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Eur J Cardiothorac Surg 2001;19:667-670
© 2001 Elsevier Science NL

Pulmonary valve insertion late after repair of Fallot's tetralogy

Department of Cardiothoracic Surgery, Leiden University Medical Centre, Leiden, The Netherlands

Received 10 October 2000; received in revised form 1 February 2001; accepted 28 February 2001.

Corresponding author. Afdeling Cardiothoracale Chirurgie D 6-S, LUMC, P.O. Box 9600, 2300 RC Leiden, The Netherlands. Tel.: +31-71-5262-355; fax: +31-71-5248-284
e-mail: m.g.hazekamp{at}lumc.nl

	Abstract

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion References

 
Objectives: To analyze the results of pulmonary valve insertion late after initial repair of Fallot's tetralogy. Pulmonary insufficiency (PI) after correction of Fallot's tetralogy is usually well tolerated in the short term, but is associated with symptomatic right ventricular dilatation and an increased risk of ventricular arrhythmias over longer periods of time. Methods: From 1993 to July 2000, 51 patients were reoperated for PI at a mean age of 25.7±11.9 years. The mean age at initial repair was 6.4±7.2 years. Patients with a conduit inserted at initial operation, with absent pulmonary valve syndrome or with a more than moderate ventricular septal defect at reoperation were excluded from the study. A cryopreserved pulmonary (96%) or aortic (4%) homograft was implanted in the orthotopic position with the use of cardiopulmonary bypass 19.3±9.1 years (2.7–40.3 years) after initial correction. Preoperative symptoms (New York Heart Association, NYHA class), degree of PI (echo-Doppler, MRI), right ventricular dimensions (MRI) and QRS duration were compared to findings at last follow-up. Results: Follow-up is complete and had a mean duration of 1.7±1.4 years. Hospital mortality was 2%. No serious morbidity occurred. Severe PI was present preoperatively in all patients. At last follow-up echo-Doppler studies showed PI to be absent or trivial in 96% and mild in 4% of patients. In 13 patients MRI studies were performed both pre- and postoperatively: in this group PI was reduced from a mean of 48 to 4%. After 6 months NYHA capacity class had improved significantly from 2.3±0.6 to 1.4±0.5. After 1 year end-diastolic and end-systolic right ventricular volumes were reduced significantly. Right ventricular ejection fraction and QRS duration remained unchanged. Conclusions: PI late after correction of Fallot's tetralogy may lead to serious symptomatic right ventricle dilatation. After pulmonary homograft insertion right ventricular dimensions decrease rapidly and functional improvement is observed in almost all patients.

Key Words: Tetralogy of Fallot • Congenital heart disease • Pulmonary valve insufficiency • Cardiac surgery • Homograft • Reoperation

	1. Introduction

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion References

 
Correction of tetralogy of Fallot may lead to postoperative pulmonary insufficiency (PI), especially if a transannular patch has been inserted into the right ventricular outflow tract (RVOT). Postoperative PI is generally well tolerated for a long time [1]. However, finally an increase in right ventricular dimensions leads to a diminished exercise capacity, arrhythmias and an increased risk of sudden death [2].

Pulmonary valve replacement (PVR) has been reported to normalize right ventricular dimensions and results generally in remarkable clinical improvement [3–6]. Optimal timing of surgery, however, is uncertain.

As the population of adult patients with an earlier surgical correction of Fallot's tetralogy is growing we will be more and more confronted with an increasing number of patients with PI and right ventricular failure.

We report our experience with PVR following repair of Fallot's tetralogy to investigate the effect of PVR on the degree of PI, right ventricular dimensions and validity class. A subgroup of 13 adult patients had pre- and postoperative MRI studies to determine exactly the effect of PVR on right ventricular and regurgitating volumes.

	2. Materials and methods

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion References

 
From 1993 to July 2000, 51 patients had PVR for PI following repair of Fallot's tetralogy. Twenty-nine (57%) were male. Patients with pulmonary atresia were excluded from this study as well as patients with absent pulmonary valve syndrome, a conduit inserted at first operation, a more than moderate ventricular septal defect (VSD) or a presentation with predominantly RVOT obstruction (gradient of more than 50 mmHg). Three patients with agenesia or post-surgery occlusion of one of the pulmonary artery branches (with left-sided pneumonectomy in one) were included in the study.

During initial surgery for tetralogy of Fallot a transannular patch had been used in 41 (80%) patients. In others combinations of pulmonary valvotomy, RVOT patches and resection of infundibular muscle had been performed. Eight patients were reoperated after initial Fallot repair but before later PVR. Indications for reoperation were persistent Pott's shunt with narrowing of the left pulmonary artery, residual VSD, residual RVOT obstruction, or discrete subaortic stenosis. In three patients percutaneous balloon angioplasty and stenting of branch pulmonary artery stenosis had been performed. Two patients had received an implantable cardioverter device.

The initial repair was at the mean age of 6.4±7.2 years. PVR was performed at a mean age of 25.7±11.9 years. Twelve patients were under 15 years of age at the time of PVR. The interval between initial operation and later PVR had a mean value of 19.3±9.1 years (Fig. 1). The largest interval was 40.3 years in a patient who had Fallot repair in 1959. The shortest interval was 2.7 years in a girl who had initial correction with a transannular patch at the age of 4 months.

View larger version (45K): [in this window] [in a new window]   Fig. 1. Interval in years between initial correction of tetralogy of Fallot and later pulmonary valve insertion.

Symptomatic ventricular arrhythmias were present prior to PVR in five (10%) patients. Two of them had a cardioverter device implanted. Complete right bundle branch block was present in 42 (82%) patients. The mean length of the QRS complex was preoperatively 150±32 ms. Thirty-one (61%) patients were without medication, eight (16%) patients used diuretics, nine (18%) patients used anti-arrhythmic medication and three (5%) patients used both anti-arrhythmic medication and diuretics.

All patients had preoperative echo-Doppler studies. Preoperative MRI was done in 34 (67%) patients. CT scans were performed in other patients (e.g. pacemakers or cardioverter devices excluded the use of MRI). PI was found to be moderate to severe or severe in all patients. Right ventricular dimensions were markedly increased in all cases. Tricuspid valve regurgitation that was considered to need surgical intervention at the time of PVR was present in ten (20%) patients.

All patients were operated with normothermic or moderately hypothermic cardiopulmonary bypass. Cannulation of femoral vessels was performed if considered necessary. The majority of pulmonary valve insertions (80%) were performed on beating hearts. Aortic cross-clamping was dependent on the surgeon's preference or on concomitant procedures. Small residual VSDs were suture closed in eight patients, while de Vega tricuspid annuloplasty was performed in ten patients. Other procedures were reduction of a dilated ascending aorta in two patients, resection of subaortic stenosis in two patients, atrial septal defect closure in one patient and closure of a coronary artery fistula in another patient. Cryopreserved pulmonary homografts were used in 49 patients and aortic homografts were used in two patients. The mean homograft diameter was 25±1.8 mm. Homografts were always inserted in the orthotopic pulmonary position with one proximal and one distal end-to-end running suture after longitudinally opening the proximal pulmonary artery and slightly extending this incision if necessary across the former pulmonary annulus. Calcified outflow tract patch material was resected as much as possible.

For completion of follow-up all patients' hospital charts were reviewed and all patients were submitted to a question list by telephone. NYHA capacity class was defined by clinical examination and answering of standardized question forms. QRS duration was measured on ECG at last follow-up.

Data are presented as mean values with standard deviations unless otherwise stated. Variables were evaluated by using Student's t-test.

	3. Results

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion References

 
Hospital mortality occurred in one patient with congenital agenesia of the right pulmonary artery who was operated for severe right ventricular failure 23 years after Fallot repair. One other patient died suddenly at home 18 months following uncomplicated PVR. Fallot repair had been performed 26 years previously.

Resternotomy for bleeding was necessary in three patients. Other complications were not observed.

Functional capacity significantly improved from a mean NYHA capacity class of 2.3±0.6 to 1.4±0.5 at last follow-up (P<0.001) (Table 1). Diuretic medication could be discontinued in all patients after PVR, while anti-arrhythmic medication was continued as preoperatively.

Postoperative echo-Doppler studies showed PI to be absent or trivial in 96% of the patients. In two (4%) patients PI was considered to be mild to moderate. In one of these patients re-PVR was considered. Tricuspid insufficiency was absent or trivial in all patients that underwent a tricuspid annuloplasty at the time of PVR. End-diastolic right ventricular dimensions were already decreased shortly after surgery.

Evaluation of pre- and postoperative MRI scans in a subset of 13 patients (mean age 28.9±9.2 years) showed PI to be significantly reduced from a mean of 48% (range 25–66%) to 4% postoperatively (range 0–26%) (P<0.001) (Table 1). RVEDV was significantly reduced from a mean value of 292±69 to 214±56 ml 1 year after PVR, which indicated a 26% reduction (P<0.001). RVESV decreased from 175±47 to 133±33 ml 1 year after PVR (P<0.001). RVEF changed from a preoperative value of 37±8 to 44±13% 1 year after PVR; however, this change was not significant (Table 2).

In the patients with ventricular arrhythmias prior to PVR no decrease in the frequency of these arrhythmias was observed after PVR. One year after PVR the length of the QRS duration had not changed significantly (150±32 ms preoperatively and 148±31 ms postoperatively) (Table 1).

	4. Discussion

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion References

 
Although PI after correction of tetralogy is mostly the result of transannular patch augmentation of the RVOT, valve insufficiency may also be observed following valvotomy and other procedures to relieve pulmonary stenosis. As the patients in this series were referred from several hospitals it was not possible to determine the percentage of patients with initial transannular patching that underwent later PVR. However, 80% of the patients in this series had transannular patching at initial repair. If a transannular augmentation of the RVOT is deemed necessary, the transannular patch should not be wide and the incision into the right ventricular muscle should be as limited as possible. Patch material should be stiff to avoid later dilatation. We could not find a relation between age at initial operation and need of later PVR.

PI is mostly well tolerated for longer periods of time but finally progressive right ventricular dilatation develops [1]. When PI is accompanied by residual VSD and/or RVOT obstruction right ventricular failure may present earlier [7]. Functional capacity decreases as the patient suffers from diminished exercise tolerance. Right ventricular dilatation is associated with an increased risk of ventricular arrhythmias and sudden death [2]. Several authors have reported beneficial effects of the restoration of pulmonary valve function [3–6]. In this series we also observed a remarkable improvement in functional capacity following PVR.

Pulmonary homografts are generally used for this purpose although bioprosthetic conduits and mechanical valve prostheses have been reported as alternatives [8].

We have used homografts as they form excellent pulmonary valve substitutes with very low gradients. Pulmonary homografts are preferred; aortic homografts were used in two patients when a pulmonary valve was not available. As the patients are usually young and in females a pregnancy wish may be present, life-long anticoagulation is avoided by using homograft valves. Nevertheless, homografts may suffer from calcific degeneration and need later replacement. Pulmonary homografts are reported to last longer than aortic homografts in the pulmonary position [9]. As at PVR adult-sized pulmonary homografts are inserted in the orthotopic position they may perform better than homografts that are used as extracardiac conduits [10]. Possibly, the durability of cryopreserved pulmonary homografts used for late PVR following Fallot repair is similar to that of pulmonary homografts which are orthotopically used to reconstruct the RVOT in the Ross procedure.

In the International Ross Registry report of 2610 documented Ross operations freedom from reoperation on the RVOT was 91% at 10 years and 84% at 25 years [11].

Optimal timing of PVR is still the subject of debate as the chance of right ventricular normalization must be weighed against a risk of later reoperation for homograft failure. As homograft failure has been reported to be more accelerated in children, PVR after initial Fallot surgery will not be undertaken easily in the pediatric age group [9]. In this study 12 patients had PVR at an age of less than 15 years because of evident functional impairment. Two of these patients did not have transannular patching suggesting that other factors such as right ventricular muscle resection may play a role in the development of right ventricular failure.

In this series the indications to perform PVR were the following: symptomatic deterioration (fatigue and dyspnea at exercise), increase in right ventricular dimensions as measured with echo or MRI studies and/or progression of ventricular arrhythmias. Our policy has been to accept patients for PVR rather early. However, as the patients were referred to us by different hospitals, this series is not homogeneous with concern to indications for surgery. Right ventricular function may remain compromised if PVR is performed too late. Although no studies exist that report a significant reversal of ventricular arrhythmias after PVR, earlier PVR should be considered. If more than mild tricuspid incompetence has developed because of right ventricular dilatation, PVR should probably have been performed at an earlier stage. A scoring system may be of value to define the optimal moment for PVR. Several factors should be included such as functional capacity class, right ventricular dimensions, degree of PI, degree and nature of arrhythmias, medication if any, QRS duration, residual VSD or RVOT obstruction and tricuspid insufficiency.

PVR is generally safe and uncomplicated as has been reported by others [3–6]. One patient with congenital agenesia of the right pulmonary artery died following PVR for severe right ventricular failure 23 years after Fallot repair. Progressive unexplained hypoxia developed shortly after operation and led to low cardiac output. One other patient died suddenly 18 months after uncomplicated PVR. During follow-up no abnormalities had been observed.

Although three patients had resternotomy for bleeding following PVR no other serious complications occurred. The mean hospital stay was 8.7±4.2 days.

Right ventricular volumes decreased significantly following PVR as was demonstrated by MRI pre- and postoperatively in a selected group of patients. PI was demonstrated both by echo-Doppler studies and by MRI to decrease in this series.

In our opinion phase-contrast MRI is valuable in the quantification of regurgitating volume. Echo-Doppler is less accurate and radionuclide studies do not provide additional anatomical information. By using serial MRI studies in the follow-up of Fallot patients accurate quantitative data can be obtained and the progression of PI and the deterioration of right ventricular function can be noted in an early phase [12,13]. Further MRI studies can be helpful in developing a scoring system that optimizes the timing of PVR late after correction of Fallot's tetralogy.

	Footnotes

 
Presented at the 14th Annual Meeting of the European Association for Cardio-thoracic Surgery, Frankfurt, October 7–11, 2000.

	References

Top Abstract 1. Introduction 2. Materials and methods 3. Results 4. Discussion References

 

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