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Right Ventricular Dysfunction and Pulmonary Valve Replacement After Correction of Tetralogy of Fallot Frank T. H. de Ruijter, MD, Ineke Weenink, MD, Francois J. Hitchcock, MD, Erik J. Meijboom, MD, PhD, and Ger B. W. E. Bennink, MD, PhD Children’s Heart Center, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, The Netherlands Background. Correction of tetralogy of Fallot often leads to pulmonary regurgitation, sometimes warranting pulmonary valve replacement, for which indications and timing to achieve optimal results are not yet clear. This retrospective study describes follow-up and reinterventions in our tetralogy of Fallot population. Methods. Review of all consecutive patients operated on for tetralogy of Fallot between 1977 and 2000 was conducted. Included are date and type of repair, Doppler echocardiography (two-dimensional echocardiography), electrocardiographs, reoperations, and physical condition. Results. Total repair was performed in 171 patients at a mean age 1.9 ⴞ 2.5 years, follow-up time counted 9.6 ⴞ 7.0 years. Right ventriculotomy was used in 92%, and transatrial ventricular septal defect closure was used in 8%; 74% received a transannular outflow patch. Twentyyear survival was 91%. Last follow-up electrocardiographs showed right bundle branch block in 67% and serious arrhythmias in 11%. Two-dimensional echocardiography demonstrated severe pulmonary insufficiency and dilated right ventricle in 31% and 38%, respectively, increasing with postrepair age (p < 0.001). Poor clinical condition (New York Heart Association class IIⴙ) and echocardiographic proof of right atrial dilatation (p ⴝ 0.012) and arrhythmias (p ⴝ 0.03) were significantly associated. Furthermore, the influence of residual hemodynamic lesions, such as a remaining ventricular septal defect or pulmonary stenosis, or right ventricular dilatation was important (p ⴝ 0.04). Reintervention was necessary in 32 patients (19%; 10-year freedom, 83%), including angioplasty for residual stenosis and pulmonary valve replacement. At a mean age of 9.2 years after correction, 14 patients received a homograft, and 2 patients received a heterograft. In 7 patients the right ventricle returned to normal dimensions and symptoms disappeared. The incidence of right ventricular dilatation was considerably higher (p ⴝ 0.020) in patients with a transannular patch; the transatrial approach showed the opposite (p ⴝ 0.03), and patients presented with lower QRS duration (p ⴝ 0.007), although no difference could be found between survival after both surgical techniques. Effects of early timing (correction < 6 months) on right ventricular dysfunction could not be established. Conclusions. Severe right ventricular dilatation and pulmonary regurgitation secondary to outflow tract repair in tetralogy of Fallot are frequently occurring sequelae developing slowly over time. Indications for pulmonary valve replacement remain controversial because echocardiographic findings or arrhythmias are not always accompanied by deterioration of clinical condition. However, right atrial dilatation and additional hemodynamic lesions demand increased vigilance. Transatrial repair is associated with a favorable outcome. (Ann Thorac Surg 2002;73:1794 – 800) © 2002 by The Society of Thoracic Surgeons N up, pulmonary valve insufficiency is frequently encountered even in asymptomatic patients [5–7]. Severe longstanding pulmonary regurgitation (PR) can lead to deleterious complications [1, 2, 8 –15]. In a small number of patients with both deterioration of symptomatic status and objective evidence of progressive right heart failure, pulmonary valve replacement (PVR) is considered to relieve symptoms [1, 4, 16 –20]; this number is expected to grow [16, 21], especially those in whom a transannular patch was used [22]. But specific indications and timing of PVR remain unclear. This study is an inventory of our tetralogy of Fallot population from initial repair to present status, focusing on the development of RV indicators and occurrence of reinterventions in follow-up with special attention to PVR. owadays, clinical management of tetralogy of Fallot (obstruction of right ventricular [RV] outflow, malalignment ventricular septum defect, and RV hypertrophy) involves a total surgical correction in early childhood. This decreases the necessity of prior palliative shunt procedures and has a low operative mortality and excellent long-term results [1–5]. However, in the follow- Presented at the Forty-eighth Annual Meeting of the Southern Thoracic Surgical Association, San Antonio, TX, Nov 8 –10, 2001. Address reprint requests to Dr Bennink, Department of Cardiothoracic Surgery, Pediatric Heart Center, Lundlaan 6, KG 01.319.0 3584 EA Utrecht, The Netherlands; e-mail: [email protected]. © 2002 by The Society of Thoracic Surgeons Published by Elsevier Science Inc 0003-4975/02/$22.00 PII S0003-4975(02)03586-5 Ann Thorac Surg 2002;73:1794 – 800 DE RUIJTER ET AL RIGHT VENTRICULAR DYSFUNCTION AND PULMONARY VALVE REPLACEMENT 1795 Material and Methods Population The reports of all consecutive surviving patients, operated on in the Children’s Heart Center Utrecht between 1977 and 2000 and residing in the Netherlands, were examined retrospectively. The population (n ⫽ 171) consisted of 106 (62%) male and 65 female patients with a mean follow-up time of 9.6 ⫾ 7.0 years (range, 0.2 to 32.6 years; median, 8.0) and mean age of 13.8 ⫾ 8.0 years (range, 0.2 to 37.8 years). Excluded from this survey were 99 patients with follow-up abroad directly after operation (82), pulmonary atresia (13), complete atrioventricular septal defect, subaortic conus, or death before complete correction (2). Uncomplicated tetralogy of Fallot was encountered in 150 patients, 12% (n ⫽ 21) had additional cardiac complications (abnormal coronary artery, systemic to pulmonary collaterals, pulmonary artery hypoplasia, branch stenosis). Pentalogy (with atrial septal defect) was reported in 23 (13%), 12 had a persistent ductus arteriosus, and 21 a bicuspid pulmonary valve. Comorbidity included Down syndrome, velocardiofacial syndrome, and VATER association (vertebral defects, imperforate anus, tracheoesophageal fistula, and radial and renal dysplasia) in 47 patients (27%). In 33 patients (19%) a palliative shunt was initially required to overcome symptoms (age, 0.9 ⫾ 1.4 years; Blalock-Taussig, 8; central aortopulmonary, 25). Total correction was performed at a mean age of 1.9 ⫾ 2.5 years (range, 0.2 to 21.7 years). Most patients were corrected using a transannular patch (n ⫽ 126, 74%) in the RV outflow tract. In the remaining 26% infundibulectomy or pulmonary valve commissurotomy was sufficient to overcome the obstruction. In the majority the ventricular septal defect (VSD) was repaired through right ventriculotomy (157 patients, 92%); only 14 patients underwent transatrial repair. Mean follow-up time postoperatively was 8.0 ⫾ 6.5 years (range, 0 to 27.9 years; median, 6.3 years). Doppler echocardiographic data (especially RV dimensions, pulmonary stenosis and regurgitation, right atrial dilatation, and tricuspid insufficiency), cardiac catheterizations, electrocardiographs, and 24-hour Holter recordings (QRS complex duration, dysrhythmias) were reviewed. Furthermore, physical condition (New York Heart Association functional class I to IV), cause and time of death, age, indications, and type of reintervention were recorded. Right Ventricular Dilatation and Pulmonary Regurgitation Echocardiographic data (two-dimensional views) describing the RV were categorized as follows: a normal RV, ie, left ventricle to RV ratio of 3:1, was attributed the number 0 or none, a severely dilated RV (RV size equal or even exceeding left ventricular dimensions) was assigned the value 2, and anything in between was classified as a (mildly) dilated RV, associated with the number 1. Pulmonary valve incompetence was classified by the cardi- Fig 1. Actuarial survival as a function of age. ologist on a scale from 1 to 4, 1 meaning trivial, 2 mild, 3 and 4 are in this study regarded as severe. Statistical Analysis All data are presented as the mean value ⫾ one standard deviation (range). Probability of survival, freedom of reintervention, and PVR after repair were calculated by Kaplan-Meier analysis with the use of the SPSS program (SPSS Inc, OH). Differences among groups were calculated with Fisher’s exact test, log rank test, and Student’s t test. For the time analysis of RV dilatation and PR, data were clustered and adjusted for dependence of the outcome within a patient. The Mixor program (version 2) [23] then provided a graph from the mean probability in every category and year after corrective operation. Results Follow-Up A total of 16 patients died during follow-up after complete repair. However, of the 11 deaths almost immediately after the operation, 2 patients had tracheobronchomalacia, 1 suffered from cystic fibrosis combined with septic shock, and 2 from trisomy 13 and VATER association. Late deaths included 3 unrelated deaths (eg, car accident) and 2 patients with sudden cardiac death. Actuarial survival was 91% at both 10 and 20 years (Fig 1). Total mortality attributed only to the heart disease itself counted 4.7%. At latest follow-up date most patients are in New York Heart Association class I, yet a considerable number of them presented with some insufficiency of the pulmonary valve, RV dilatation, tricuspid valve regurgitation, and right atrial dilatation (Table 1). A significant association between poor clinical condition (New York Heart Association functional class II⫹) and objective echocardiographic measurements was only found in the demonstration of right atrial dilatation (p ⫽ 0.012); tricuspid insufficiency came close (p ⫽ 0.08). The presence of arrhythmias was also related to poor condition (p ⫽ 0.03). 1796 DE RUIJTER ET AL RIGHT VENTRICULAR DYSFUNCTION AND PULMONARY VALVE REPLACEMENT Ann Thorac Surg 2002;73:1794 – 800 Table 1. Patient Data at Last Follow-Up PVR Characteristic n Deaths (early ⫹ lost to FU) Follow-up age postoperatively PVR Reinterventions except PVR n Significant arrhythmias Severe PR Severe RV dilatation Tricuspid insufficiency RA dilatation Pulmonary stenosis ⬎30 mm Hg All Patients Without PVR 171 16 (15) 8.0 ⫾ 6.5 22 NYHA I NYHA II⫹ 155 14 8.1 ⫾ 9.3 16 9.2 ⫾ 6.7 5 4.4 ⫾ 6.6 1 133 13 10 48 56 63 19 20 0 15 6 5 13 12 5 6 12 3 7 1 4 5 3 6 140 16 11 (8%) 44 (31%) 53 (38%) 56 (40%) 16 (11%) 20 (14%) 6 (38%) 5 (31%) 7 (43%) 12 (75%) 6 (38%) 6 (38%) FU ⫽ follow-up; NYHA ⫽ New York Heart Association; atrial; RV ⫽ right ventricular. NYHA I NYHA II⫹ PR ⫽ pulmonary regurgitation; In 15% (26 patients), RV outflow tract aneurysms were detected (ranging from trivial to mild, only one requiring reoperation). In 26% (44 patients) echocardiography showed a residual VSD; 9 were closed and 35 were very small or closed spontaneously. Postrepair cardiac catheterization was performed in 38 patients, mostly associated with PVR or to quantify pulmonary stenosis before angioplasty. Arrhythmias Cardiac arrhythmias on routine electrocardiographic examinations were prevalent, especially complete right bundle branch block (67%). First-degree atrioventricular block was found in 16 patients; in 6 patients this preceded the onset of more complex rhythm disturbances. Furthermore, 1 patient with Wolf-Parkinson-White syndrome and 1 with sick sinus syndrome were encountered. Holter monitoring was deemed necessary in 17 patients with severe arrhythmias, such as (supra)ventricular tachycardia runs or multiple and multiform VES. Three patients received medication and 1, a pacemaker implantation. Of these patients 6 had PVR. ⱍ Before After 16 2 (1) PVR ⫽ pulmonary valve replacement; RA ⫽ right and fatigue; indications for and outcome of repeated operative solution are depicted in Table 2. Most valves used were homografts, although two were Hancock heterografts. Good results were reported in 7 patients: an RV that returned to normal dimensions and disappearance of symptoms. A QRS complex exceeding 180 ms predicted serious problems in at least 2 of 3 patients. All important arrhythmias present before reoperation persisted after the procedure. Four patients had a reintervention for residual pulmonary stenosis first. Tricuspid insufficiency as indication for PVR was documented in patients with both good and less favorable outcome. Time and Type of Repair and Outcome Figure 3 demonstrates that RV dimensions in patients without or before PVR slowly increase over time. A Reinterventions A total of 32 patients (19%) underwent reoperation at a mean age of 8.7 ⫾ 5.6 years (range, 1.3 to 22.3 years; at 6.3 ⫾ 5.8 years after correction), of which 8 did it more than once (Fig 2). Follow-up time after first reintervention was 6.0 ⫾ 5.8 years. The majority of reinterventions, PVR excluded (10-year freedom, 83%), consisted of angioplasty for residual pulmonary stenosis (n ⫽ 12) and residual VSD closure (n ⫽ 6). Pulmonary Valve Replacement In 16 patients (9%) both physical and heart condition deteriorated to such an extent that PVR was considered (mean age after repair, 9.2 ⫾ 6.7 years). Predominant clinical symptoms include diminished exercise tolerance Fig 2. Freedom of reintervention or pulmonary valve replacement (PVR) as a function of years after operation. Ann Thorac Surg 2002;73:1794 – 800 Table 2. Details of Pulmonary Valve Replacements Indications for PVR Postrepair Clinical RV Tricuspid Pulmonary QRS ⬎ Patient Age (y) Condition PR Dilatation Insufficiency Stenosis Arrhythmias 180 ms Homograft 1 0.1 2 x x x x x x x 1.0 1.2 x x x 4 5 6 7 2.7 4.5 5.0 6.0 x x x x x x x x x x x x x x 8 6.5 x x x x x 9 10 11 9.0 11.9 14.0 x x x x x x x x x x x x 12 15.2 x x x x 13 14 15 16 15.8 16.6 17.5 21.0 x x x x 94% x x x x 75% x x x x x x x x 100% 100% DCRV ⫽ double chamber right ventricle; patch; VSD ⫽ ventricular septal defect. x x Dilatation pulmonary artery Abnormal lung vein, extra TAP, pacemaker x x x x x x x x x x x x x x x x x x PR ⫽ pulmonary regurgitation; x 38% 19% PS ⫽ pulmonary stenosis; DCRV Residual VSD Angioplasty pulmonary artery x 38% Angioplasty pulmonary artery Mitral valve correction Angioplasty pulmonary artery x x x x 88% PVR ⫽pulmonary valve replacement; Outcome 0.1 Death shortly postoperatively 2nd PVR 7 yr later, sudden cardiac death 12 y later 19.0 0.0 3.6 1.9 0.1 21.9 Residual RV dilatation 1.8 Residual PS Good Good Heart transplant 19 y postoperatively Good 9.8 2.8 2.8 Residual RV dilatation Residual PS Good 0.1 Good 3.6 3.0 3.0 0.6 Good Residual RV dilation Residual PS Good RV ⫽ right ventricular; TAP ⫽ transannular DE RUIJTER ET AL RIGHT VENTRICULAR DYSFUNCTION AND PULMONARY VALVE REPLACEMENT 3 Other Problems Follow-up After PVR (y) 1797 1798 DE RUIJTER ET AL RIGHT VENTRICULAR DYSFUNCTION AND PULMONARY VALVE REPLACEMENT Fig 3. Right ventricular (RV) dilatation index as a function of years after operation. similar plot could be drawn for pulmonary valve insufficiency. Statistical analysis showed that the increase was significant in both cases (p ⬍ 0.001). In 126 patients corrected with a transannular patch, 50 (39%) had developed severe RV dilatation at last followup; if the surgeon did not use this patch, this number decreased to 26% (n ⫽ 12). Statistically, the incidence of RV dilatation was thus considerably higher in patients who underwent correction with the use of a transannular outflow patch (p ⫽ 0.020). The group in which the VSD was closed by transatrial approach (n ⫽ 14) showed the opposite (p ⫽ 0.03): no patients with severe RV dilatation. Only 1 patient underwent reoperation for residual VSD, and 1 patient had PVR; moreover, this group presented with clearly lower QRS duration (p ⫽ 0.007; mean, 108.0 ms compared with 131.6 ms in right ventriculotomy group). Despite these effects, no difference could be found between survival of both transannular patching and transatrial VSD repair and their surgical opposites. Results of patients with early correction (ie, before 6 months of age) demonstrated two reoperations, of which one was PVR. Effects of early timing on RV dysfunction could not be established. Finally, the influence of residual hemodynamic lesions such as a remaining VSD, small RV outflow tract aneurysm, or pulmonary stenosis more than 30 mm Hg on RV dilatation could not be neglected (p ⫽ 0.04). Comment In conjunction with recent large studies, the prognosis of a newborn with tetralogy of Fallot is excellent [1– 4, 21]. Most patients were in New York Heart Association functional class I, and we found a 20-year actuarial survival of 91%. This is lower than the general population, as pointed out by Murphy and colleagues [1], but it is still a very reasonable outcome compared with other serious congenital heart defects. The group of patients with pulmonary atresia needs to be distinguished, as they Ann Thorac Surg 2002;73:1794 – 800 present with more problems in follow-up, confirming the results of Knott-Craig and associates [3]. We doubt whether changes toward earlier timing of the initial repair greatly affect the late outcome. Our data show that patients operated on after the age of 6 months have a comparable probability of needing reintervention or PVR, and there is no difference in RV dimensions. The fact that their follow-up time was significantly longer and most operations were performed in the early days of our center certainly militate for a time-dependent or surgically dependent effect. Evaluating transannular patching, this time factor was ruled out, and our results met those of previous observations [22], indicating that both severe PR and RV dilatation were more prevalent in those patients who received this outflow patch. The positive impact of the transatrial approach was confirmed, although this technique was not used extensively in our population; we have more recently changed to a more frequent implementation of this technique. The shorter duration of the QRS complex, did not result in fewer dysrhythmias in follow-up, in accordance with Oechslin and coworkers [19], pointing out that the site of ventricular arrhythmia is predominantly around the margins of the VSD patch and not the ventriculotomy per se, therefore putting every patient at equal risk. In regard to the severity of late RV outflow tract complications, our results did confirm those of Dietl and colleagues [24], who are in favor of the right atrial technique. Recommending preservation of the pulmonary valve or, if a transannular patch is necessary, restricting its width (minimizing regurgitant flow) and having a preference for the transatrial approach seems logical. Right ventricular dysfunction and pulmonary valve insufficiency slowly develop after repair of tetralogy of Fallot and become present in almost all patients to some degree. Similar to Meijboom and coworkers [21], Zakha and associates [9], Jonsson and colleagues [7], and Horneffer and associates [2], who also used echocardiography in followup, we found evidence of more than trivial PR in a considerable number of patients. Meijboom and colleagues [21], however, did not confirm the increase we found with time of both PR and RV dilatation. Exercise capacity is found to be decreased in patients with dilated RV [13, 21], but most patients remain asymptomatic. Although some argue that long-standing PR has no adverse effect on survival [1, 2, 21, 22, 25, 26], PVR will become a more frequent consideration as the population ages. Shimazaki and coworkers [27] reported that in patients without operation and with anatomically normal hearts, except for a congenitally incompetent pulmonary valve, symptoms of right heart failure will develop 30 to 40 years later in life. A considerable variation exists among patients in ability to tolerate PR and, as a result, development of RV dilatation. Especially additional lesions make the detrimental effects more difficult to endure: 5 of our patients already had one reoperation before undergoing PVR [8, 13, 22, 26]. Furthermore, Norgard and colleagues [28] and Gatzoulis and associates [29] demonstrated the protective effect (limited PR, less ventricular dilatation, and superior exercise performance) of a poorly compliant RV. Ann Thorac Surg 2002;73:1794 – 800 DE RUIJTER ET AL RIGHT VENTRICULAR DYSFUNCTION AND PULMONARY VALVE REPLACEMENT A high percentage of our population (9%) underwent PVR. In a few patients symptoms clearly diminished and the severity of regurgitation and dilatation improved, but unlike other reports [16, 18 –20], objective outcome was not as good as expected. Some can be explained by normal deterioration of the implanted valve (in this case, mostly homografts), as also seen in groups with pulmonary atresia. Discovering a high-risk individual in an earlier stage, and offering PVR before the onset of myocardial degeneration, is now becoming the ultimate quest. Answers to this search are to be found in more specific indications for the replacement. Some authors have emphasized the importance of coexisting tricuspid valve insufficiency [17, 26], resulting from progressive RV dilatation, and advocate earlier replacement because the onset of tricuspid regurgitation may represent a very late and possibly irreversible stage of RV failure [20]. Scar tissue is an important factor in the development of malignant ventricular arrhythmias. When this is combined with poor hemodynamics, a high-risk situation can evolve [30]. In contrast to Niezen and associates [11], Meijboom and colleagues [21] could not show a correlation between RV dilatation and arrhythmias (and sudden death). Neither could be confirmed in our study, but we agree with the findings of Gatzoulis and coworkers [31], that QRS prolongation (⬎ 180 ms) is their most sensitive predictor. The presence of significant arrhythmias by itself alone cannot be an indication for PVR. Perhaps a great factor in this problem is not the indication itself, but the evidence of its presence. Different patients have different ways of responding to hemodynamics after complete repair, and this should be accounted for in diagnostic methods. Doubts about a deteriorated RV function by echocardiographic data should best be confirmed by magnetic resonance imaging mapping studies to allow optimal timing in the decision-making process tailored to the individual patient [11, 32]. Limitations In the 23 years of cardiac surgery for tetralogy of Fallot in our center, techniques and timing of the operation have changed and the experience of the surgeons has increased, suggesting a more favorable outcome in the youngest patients. During follow-up, electrocardiograph and echocardiography devices were modernized, presenting clearer pictures and allowing detection of problems earlier. Moreover, observations were performed by different cardiologists. Comparison of results may also be hazardous, because no standardized method is used in literature to quantify either PR or RV dilatation. Conclusions Severe RV dilatation and PR secondary to outflow tract repair in tetralogy of Fallot are frequently occurring sequelae in respectively 38% and 32% of our population, developing slowly with time. Indications for PVR remain controversial because warning echocardiographic findings or arrhythmias are not always accompanied by 1799 deterioration of clinical condition. However, right atrial dilatation and additional hemodynamic lesions demand increased vigilance. Transatrial repair is associated with a favorable outcome. References 1. Murphy JG, Gersh BJ, Mair DD, et al. Long-term outcome in patients undergoing repair of tetralogy of Fallot. N Engl J Med 1993;329:595–9. 2. Horneffer PJ, Zakha KG, Rowe SA, et al. Long-term results of total repair of tetralogy of Fallot in childhood. Ann Thorac Surg 1990;50:179– 85. 3. Knott-Craig CJ, Elkins RC, Lane MM, Holz J, McCue C, Ward KE. A 26-year experience with surgical management of tetralogy of Fallot: risk analysis for mortality or late reintervention. Ann Thorac Surg 1998;66:506–11. 4. Zhao HX, Miller DC, Reitz BA, Shumway NE. Surgical repair of tetralogy of Fallot. Long-term follow-up with particular emphasis on late death and reoperation. J Thorac Cardiovasc Surg 1985;89:204–20. 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. Joransen JA, Lucas RV Jr, Moller JH. Postoperative haemodynamics in tetralogy of Fallot. A study of 132 children. Br Heart J 1987;41:33–9. 7. Jonsson H, Ivert T, Brodin LA. Echocardiographic findings in 83 patients 13–26 years after intracardiac repair of tetralogy of Fallot. Eur Heart J 1995;16:1255– 63. 8. Ilbawi MN, Idriss FS, DeLeon SY, et al. Factors that exaggerate the deleterious effects of pulmonary insufficiency on the right ventricle after tetralogy repair. J Thorac Cardiovasc Surg 1987;93:36– 44. 9. Zakha KG, Horneffer PJ, Rowe SA, et al. Long-term valvular function after total repair of tetralogy of Fallot. Circulation 1988;78(Suppl 3):III-14 –9. 10. Bove EL, Byrum CJ, Thomas FD, et al. The influence of pulmonary insufficiency on ventricular function following repair of tetralogy of Fallot: evaluation using radionuclide ventriculography. J Thorac Cardiovasc Surg 1983;85:691– 6. 11. Niezen RA, Helbing WA, van der Wall EE, et al. Biventricular systolic function and mass studied with MR imaging in children with pulmonary regurgitation after repair of tetralogy of Fallot. Radiology 1996;201:135– 40. 12. Carvalho JS, Shinebourne EA, Busst C, Rigby ML, Redington AN. Exercise capacity after complete repair of tetralogy of Fallot: deleterious effects of pulmonary regurgitation. Br Heart J 1992;67:470–3. 13. Jonsson H, Ivert T, Jonasson R, Holmgren A, Bjork VO. Work capacity and central hemodynamics 13 to 26 years after repair of tetralogy of Fallot. J Thorac Cardiovasc Surg 1995; 110:416–26. 14. Lange PE, Onnasch DGW, Bernard A, Heintzen PH. Left and right ventricular adaptation to right ventricular overload before and after surgical repair of tetralogy of Fallot. Am J Cardiol 1982;50:786–94. 15. Kavey REW, Thomas FD, Byrum CJ, Blackman MS, Sondheimer HM, Bove EL. Ventricular arrhythmias and biventricular dysfunction after repair of tetralogy of Fallot. J Am Coll Cardiol 1984;4:126–31. 16. Yemets IM, Williams WG, Webb DG, et al. Pulmonary valve replacement after late repair of tetralogy of Fallot. Ann Thorac Surg 1997;64:526–30. 17. Misbach GA, Turley K, Ebert PA. Pulmonary valve replacement for regurgitation after repair of tetralogy of Fallot. Ann Thorac Surg 1983;36:684–91. 18. Bove EL, Kavey REW, Byrum CJ, Sondheimer HM, Blackman MS, Thomas FD. Improved right ventricular function following late pulmonary valve replacement for residual 1800 19. 20. 21. 22. 23. 24. 25. DE RUIJTER ET AL RIGHT VENTRICULAR DYSFUNCTION AND PULMONARY VALVE REPLACEMENT pulmonary insufficiency or stenosis. J Thorac Cardiovasc Surg 1985;90:50–5. Oechslin EN, Harrison DA, Harris L, et al. Reoperation in adults with repair of tetralogy of Fallot: indications and outcomes. J Thorac Cardiovasc Surg 1999;118:245–51. Warner KG, Anderson JE, Fulton DR, Payne DD, Geggel RL, Marx GR. Restoration of the pulmonary valve after right ventricle volume overload after previous repair of tetralogy of Fallot. Circulation 1993;88:189–97. Meijboom F, Szatmari A, Deckers JW, et al. Cardiac status and health related quality of life in the long term after surgical repair of tetralogy of Fallot in infancy and childhood. J Thorac Cardiovasc Surg 1995;110:883–91. Kirklin JK, Kirklin JW, Blackstone EH, Milano A, Pacifico AD. Effect of transannular patching on outcome after repair of tetralogy of Fallot. Ann Thorac Surg 1989;48:783–91. Hedeker D, Gibbons R. Mixor version 2: a computer program for mixed-effects ordinal regression analysis. Division of Epidemiology and Biostatistics, School of Public Health, University of Illinois, 1999. Dietl CA, Cazzaniga ME, Dubner SJ, Perez-Balino NA, Torres AR, Favaloro RG. Life threatening arrhythmias and RV dysfunction after surgical repair of tetralogy of Fallot. Circulation 1994;90:7–12. Wessel HU, Cunningham WJ, Paul MH, Bastanier CK, Muster AJ, Idriss FS. Exercise performance in tetralogy of 26. 27. 28. 29. 30. 31. 32. Ann Thorac Surg 2002;73:1794 – 800 Fallot after intracardiac repair. J Thorac Cardiovasc Surg 1980;80:582–93. Finck SJ, Puga FJ, Danielson GK. Pulmonary valve insertion during reoperation for tetralogy of Fallot. Ann Thorac Surg 1988;45:610–3. Shimazaki Y, Blackstone EH, Kirklin JW. The natural history of isolated congenital pulmonary valve incompetence: surgical implications. Thorac Cardiovasc Surg 1984;32:257–9. Norgard G, Gatzoulis MA, Moraes F, et al. Relationship between type of outflow tract repair and postoperative right ventricular diastolic physiology in tetralogy of Fallot. Implications for long-term outcome. Circulation 1996;94:3276– 80. Gatzoulis MA, Clark AL, Cullen S, et al. Right ventricular diastolic function 15 to 35 years after repair of tetralogy of Fallot. Circulation;91:1775– 81. Friedli B. Electrophysiological follow-up of tetralogy of Fallot. Pediatr Cardiol 1999;20:326–30. Gatzoulis MA, Till JA, Somerville J, Redington AN. Mechanoelectrical interaction in tetralogy of Fallot: QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death. Circulation 1995; 92:231–7. Rebergen SA, Chin JGJ, Ottenkamp J, van der Wall EE, de Roos A. Pulmonary regurgitation in the late postoperative follow-up of tetralogy of Fallot: volumetric quantitation by NMR velocity mapping. Circulation 1993;88:2257– 66. DISCUSSION DR DARRYL S. WEIMAN (Memphis, TN): Doctor Kanter seems to think that the porcine valve replacement is superior to the homograft. Did you have a chance to look at your data in that regard? DR BENNINK: It is very interesting. In Holland, research was done by the Leiden group in which they put in the growing pig a biologic valve, mainly the Freestyle Medtronic valve, and because of the disastrous results, we did not dare to use that valve in humans. Now that I have seen this result of Dr Kanter, I can see that the pig is a little bit different from the human being. I knew that before, but it is not always wise to extrapolate all the results you get from animal experiments. But mainly it is homograft insertion that we do, and the Groningen group in Holland even implants mechanical valves in the pulmonary position. DR CONSTANTINE MAVROUDIS (Chicago, IL): Some of the very important work on postoperative arrhythmias in these patients has been done by Michael Gatzoulis in Great Britain. He showed that atrial reentry tachycardia is more common than ventricular tachycardia in these patients. You did note in one of your slides that you used arrhythmias as part of your indications for operation. But did you do anything about these arrhythmias at the time of operation? In other words, did you perform a maze procedure in any of these patients? It would be interesting to hear what your thoughts are on this subject. DR BENNINK: Thank you for your comments, Dr Mavroudis. We have not done anything with it yet, but, as you probably know, one of your former fellows is now working in our institute and we are planning to come and visit you in Chicago in January to see exactly how to do those procedures, so we are really considering it. One of our cardiologists is interested in electrophysiology. I think it is a wise thing to do, especially if the procedure is not too much prolonged. Then I think it will contribute to the outcome of the patients. Up until now we did not perform any arrhythmia surgical procedures.