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Eur J Cardiothorac Surg 1999;16:125-130
© 1999 Elsevier Science NL
Department of Cardiothoracic Surgery, RT 2152 Rigshospitalet, Blegdamsvej 9, 2100 Copenhagen Ø, Denmark
Corresponding author. Tel.: +45-3545-2152; fax: +44-3544-2548
e-mail: bar{at}rh.dk
| Abstract |
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Key Words: Congenital heart disease Tetralogy of Fallot Surgery Long term follow-up Quality of life
| 1. Introduction |
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Most previous papers have presented long-term follow-up with a mean follow-up of 520 years [612].
In 1982, the 1419-year follow-up of our early series was published [13]. The object of the present study was to present the 2037-year follow-up of our material.
| 2. Material and methods |
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2.1. Surgery
One hundred and eighty five patients underwent corrective repair of Tetralogy of Fallot with pulmonary stenosis at our institution between January 1960 and July 1977.
Prior to Tetralogy of Fallot repair, 97 patients had undergone a palliative surgical procedure. Seventy four had received a Blalock-Taussig shunt, nine had received pulmonary valvulotomy, 11 had received a Waterston-shunt, six had received a Brocks infundibulotomy, three had received a Pott-shunt, and four had received a Barrett pleurodesis. Sixteen patients had received more than one palliative procedure. Three patients had been explored via a thoracotomy, without any attempt of palliation performed.
Repair including relief of the right ventricular outflow obstruction and closure of the VSD was performed with techniques and approaches corresponding to time. The VSDs were closed with a transventricular approach, always by use of patch. Muscle removal was generally more generous than today. A RV/LV of less than 0.75 was accepted.
About one-third of the patients received a transannular right ventricular outflow patch (pericardium or dacron) while three patients received valved conduits (one Polystan® pulmonary valve conduit and two Hancock conduits).
2.2. Statistics
Survival data have been evaluated using KaplanMeiers product limit method. Comparisons between groups have been made using a log-rank test or chi square test, where appropriate
| 3. Results |
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The operative activity during the following periods was: 19601964: 53 (27 discharged), 19651969: 27 (15 discharged), 19701974: 53 (39 discharged) and 19751977: 52 (44 discharged) (Fig. 1). Among operative survivors median age at operation was 12.8 years (range 5 months41 years) (Fig. 2). At the follow-up, 109 patients were alive while another 16 patients had died, six of the reoperated.
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One patient underwent an aortic valve repair for severe insufficiency. One patient was given a pulmonary stenosis repair by mono-cusp outflow patch implantation at the age of 13 and was operated for tricuspid insufficiency and persistent VSD 1 year later, finally to be heart transplanted at the age of 23, but died 3 weeks postoperatively.
Three patients died in immediate relation to the reoperation, including the heart transplant patient already mentioned. A further three of the reoperated patients died later.
3.3. Survival and causes of late death
Although previous palliative procedures were associated with increased operative mortality (P=0.04 by Chi-square test), there were no differences in survival (from birth and from Fallot repair) between operative survivors who had had a palliative procedure prior to the Fallot repair and those who received the repair as the primary operation (Fig. 3). Sixteen patients had died between discharge and follow-up (1997). The causes of death were all cardiac, seven were sudden deaths probably related to arrhythmias. The time and causes of death are presented in Table 1. Three patients died early following the reoperation and another three died late.
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Fifty two patients (28 female and 24 male) out of 97 who answered the question had one or more children. Two of the female patients had each given birth to a child with Tetralogy of Fallot, while all other children were healthy and none had congenital heart disease.
Seventeen patients out of 100 who answered the question were presently using some kind of cardiac medication (10 used anti-arrhythmics, 10 used diuretics, two used digoxin and two used anticoagulative medication).
Fifty patients out of 99 who answered the question performed sport activities, primarily athletics, badminton, swimming and bicycling.
Eighty nine patients out of 105 who answered the question were working in a variety of ordinary professions. All kinds of professions from academics to hard manual labor were represented. Out of the 16 who were not in employment, two were housewives, four were unemployed, one was retired, and eight received disability pension. The impression was that many of these patients did not attend regular medical check-ups.
Medical reexaminations were almost exclusively performed as a consequence of symptoms.
| 4. Discussion |
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In the early era of Tetralogy of Fallot repair, the use of a right ventricular outflow patch was associated with very high risk and there are only 10 patients with outflow patches among our survivors. Additional surgical technical causes of fatal outcome were: insufficient relief of the right ventricular outflow tract obstruction, A-V block, bleeding, injury to the aortic valve and cardiac failure possibly caused by poor myocardial protection. The poor surgical technique was also the main cause of reoperation for ineffective VSD closure. Although the operative mortality was high, the frequency of reoperations was the same as that reported by others for the same indications [12].
Since detailed information about remaining obstruction and/or insufficiency are lacking in this study, our results might be interpreted as if some residual right ventricular stenosis and/or insufficiency is well tolerated by most patients and this is also in agreement with the observation of others [7]. Miyamura et al. [14] presented a 2029-year follow-up of 50 patients and showed no adverse effects of transannular patching. The negative influence of pulmonary insufficiency on early and late outcome has, however, been stressed by others, both in relation to working capacity and risk of arrhythmias, reoperation and/or late death [15]. Vetter et al. [16] demonstrated the relation between transannular patching and increased right ventricular volume and reduced working capacity following repair of Fallot in an echocardiographic and exercise study. Zhao et al. [12] found that the length of the transannular patch was a predictor of the need for reoperation. Because of the very high operative mortality associated with right ventricular outflow patch, patients without outflow patch are over-represented in our series in comparison with later series.
How these results should be interpreted in relation to the use of valved conduits in the repair of Fallot with a need for transannular patching, is therefore not clear. Monocusp valves might serve to reduce the immediate postoperative problems, while most of them will have no long-term function but probably do little or no harm. Valved conduits (homografts and others) will serve well in the early postoperative period but eventually all will deteriorate and become obstructed and will have to be replaced with regular intervals (1015 years or less) [17]. The outcome in this series justifies the advice to minimize pulmonary insufficiency, as suggested above with the conservative approach to outflow patching, and reserve valved conduits for patients with an additional indication usually including a suspicion of increased pulmonary vascular resistance. The study by Norgard et al. [18] of the right ventricular diastolic function following Tetralogy of Fallot repair indicates that a restrictive physiology might be beneficial to long-term outcome. In the present material, only one patient was recently given a homograft in the right ventricular outflow tract for severe pulmonary insufficiency and ventricular dilatation.
In this series, there were seven late, sudden deaths indicating an important problem to look for and try to prevent. This incidence is identical to that observed by Jonsson et al. [19] while Waien et al. [11] found a very low risk in adults with previous repair. The cause of sudden death may be heart block or taccyarrhythmia/ventricular fibrillation. A trifascicular block pattern was associated with early sudden death [20]. The risk of symptomatic arrhythmia is high when marked right ventricular enlargement and QRS prolongation develop [21]. QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death [21]. Earlier repair seems to reduce the risk of late ventricular arrhythmias [19]. Deanfield et al. [22] have found late Tetralogy of Fallot repair to be associated with more arrhythmias. This might explain our high incidence of late, sudden death. Transatrial VSD closure with short transannular incision, less aggressive muscle resection in right ventricular outflow tract and less generous outflow patching have been found to be associated with reduced ventricular arrhythmias and less right ventricular dilatation/dysfunction and improved working capacity [23,24]. Diagnostic possibilities and treatment options have increased, and today the treatment includes the option of cardioverter-defibrillator implantation. Hopefully, more aggressive medical treatment of ventricular arrhythmias, will reduce the number of sudden deaths. Arrhythmias after repair of Tetralogy of Fallot also include atrial taccyarrhythmias [25].
As a consequence of the present study, the patients will be approached and offered a check-up by a qualified cardiologist. It will take a long time before all patients have been reexamined. It is possible that a few patients in the series will be in need of a reoperation, e.g. those three who primarily received valved conduits. In addition, such a follow-up program can be expected to produce additional interesting information.
| 5. Conclusions |
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| Footnotes |
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| Appendix A |
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Dr Norgaard: We expect that if the patients had been followed closer we may have been able to discover some cardiac arrhythmias and intervene. As a consequence of this study, we will offer all the patients that are still alive to be followed closely by cardiologists.
Dr C. Knott-Craig (Oklahoma, OK): With respect to the late deaths, were any of those related to exercise? How do you advise your patients whether they are able to exercise? For example, do you do an exercise Holter monitoring before allowing them to exercise, or do you just give them carte blanche ability to exercise if they feel fit enough?
Dr Norgaard: We have not pointed out any kind of restrictions to these patients. We think if the patients should really benefit from Fallot repair, they should be able to live a normal life, and at controls performed by cardiologists, cardiac arrhythmias should be discovered.
Dr Knott-Craig: Were any of the late deaths related to exercise at all?
Dr Norgaard: We don't know what the patients were doing at the time of death.
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