non neonatal transatrial, transpulmonary repair. Ann Thorac Surg 1992;54:903–7.
[16] Katz NM, Blackstone EH, Kirklin JW, Pacifico AD, Bergeron Jr. LM. Late survival and symptoms after repair of tetralogy of Fallot. Circulation 1982;65:403–8. [17] Rosenthal A, Behrendt D, Sloan H, Ferguson P, Snedecor SM, Schork A. Long-term prognosis (15 to 26 years) after repair of tetralogy of Fallot: I - Survival and symptomatic status. . Ann Thorac Surg 1984;38:151–6. [18] Strieder DJ, Aziz K, Zaver AG, Fellows KE. Exercise tolerance after repair of tetralogy of Fallot. Ann Thorac Surg 1975;19:397– 405.
Total correction of tetralogy of fallot in adults-surgical experience M Rammohan, Balram Airan*, Anil Bhan, Rajesh Sharma, Sushant Srivastava, Anita´ Saxena, Kumar A. Sampath, P. Venugopal Department of Cardiothoracic and Vascular Surgery Cardiothoracic Sciences Centre, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110 029, India Received 16 July 1997; accepted 22 October 1997
Abstract A total of 100 patients of tetralogy of Fallot aged 13 years and over were operated upon at the All India Institute of Medical Sciences, New Delhi, India between January 1991 and December 1996. There were 69 males (69%) and 31 females (31%). Age ranged from 13 years to 43 years (mean 19.66 years). Twenty % of patients had preoperative complications like haemoptysis, cerebrovascular accidents, brain abscess and infective endocarditis. Twenty-two patients had previous palliative shunts. Fifteen patients had coil embolisation of major collaterals prior to surgery. In hospital mortality rate was 4%. Follow-up ranged from 1 month to 5 years (mean 3.4 years). There was one late death due to infective endocarditis. Postoperatively 93.6% patients were in NYHA class I. Significant residual defects warranting re-operation were present in three patients. Total correction of tetralogy of Fallot in older patients can be performed with acceptable results. 1998 Elsevier Science Ireland Ltd. Keywords: Tetralogy of Fallot; Adult patient; Total correction
1. Introduction Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease in which survival into adulthood is possible. Only 66% of patients survive up to 1 year of age without surgery; 49% to 3 years and 24% to 10 years of age [1]. The current trend is to perform total correction as early as possible and preferably during infancy. The results of total correction performed during infancy are superior to correction done later in life [2]. TOF in adults represents a special subset of patients with peculiar problems namely the effects of prolonged cyanosis, polycythemia, coagulation defects, development of collaterals, secondary myocardial dysfunction and *Corresponding author.
morphological and physiological consequences of previous palliative shunts. In our country, patients continue to present late in their life with one or more of the problems addressed to. We present our experience with 100 such patients who had the surgical correction of tetralogy of Fallot in adulthood (13 years and over) from All India Institute of Medical Sciences, New Delhi, India.
2. Materials and methods A total of 100 patients, 13 years of age and over with a diagnosis of tetralogy of Fallot were operated upon at our institute between January 1991 and December 1996. They constituted 8.65% of all total corrections for tetralogy of Fallot and 2.26% of all
0167-5273 / 98 / $19.00 1998 Elsevier Science Ireland Ltd. All rights reserved. PII S0167-5273( 97 )00279-9
122
M. Rammohan et al. / International Journal of Cardiology 63 (1998) 121 – 128
Table 1 Preoperative complications in adult patients with tetralogy of Fallot Haemoptysis Cerebrivascular accidents (brain abcess-4) Infective endocarditis Epistaxis
– –
10 7
– –
2 1
congenital cardiac surgeries performed during the same period. There were 69 males (69%). Ages of the patients ranged from 13 years to 43 years. The mean age at presentation was 19.66 years. At presentation 58 patients (58%) were in NYHA class II and 42 (42%) were in NYHA class III. Ten patients had history of haemoptysis and one had history of epistaxis. Two patients required hospital admission prior to surgery Table 2 Palliative surgeries performed in adult patients with tetralogy of Fallot Classical left BT shunt Right modified BT shunt Left modified BT shunt Central aortopulmonary shunt Pott’s shunt
– – – – –
9 8 4 2 1
because of massive haemoptysis. Seven patients had history of cerebrovascular accidents with residual hemiparesis in five. Of these seven patients, four had documented brain abscesses; two patients required repeated aspirations of brain abscess because of incomplete evacuation at the first sitting. In another three patients the cause was not documented. Two patients had preoperative endocarditis, one of whom had shunt endocarditis. (Table 1). Palliative Surgery: Twenty-two patients underwent 24 palliative systemic to pulmonary artery shunt operations prior to total correction (Table 2) (Fig. 1). Two patients underwent shunt surgeries twice. One of them had a right modified Blalock–Taussing shunt at 13 years of age. Fifteen days later the shunt got blocked and a left modified Blalock–Taussig shunt was performed. The other patient had left classical Blalock–Taussing shunt at 6 years of age. A central aortopulmonary shunt was performed at 28 years of age when the classical shunt was blocked. She underwent successful total correction after another 8 years. The time interval between palliative shunts and total correction varied from 20 months to 23 years
Fig. 1. Aortic arch injection in left anterior oblique view shows Pott’s shunt between descending thoracic aorta and left pulmonary artery (arrow) filling both pulmonary arteries.
M. Rammohan et al. / International Journal of Cardiology 63 (1998) 121 – 128 Table 3 Pulmonary anatomy in adult patients with tetralogy of Fallot Small main pulmonary artery Bifurcational stenosis Ostial stenosis of left pulmonary artery Small right pulmonary artery Small left pulmonary artery
– –
9 7
– – –
4 1 1
(mean 10.6 years). Four patients on angiography before total correction were found to have blocked shunts, three of whom had classical left Blalock]Taussig shunt and one patient had right modified Blalock–Taussig shunt. In only one patient, there was distortion of left pulmonary artery due to the previous left classical Blalock]Taussig shunt. None of the patients developed pulmonary hypertension or pulmonary vascular obstructive disease secondary to shunt including the patient who had Pott’s shunt at the age of 6 years and had total correction at the age of 43 years. Chest X-ray showed cardiac enlargement in 19 patients (19%). Reticular pattern due to well developed bronchopulmonary collaterals was evident in 26 patients (26%). Rib notching, due to long standing
123
Blalock–Taussig (BT) shunt was seen in three patients (3%). All patients underwent echocardiography and complete cardiac catheterisation and angiography. Echocardiography was particularly useful in delineating the aortomitral continuity and additional ventricular septal defects if any. On cardiac catheterisation right ventricular systolic pressures were typically equal to the systemic pressures in all patients. In 69 patients, pulmonary artery could not be entered. None of the previously shunted patients had pulmonary hypertension. Systemic arterial saturation varied from 65% to 89.5% (mean 78.5%). Angiography showed distortion of left pulmonary artery due to the previous left classical Blalock– Taussig shunt in one patient (Table 3). Shunt angiogram in previously palliated patients showed that four shunts were blocked. Angiography was useful in delineating coronary artery anomalies (Fig. 2). In five patients unexpected coronary arterial anomalies were detected in operating room. Aortopulmonary collaterals: Fifteen patients (15%) underwent coil embolization prior to surgery for significant aortopulmonary collaterals. Two patients needed surgical ligation. In one of the two, collateral
Fig. 2. Aortic root angiogram in right anterior oblique view shows common origin of right and left coronary arteries from right coronary sinus (arrow).
M. Rammohan et al. / International Journal of Cardiology 63 (1998) 121 – 128
124
Table 5 Associated procedures performed in adult patients with tetralogy of Fallot
was ligated at the time of making a systemic-pulmonary shunt and in the other patient it had to be ligated postoperatively because of significant left to right shunt. Associated anomalies (Table 4): One patient had aneurysmal right atrium along with cleft in the septal tricuspid leaflet with tricuspid valve prolapse causing severe tricuspid regurgitation. Another patient had a criss-cross pattern of ventricles with dextroposed left atrial appendage. Two patients were found to have healed vegetations in the operating room; in one on the tricuspid valve and in the other on the pulmonary valve. Operative details: Total correction was performed through median sternotomy. Cardiopulmonary bypass was established with standard aortic and bicaval cannulation. All functioning shunts were ligated at this stage. In one patient Pott’s shunt was closed under total circulatory arrest. Myocardial preservation was achieved by moderate hypothermia, multidose antegrade modified cold prime cardioplegia and topical ice slush. Left atrium was routinely vented. Transatrial approach was used in 50 patients (50%). In the other 50 patients transventricular correction was done. Interrupted mattress sutures were used to close the ventricular septal defect in 52 patients; in 30 patients continuous suture was used. Interrupted sutures along the posteroinferior margin of the ventricular septal defect and continuous suture along the rest of the ventricular septal defect margin were used in the rest. Transannular pericardial patch was placed in 60 patients (60%). The patch was extended up to the pulmonary trunk bifurcation or onto the proximal pulmonary artery branches in 13
Closure of previous palliative shunt Patch closure of ASDs a Tricuspid valve repair1right atrial aneurysm repair a
a b
Atrial septal defect. Ventricular septal defect.
– – – –
2 1 1 1
Right aortic arch Left superior vena cava Criss cross ventricles Dextroposed left atrial appendage Double aortic arch Retroaortic innominate vein Left subclavian artery stenosis Left subclavian artery aneurysm
18 2 1
Atrial septal defect
patients. In one patient, right pulmonary artery was augmented with pericardial patch. In four patients, the atrial septal defect was partially left open because of the borderline size of pulmonary arteries. Abnormal coronary arteries: Twelve patients had coronary artery anomalies. In six patients, left anterior descending artery was crossing the right ventricular outflow tract; of these, two patients had single coronary artery arising from right coronary sinus and in two other patients there was additional large left anterior descending artery arising from right coronary artery. In the remaining six patients a large conal branch was crossing the right ventricular outflow tract. Associated procedures: (Table 5). One patient underwent closure of the tricuspid cleft, tricuspid annuloplasty and repair of aneurysmal right atrium. Since 1995 we have been using Aprotonin prophylactically during total correction in adult tetralogy of Fallot patients to reduce the amount of blood loss. Since the same time, these patients were bled (one or two units depending upon the haematocrit) before surgery. The blood was infused postoperatively to control bleeding.
3. Results
3.1. Early results
Table 4 Associated anomalies in adult patients with tetralogy of Fallot Large ostium secundum ASDs a Multiple VSDs b Aneurysmal rightartrium Cleft tricuspid leaflet
– – –
– – –
28 6 1
– –
1 1
–
1
–
1
–
1
Hospital stay of the patients ranged from 6 days to 51 days (mean 13.7 days). Mortality: The early mortality was 4% (Table 6). Two patients died of low cardiac output on the 8th and 9th postoperative days. Echocardiography in both these patients showed poor right ventricular function Table 6 Causes of early mortality following tetralogy of Fallot repair in adults Low cardiac output Intrapulmonary haemorrhage Hepatorenal failure
– – –
2 1 1
M. Rammohan et al. / International Journal of Cardiology 63 (1998) 121 – 128
and there was no demonstrable residual ventricular septal defect or significant right ventricular outflow tract obstruction. One patient developed severe jaundice and acute renal failure on the 2nd postoperative day and died on the 10th postoperative day. One patient had persistent bleeding from the day of surgery and ultimately died of the 22nd postoperative day of massive intrapulmonary haemorrhage. Early complications: (Table 7). Four patients had complete atrioventricular conduction block in the early postoperative period. Three of these reverted spontaneously to normal sinus rhythm before discharge, while one patient needed permanent pacemaker implantation. One patient had generalised tonic-clonic convulsions on the 4th postoperative day. He recovered without any sequelae. One patient, a case of previous pulmonary tuberculosis had recrudescence and required antituberculous treatment.
3.2. Late results Follow-up ranged from 1 month to 5 years (mean 3.4 years). At the latest follow-up 89 patients (93.6%) were in NYHA functional class I and 6 patients (6.31%) in class II. One patient died 2 months after repair due to infective endocarditis. He had right ventricular outflow tract patch dehiscence and underwent repeat surgery for patch reconstruction. He finally succumbed to persistent toxaemia and multiorgan failure. Forty patients, randomly selected, had postoperative echocardiographic examination. Echocardiography showed significant residual ventricular septal defect in three patients, one of whom had significant right ventricular outflow tract obstruction also. One patient had right ventricular diastolic dysfunction and another had moderate tricuspid regurgitation. Four other patients had small residual ventricular septal defects of no haemodynamic significance.
Excessive bleeding Transient complete heart block Pleural effusion Permanent complete heart block Haematemesis Sternal wound infection Recrudescence of pulmonary tuberculosis
– – – – – – –
Cardiac catherisation was performed in three patients which included two patients who had residual ventricular septal defect on echocardiography and one patient who was persistently symptomatic and in whom echocardiographic examination was not conclusive. In this patient, the cardiac catheterisation was also normal. Cardiac catheterisation in the other two patients with significant residual ventricular septal defect showed significant left to right shunt.
3.3. Late complications Table 8 Five patients had congestive cardiac failure at 1, 2.5, 2.5, 3 and 6 months after surgery (mean 3 months). Echocardiography did not show residual surgical lesion in any of these patients. Tow patients had right ventricular dysfunction and one patient had biventricular dysfunction. They were managed conservatively and all of them recovered well. One patient had tuberculous pericardial effusion 2 months postoperatively which was managed with antituberculous treatment and catheter drainage. None of the patients had significant arrhythmias on follow-up. Two patients had persistent atrial bigeminy which responded to discontinuation of digoxin. Patients who had tricuspid regurgitation and right ventricular diastolic dysfunction are asymptomatic on medication presently. None of the patients had left ventricular dysfunction or pulmonary incompetence. Re-operation: Four patients underwent re-operation at 2, 2, 5 and 22 months after total correction. Three patients had closure of residual ventricular septal defects one of whom had residual right ventricular outflow tract obstruction as well. One patient underTable 8 Late complications following tetralogy of Fallot repair in adults Residual VSDs a Tricuspid regurgitation Right ventricular diastolic dysfunction
Table 7 Early complications following tetralogy of Fallot repair in adults 15 3 3 1 1 1 1
125
a
– – –
3 1 1
Congestive cardiac failure Pericardial effusion Arrhythmias Pleural effusion Convulsions RVOT b patch dehiscence
– – – – – –
Ventricular septal defect (one of these patients had associated significant right ventricular outflow tract obstruction). b Right ventricular outflow tract (infective endocarditis was the cause of right ventricular outflow tract patch dehiscence and residual vertricular septal defect in one patient each).
5 2 2 1 1 1
126
M. Rammohan et al. / International Journal of Cardiology 63 (1998) 121 – 128
went right ventricular outflow tract patch reconstruction twice for patch dehiscence secondary to infective endocarditis. He died one month after surgery due to multiorgan failure and toxaemia.
4. Discussion Survival to adulthood despite chronic cyanosis in some patients with tetralogy of Fallot is partly due to the less severe form of the disease and development of collaterals leading to increased pulmonary blood flow [3,4]. Since the first successful surgical correction of tetralogy of Fallot by Lillehei et al., [5] in 1955, there has been tremendous improvement in surgical techniques and methods of myocardial protection. Consequently the morbidity and mortality following repair of tetralogy of Fallot has drastically reduced. The present day consensus is to perform total correction at the earliest preferably during infancy if the anatomy is suitable. Not many series of results of total correction of tetralogy of Fallot in adults are available. In our study, twenty patients had complications of tetralogy of Fallot prior to surgery and most of these were due to the presence of extensive bronchial collaterals (ten patients had haemoptysis). This is comparable with the experience of Presbitero et al., [6] and Stanley John et al., [7]. The incidence of haemorrhagic and neurologic complications in adults is definitely more compared to childhood. In our study, more than half of the patients with bleeding tendencies, namely haemoptysis and epistaxis required repeated hospital admissions prior to total correction. Sizeable collaterals requiring intervention were present in seventeen patients (17%) in our experience. Fifteen of these patients had successful preoperative coil embolisation. Surgical intervention was needed in two cases. In contrast to reports of Abraham et al., [4] and Stanley John et al., [7] who reported 15% incidence of congestive cardiac failure, none of our patients had such complication in the preoperative period. The high incidence of cardiac failure was attributed to long standing pressure overload and the consequent pathological changes in the right ventricle [7]. Twenty-two patients (22%) in our study underwent previous palliative shunt operations prior to total
correction. In the experience of Presbitero et al., [6] and Mattila et al., [8] more than half of the patients received preliminary shunts. Pulmonary artery distortion consequent to such shunts was seen in only one of our patients in whom the left pulmonary artery was distorted due to left classical Blalock–Taussig shunt. None of the patients including the one who had Pott’s shunt developed pulmonary vascular obstructive disease. In contrast to the report of Nandi and coworkers [9] we did not find increased mortality after total correction in patients who underwent palliative shunt operations previously. Excessive bleeding was frequently encountered during the dissection of old shunts which however did not alter the mortality rate in our experience. Most of the early morbidity in our series was due to the excessive bleeding following surgery. Fifteen patients (15%) had such complication. As mentioned earlier, we used Aprotonin and autologous blood in patients where excessive bleeding was anticipated. This resulted in reduced blood loss during and after surgery. Although we do not have controlled study, based on initial experience, we feel that this two options might be effective in reducing blood loss in these patients. Complete heart block is uncommon following total correction. Only 1.3% of patients of all ages in Kirklin’s series [10] had this complication. In our study only one patient required permanent pacemaker implantation. Casteneda [11] and Kirklin et al., [10] in their total experience had a mortality between 2% and 5%. Kirklin et al., [10] found that old age was an incremental risk factor for early mortality following total correction. Our early mortality of 4% is comparable to that of others [6,7,11]. Based on our results, we feel that old age may not be an incremental risk factor with the current mode of intraoperative and postoperative management. Although our follow-up period is not very long, there was only one late death. David Hu et al., [12] reported the results of total correction in 32 patients who were 40 years of age or older at the time of surgery. They followed the patients up to a mean of 110 months. Five patients died of cardiovascular related causes in their series. There were four late deaths out of 39 adults patients who underwent total correction in the study of Presbitero et al., [13]. Both groups proved that total correction in adult patients
M. Rammohan et al. / International Journal of Cardiology 63 (1998) 121 – 128
could be performed with less incidence of early and late mortality. Presbitero et al., [13] found significant arrhythmias in at least nine out of 39 adult patients on follow-up. Bifascicular block was found in 18% of patients one of whom died suddenly. Our results are more encouraging. Only two patients had persistent atrial bigeminy on follow-up which responded to discontinuation of digoxin. The exact incidence of infective endocarditis in adults following total correction is not known. Two patients in our study had infective endocarditis within 6 months of surgery. One of them died following right ventricular outflow tract patch dehiscence. Residual right ventricular outflow tract obstruction following total correction is less common in adults than in children most probably because of milder degrees of right ventricular outflow tract narrowing. Significant right ventricular outflow tract obstruction occurred in one out of 96 late survivors in our series. Although residual ventricular septal defects occur postoperatively, less than 1% of them are significant enough to need re-operation. [14] Mattila et al., [8] reported two cases following total correction in 39 adults who needed re-operation. We had three cases (3.1%) of significant residual ventricular septal defects out of 96 patients, (one of which was due to infective endocarditis) which needed re-operation and had successful outcome. The use of transannular pericardial patch did not result in increased operative mortality or significant pulmonary regurgitation in our patients. This is consistent with the results of Casteneda et al., [11]. We are of the opinion that adequate use of transannular patch should prevent the important complication of residual right ventricular outflow tract obstruction in adults who had already suffering from long standing right ventricular hypertension. We did not find any difference in the outcome after total correction done by transatrial or transventricular route. Karl and Mee et al., [15] advocate transatrial approach because of the late right ventricular dilatation and dysfunction and increased risk of ventricular arrhythmias that might follow ventriculotomy. Although our follow-up period is short, we did not encounter such complications following transventricular approach. 93.6% of patients following surgery in our study
127
are in NYHA class I. Our results are similar to those of Katz et al., [16] and Rosenthal et al., [17] who reported more than 90% improvement in functional status following total correction at all age groups. However Strieder [18] found that functional results were not as good in older patients when assessed by exercise tolerance test. We are yet to follow our patients when exercise tolerance test.
5. Conclusion Total correction of tetralogy of Fallot can be performed in older patients with good results. Functional improvement following surgery is marked and most of the patients can return to normal life. The morbidity and mortality in our experience do not alter the outcome. In our experience the incidence of residual defects is quite low.
References [1] Bertranou EG, Blackstone EH, Hazelrig JB, Turner Jr. ME, Kirklin JW. Life expectancy without surgery in tetralogy of Fallot. Am J Cardiol 1978;42:458–66. [2] Casteneda AR, Freed MD, William RG, Norwood WI. Repair of tetralogy of Fallot in infancy. Early and late results. J Thorac Cardiovasc Surg 1977;74:372–81. [3] Higgins CB, Mulder DG. Tetralogy of Fallot in the adult. Am J Cardiol 1972;29:837–46. [4] Abraham KA, Cherian G, Dayasagar RV, Sukumar IP, Krishnaswami S, Stanley J. Tetralogy of Fallot in adults. A report on 147 patients. Am J Med 1979;66:811–6. [5] Lillehei CW, Cohen M, Warden HE, et al. Direct vision intracardiac surgical correction of tetralogy of Fallot, pentology of Fallot and pulmonary atresia defects, report of first ten cases. Ann Surg 1955; 418–45. [6] 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. [7] Stanley J, Kejriwal NK, Ravikumar E, Bashi VV, Mohanty BB, Sukumar IP. The clinical profile and surgical treatment of tetralogy of Fallot in the adult: results of repair in 200 patients. Ann Thorac Surg 1986;41:502–6. [8] Mattila S, Luosto R, Ketonen P, Nieminen M, Merikallio E, Kyllnen EJ. Total correction of tetralogy of Fallot in adults: Scan. J Thorac Cardiovasc 1984;18:23–7. [9] Nandi PL, Chaung KL, Mok CK. Total correction of tetralogy of Fallot: a review of 280 cases. JR Coll Surg Edinb 1981;26:340–5. [10] Kirklin JW, Blackstone EH, Jonas RA et al. Morphologic and surgical determinants of outcome events after repair of tetralogy of Fallot and pulmonary stenosis. J Thorac Cardiovasc Surg 1992;103:706–23.
128
M. Rammohan et al. / International Journal of Cardiology 63 (1998) 121 – 128
[11] Casteneda AR. Classical repair of tetralogy of Fallot: Timing, technique and results. Semin Thorac Cardiovasc Surg 1990;2:70–9. [12] David HCK, Seward JB, Puga FJ, Fuster V, Tajik AJ. Total correction of tetralogy of Fallot at age 40 years and older: long term followup. J Am Coll Cardiol 1985;1:40–4. [13] Presbitero P, Demarie D, Aruta E et al. Results of total correction of tetralogy of Fallot performed in adults. Updated in 1996. Ann Thorac Surg 1996;61:1870–3. [14] Dobell ARC, Charrette EP, Chughtai MS. Correction of tetralogy of Fallot in the young child. J Thorac Cardiovasc Surg 1968;55:70–9. [15] Karl TR, Sano S, Pornviliwan S, Mee RBB. Tetralogy of Fallot: favourable outcome of non neonatal transatrial, transpulmonary repair. Ann Thorac Surg 1992;54:903–7.
[16] Katz NM, Blackstone EH, Kirklin JW, Pacifico AD, Bergeron Jr. LM. Late survival and symptoms after repair of tetralogy of Fallot. Circulation 1982;65:403–8. [17] Rosenthal A, Behrendt D, Sloan H, Ferguson P, Snedecor SM, Schork A. Long-term prognosis (15 to 26 years) after repair of tetralogy of Fallot: I - Survival and symptomatic status. . Ann Thorac Surg 1984;38:151–6. [18] Strieder DJ, Aziz K, Zaver AG, Fellows KE. Exercise tolerance after repair of tetralogy of Fallot. Ann Thorac Surg 1975;19:397– 405.