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Ann Thorac Surg 1997;64:526-530
© 1997 The Society of Thoracic Surgeons
The Hospital for Sick Children and the Toronto Congenital Cardiac Centre for Adults, Toronto, Ontario, Canada
Accepted for publication March 3, 1997.
| Abstract |
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Methods. All patients who underwent pulmonary valve replacement in our center late after repair of tetralogy of Fallot were reviewed.
Results. Eighty-five patients had elective pulmonary valve replacement late (median, 9.3 years) after repair. Operative risk was low (1.1%). Ninety percent of survivors are in New York Heart Association class I. Survival 10 years after pulmonary valve replacement is 95%, with 86% of the patients free of reoperation for valve failure.
Conclusions. Pulmonary valve replacement is infrequently required after repair of tetralogy of Fallot. Pulmonary valve replacement may be performed electively with little risk; it improves symptoms of right heart failure and provides satisfactory long-term survival with low risk of early valve failure. As the population of patients who have had repair of tetralogy of Fallot ages, pulmonary valve replacement will become a more frequent consideration.
| Introduction |
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Pulmonary insufficiency and right ventricular dysfunction, which are universally present to some degree after tetralogy repair, may be poorly tolerated if there are additional residual lesions [69]. Indeed, patients with isolated pulmonary valve insufficiency in hearts that are otherwise anatomically normal are usually asymptomatic until age 30 to 40 years [10]. In patients with tetralogy, Redington's group [11, 12] has demonstrated that reduced right ventricular compliance may protect against the detrimental effects of pulmonary valve incompetence late after repair. Several recent reports [5, 11, 13, 14] correlate pulmonary insufficiency with right ventricular dilatation and reduced capacity for exercise. Increasing evidence [1519] suggests that pulmonary valve insufficiency is associated with the development of arrhythmias and possibly with sudden death.
We report the outcome of patients after tetralogy repair who required pulmonary valve replacement (PVR) at The Hospital for Sick Children, Toronto, and the Toronto Congenital Cardiac Centre for Adults.
| Material and Methods |
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We reviewed operative records of prior palliative procedures, initial repair, and PVR, including clinical status before PVR, the indications for PVR, and subsequent clinical course. Follow-up data (physical examination, electrocardiography, chest roentgenography, echocardiography, and sometimes [n = 11] cardiac catheterization) were available from our own outpatient clinics or from tertiary referral centers for all but 1 patient.
Thirty-one (36%) of the patients are female; 54 are male. Mean age at the time of repair of TOF was 8 years (range, 0.5 to 40 years; median, 5.6 years). Forty-nine patients (58%) had a palliative operation preceding their repair, most commonly a Blalock-Taussig shunt (n = 40). At the initial repair, right ventricular outflow tract obstruction was treated by transannular patch in 56 (66%) of the patients; the pulmonary valve annulus was preserved in 29 (34%). Anticoagulation was not recommended after PVR, but patients were encouraged to take low-dose long-term acetylsalicylic acid.
Statistical Analysts
Survival data were analyzed using the Kaplan-Meier method (Statistica, Version 5; Softstat Inc, 199496).
Indication for Pulmonary Valve Replacement
Most patients have pulmonary insufficiency after TOF repair and tolerate it for many years. However, in a previously stable patient, the onset of symptoms, usually fatigue and dyspnea, and the development of arrhythmias, either ventricular tachycardia or supraventricular tachycardia, or the onset of tricuspid insufficiency are clinical indications for consideration of PVR.
A total of 85 patients had PVR at a time remote from their initial repair. Their age at initial repair is shown in Figure 1
. At the time of their PVR, their mean age was 19.6 years (range, 2 to 54 years; median, 15.4 years) (Fig 2
). Mean time from repair to PVR is 11.6 years (range, 0.4 to 36 years; median, 9.3 years) (Fig 3
).
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In 29 patients, PVR was an isolated procedure. In the other 56 patients (66%), additional procedures were required: closure of the residual ventricular septal defect (n = 13), cryoablation of arrhythmia (n = 13), branch pulmonary arterioplasty (n = 30), and miscellaneous procedures in 25 (aortic valve replacement, 2; mitral valve repair, 3; tricuspid valve repair, 6; atrial septal defect, 9; and others, 5). In addition, most patients had dilatation of the right ventricular outflow tract incision or patch, and this was plicated during the PVR operation.
Methods
We used two types of devices to replace the pulmonary valve. Six patients had a valved conduit (porcine/Dacron in 3, pulmonary homograft in 2, Polystan in 1), and in 79, we sewed a prosthetic valve into the pulmonary annulus and covered it with a pericardial patch. The pericardial patch extended from the pulmonary artery bifurcation onto the right ventricular infundibulum; we referred to this technique as a pulmonary valve implant. Among the patients with a pulmonary valve implant, 40 had a porcine valve, 36 a pericardial valve, and 3 some other type.
We performed all PVR operations through a median sternotomy with cardiopulmonary bypass and hypothermia (32° to 35°C). Aortic cross-clamping was used only when required to facilitate the intracardiac repair of associated lesions. Femoral artery/vein cannulation was used to initiate bypass before sternotomy, if required to prevent or control bleeding.
| Results |
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The postoperative course in the other patients was generally uneventful. No complications related to the PVR were observed in the early postoperative period. Anticoagulants were not prescribed. Low-dose acetylsalicylic acid, three times per week, was recommended.
The surviving patients have been followed up from 1 month to 26 years postoperatively (mean follow-up, 5.8 years). There have been three late deaths. The actuarial survival is 95% ± 3% at 10 years and 87% ± 8% at 15 years (Fig 4
).
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Preoperative VT was observed in 19 patients, but at intraoperative electrophysiologic mapping, it was inducible and monomorphic in only 13, all of whom underwent arrhythmia ablation during the PVR. Of the others, the arrhythmia could not be induced at operation in 1 patient, the arrhythmia was localized to the left ventricular endocardium and ablation was not attempted in another, and VT was multifocal in the remaining 4 patients. Survival of the 19 patients presenting with VT did not differ statistically from that of the 66 patients without documented preoperative VT.
The time from initial repair to PVR is a mean of 13.3 years for patients with no associated lesions versus 10.7 years for those with other lesions, a difference that is not statistically significant. Similarly, the age at PVR was not different among patients with isolated pulmonary insufficiency compared with those with associated lesions.
Symptoms improved after PVR in most patients. The functional status of all surviving patients at the time of late follow-up is compared with their status before PVR in Figure 5
. However, as the prosthetic valve fails, one expects their functional status will deteriorate.
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During the follow-up period of observation, 8 patients have required replacement of their prosthetic pulmonary valve, a mean of 8.9 years (range, 3.4 to 16.9 years) after PVR. By actuarial analysis, 86% ± 7% of patients are free of reoperation 10 years after valve replacement (Fig 6
). The analysis of survival free of reoperation for porcine (n = 40) versus pericardial pulmonary valve implants (n = 36) reveals no statistically significant difference, but the trend suggests that with longer follow-up the porcine valve will prove to be more durable (Fig 7
).
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| Comment |
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Indeed, in the rare situation of patients with anatomically normal hearts with absent pulmonary valve, Shimazaki and coworkers [10] reported that symptoms were rare before the age of 30 years. However, beyond age 30 to 40 years, progressively more patients had increasing fatigue and dyspnea and the onset of right heart failure.
It seems probable that tetralogy patients with pulmonary valve insufficiency will be become symptomatic at a younger age than patients with otherwise anatomically normal hearts. As a corollary, one should expect an increasing number of patients after tetralogy repair to present with progressive right heart failure caused by pulmonary valve insufficiency as they approach middle ageor younger, if they have additional lesions, although the time from repair to pulmonary valve replacement was not significantly different in the subset of patients with additional lesions.
It is difficult to estimate the total number of patients with tetralogy repair from which these 85 patients were selected for PVR. Approximately 50 patients per year undergo tetralogy repair at our center. Some PVR patients were referred from other centers. As a best estimate, 1.2% of our institutions' patients with repair of tetralogy have undergone PVR. Because the need for PVR after tetralogy repair is time-dependent, with progressively more patients considered for the procedure years after repair, the estimated average of 1.2% greatly understates the long-term risk. Changing techniques in tetralogy repair and the decreasing age at repair should have a favorable influence on both the need and timing for PVR, but these effects are not evident as yet.
There is a known risk of late sudden death due to ventricular arrhythmias after tetralogy repair [1519]. The association of ventricular arrhythmias with suboptimal hemodynamics in patients with tetralogy has been addressed by our group [16]. We hypothesize that improving right heart function by implanting a competent pulmonary valve may decrease the chance of fatal arrhythmias. We have only 19 patients in this series with VT. One late, presumed arrhythmic sudden death occurred in a patient who presented with multifocal ventricular tachycardia. Because of its multifocal origin, ablation of the arrhythmia was not attempted in this patient during PVR. We previously reported our experience with ablation therapy after tetralogy repair, but the data are not yet sufficient to support firm conclusions [20]. The indications for choosing among ablation, medication, implantable defibrillator therapy, or simply improving the hemodynamic status with PVR remain to be clarified. In this series, there is no difference in survival for patients presenting with VT (n = 19) compared with those without (n = 66), and overall, survival is 95% at 10 years after PVR.
We do not have data to demonstrate the hemodynamic improvement that occurs with pulmonary valve replacement. Many authors have published objective evidence of improved cardiac performance after PVR [14, 21, 22]. Among our patients, the subjective improvement reported was considerable (see Fig 5
), suggesting a favorable hemodynamic response to PVR. Exercise capacity will diminish, however, as the prosthetic valve deteriorates. We are currently collecting cardiopulmonary exercise data prospectively to clarify the changing functional abilities in these patients.
Pulmonary valve replacement late after tetralogy repair can be performed at low operative risk (1.1%) with good intermediate-term survival (95% at 10 years after replacement) and with reasonably good long-term durability of the valve (86% free of reoperation at 10 years). We have previously reported that multivariate analysis demonstrates that both older age at PVR and use of a larger valve improve valve longevity but not valve type. We anticipate that a porcine prosthesis will be the most durable valve, but to date there is no statistically important difference compared with the pericardial valve (see Fig 7
).
Intracardiac repair of patients with tetralogy of Fallot should be carefully done to ensure a minimum of residual hemodynamic lesions. Pulmonary valve replacement is rarely necessary. When right heart failure does develop after tetralogy repair, our data show that PVR and repair of associated lesions can be undertaken safely, with good long-term improvement, albeit at the inevitable cost of future operations to replace the prosthetic valve.
| Footnotes |
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| References |
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