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Ann Thorac Surg 1996;61:1870-1873
© 1996 The Society of Thoracic Surgeons
As Originally Published in 1988:
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
Although 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.
Material and Methods
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 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% (1/40), 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 mortality 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 cardiovascular-related 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 bundle-branch block, and postoperative Holter monitoring revealed ventricular arrhythmias of Lown grade III. The third patient died of congestive heart failure at age 45 years, 13 years after complete repair, having had a previous Potts 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 ventricular 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).
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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-up. The postoperative problems in this category of patients are the same as those encountered in young patients, but with some peculiarities related to the long-lasting impaired hemodynamic conditions.
Left Ventricular Function
Although left ventricular failure is extremely rare after operation in tetralogy of Fallot, some studies [2] have demonstrated that left ventricular volume and pressure parameters are impaired after total correction in childhood. Long-term trends [3] indicate that left ventricular ejection fraction may continue to decrease with time. The impairment in left ventricular function could be explained by the fibrosis following long-lasting cyanosis, with consequent chronic myocardial hypoxaemia, and by altered contraction of the interventricular septum due to the presence of a prosthetic patch. Furthermore, the collateral circulation from systemic to pulmonary 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 and mortality in patients who had large shunts like Waterston or Potts shunts before their total correction. However, in our series only 1 patient died of congestive heart failure, and in another 1 with chronic heart failure improvement was achieved by aortic valve replacement, even though most of our patients were palliated with shunts. So, at least for medium-term follow up, this problem does not appear relevant.
Pulmonary Hypertension
No pulmonary vascular disease developed in our patients, but 2 patients were excluded from this study at the time of operation because of the presence of pulmonary hypertension. Both these patients had had a Potts anastomosis.
Residual Defects
Severe residual pulmonary 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. Pulmonary regurgitation also is more often mild, due to fewer and smaller transannular patches (only 30% of our patients).
Residual ventricular septal defects were present in our patients with the same frequency as in other series of patients operated on for tetralogy of Fallot at a younger age.
The most significant valvular abnormalities are aortic regurgitation, present in 10% of our patients, and tricuspid regurgitation, present 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 happened in 1 of our patients, who need aortic replacement at the time of surgical correction. Aortic valve replacement is needed when the left ventricle starts to fail, because a volume overload is not well tolerated by an already impaired left ventricle.
Tricuspid valve regurgitation can be the result of a dilated right ventricle because of other residual defects, such as residual pulmonary stenosis. In this case it is important 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 arrhythmias are more frequent in older patients with tetralogy of Fallot. The incidence is about 12% to 17% in the patients operated on at a young age (1 to 7 years), and 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 sudden death. However, it has not been proved that spontaneous or induced ventricular arrhythmias, after surgical correction, indicate an elevated risk of sudden death or that suppression of these arrhythmias protects patients against sudden death.
In our series, 1 of the 2 patients who died suddenly had significant arrhythmias (Lown > III), 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 hemodynamic conditions are still alive (3 treated with sotalol). A comparison of the high incidence of ventricular arrhythmias (40%) and the incidence of late sudden 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 hemodynamic 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 hemodynamic problems and 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 improvement in functional class, and the long-term survival is better than in patients with only medical treatment. However, patients should be carefully followed up to diagnose residual hemodynamic abnormalities and significant rhythm disturbances.
Footnotes
Address reprint requests to Dr Presbitero, Piazza Donatori del Sangue 3, 10154 Torino, Italy.
References
This article has been cited by other articles:
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  A. Sadiq, K. G. Shyamkrishnan, S. Theodore, S. Gopalakrishnan, J. M. Tharakan, and J. Karunakaran Long-Term Functional Assessment After Correction of Tetralogy of Fallot in Adulthood Ann. Thorac. Surg., May 1, 2007; 83(5): 1790 - 1795. [Abstract] [Full Text] [PDF]
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J. Horer, J. Friebe, C. Schreiber, M. Kostolny, J. Cleuziou, K. Holper, and R. Lange Correction of Tetralogy of Fallot and of Pulmonary Atresia with Ventricular Septal Defect in Adults Ann. Thorac. Surg., December 1, 2005; 80(6): 2285 - 2291. [Abstract] [Full Text] [PDF]
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 F. A. Atik, E. Atik, C. R. da Cunha, L. F. Caneo, R. S. Assad, M. B. Jatene, A. Riso, and M. Barbero-Marcial Long-term results of correction of tetralogy of Fallot in adulthood Eur. J. Cardiothorac. Surg., February 1, 2004; 25(2): 250 - 255. [Abstract] [Full Text] [PDF]
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 J. Heggie, N. Poirer, W. G. Williams, and J. Karski Anesthetic Considerations for Adult Cardiac Surgery Patients with Congenital Heart Disease Seminars in Cardiothoracic and Vascular Anesthesia, June 1, 2003; 7(2): 141 - 152. [Abstract] [PDF]
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 M. E. Brickner, L. D. Hillis, and R. A. Lange Congenital Heart Disease in Adults- Second of Two Parts N. Engl. J. Med., February 3, 2000; 342(5): 334 - 342. [Full Text] [PDF]
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