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Ann Thorac Surg 2009;87:1879-1884. doi:10.1016/j.athoracsur.2009.02.068
© 2009 The Society of Thoracic Surgeons
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Original Articles: Pediatric Cardiac

Secondary Repair of Incompetent Pulmonary Valves

Nestoras Papadopoulos, MDa,*, Anoosh Esmaeili, MDb, Andreas Zierer, MDa, Farhad Bakhtiary, MD, PhDa, Feyzan Özaslan, MDa, Anton Moritz, MD, PhDa

a Department of Thoracic and Cardiovascular Surgery, Johann Wolfgang Goethe University, Frankfurt, Germany
b Department of Pediatric Cardiology, Johann Wolfgang Goethe University, Frankfurt, Germany

Accepted for publication February 24, 2009.

* Address correspondence to Dr Papadopoulos, Department of Thoracic and Cardiovascular Surgery, Johann Wolfgang Goethe University, Theodor Stern Kai 7, Frankfurt am Main, 60590, Germany (Email: nestoras.papadopoulos{at}gmail.com).


   Abstract
Top
 Abstract
Introduction
 Material and Methods
 Results
 Comment
 References
 
Background: Secondary repair of the pulmonary valve after right ventricular outflow tract (RVOT) reconstruction is infrequently reported. This article describes possible techniques of secondary pulmonary valve repair and reports follow-up results.

Methods: Secondary pulmonary valve repairs in 7 patients (5 children and 2 adults) in our institution were reviewed. All patients presented with a severe pulmonary valve regurgitation associated with RV dilatation and dysfunction after primary RVOT reconstruction.

Results: The surgical techniques varied in our series, but secondary repair of the incompetent pulmonary valve was possible in all patients. Follow-up was complete, with a mean follow-up of 4.1 ± 2.7 years. There were no operative or late deaths in our group. All valves were repaired successfully, with a mean regurgitation grade of 1.28 ± 0.5 postoperatively. The mean transvalvular gradient was 20 ± 4.1 mm Hg for children and 22.5 ± 3.5 mm Hg for adults, and no significant increase of pulmonary valve regurgitation occurred during follow-up. The mean RV dilatation index (RVDI) decreased significantly from 0.85 ± 0.25 to 0.6 ± 0.2 for children and from 1.4 ± 0.01 to 0.9 ± 0.05 for adults.

Conclusions: Our results showed functional recovery of the right ventricle after reoperation, with RVDI recovering to almost normal values in children. No significant regurgitation of the secondarily reconstructed pulmonary valve was observed during the 4-year follow-up period. Secondary repair for pulmonary valve incompetence after RVOT procedures might be a valuable alternative to conduit replacement.


   Introduction
Top
 Abstract
 Introduction
Material and Methods
 Results
 Comment
 References
 
Pulmonary valve incompetence is a frequent midterm or long-term complication after repair of pulmonary valve stenosis, mainly after transannular patch insertion. Residual lesions or secondary changes can lead to severe pulmonary regurgitation (PR) and consecutively to right ventricular (RV) dilatation, RV failure, and arrhythmias [1–5]. The reported incidence of severe PR after transannular patch repair reaches about 30% at 22 years of follow-up [6]. The frequency is expected to grow with increasing follow-up owing to the excellent long-term survival after repair of complicated pulmonary valve stenosis and to an increase of transannular patching of up to 90% in recent series [7–10]. Surgical treatment for patients with severe secondary pulmonary valve incompetence is generally pulmonary valve replacement with homografts or xenografts [11]. These substitutes all have limited long-term durability, however, and in children, conduits might be outgrown during adolescence [12].

We observed well-maintained pulmonary leaflet tissue in redo cases after operations for pulmonary valve stenosis. The regurgitant jet did not distort the leaflets and mostly they grew sufficiently in the meantime. With developing techniques and experience for aortic valve repair, pulmonary valve repair deemed feasible. Secondary repair of the pulmonary valve is not standardized and is an infrequently reported technique. Especially in children, it allows for further growth and maintains mostly autologous tissue; however, midterm and long-term durability is not evaluated yet. This article describes possible techniques of secondary pulmonary valve repair and reports the follow-up results.


   Material and Methods
Top
 Abstract
 Introduction
 Material and Methods
Results
 Comment
 References
 
Patient Characteristics
All patients who underwent secondary pulmonary valve repair between October 2000 and November 2007 in our institution were reviewed. The Johann Wolfgang Goethe University Ethic Committee approved the study, and individual consent for the study was waived. The study included 5 children and 2 adults with median ages of 8 ± 3 years (range, 5 to 13 years) and 38 ± 2.4 years (range, 36 to 40 years), respectively. The mean age at the initial operation was 13 ± 4.6 months for the children and 9 ± 4.4 years for the adults.

Primary indication was tetralogy of Fallot (TOF) in 4 patients, and pulmonary valve stenosis, pulmonary atresia with ventricular septal defect (VSD), and pulmonary valve stenosis with atrial septal defect (ASD) in 1 patient each. All patients with TOF had undergone previous operations for transannular repair of the pulmonary valve. In the patient with isolated pulmonary valve stenosis, percutaneous balloon valvuloplasty had been performed. In the patient with pulmonary valve stenosis and ASD, an intraoperative balloon valvuloplasty was performed and the ASD was closed. The patient with pulmonary atresia and VSD had initially undergone a palliation with modified Blalock-Taussig shunt. Corrective repair had been performed 19 months after palliation using a transannular, glutaraldehyde-fixed autologous pericardial patch, and the shunt had been ligated during this procedure.

The mean interval between the first operation and the redo procedure was 8 ± 2.7 years for children and 29 ± 7 years for adults. Indication for the redo procedure was based on failure of the initial procedure with significant PR and progressive RV dilatation or dysfunction, or both. Patients presented with reduced exercise tolerance.

Preoperative echocardiography and left- and right-sided heart catheterization were performed in all patients, and all were found to have severe PR. The mean preoperative RV dilatation index (RVDI = right ventricular end-diastolic diameter [RVEDD]/left ventricular end-diastolic diameter [LVEDD]; normal, 0.5) was 0.8 ± 0.2 for children and 1.4 ± 0.01 for adults. Six patients had a bileaflet pulmonary valve, and 1 patient had a trileaflet pulmonary valve.

Follow-Up
All patients had prospective follow-up with transthoracic echocardiography in our institution. Follow-up was complete with a mean of 4.1 ± 2.18 years (range, 7 months to 7.5 years). Our particular interest was the occurrence of PR, which was diagnosed by documenting diastolic flow in the RVOT with color Doppler flow imaging. The width of diastolic flow on color Doppler imaging provided a semiquantitative measure of PR severity. PR was graded as none, mild, moderate, or severe. Other variables that had been determined by transthoracic echocardiography were transvalvular gradient, RVEDD and RV end-systolic diameter (RVESD), LVEDD and LVESD, and LV ejection fraction (LVEF).

Operative Techniques
The surgical techniques varied. All operations were performed by the same surgeon.

Patient 1 was 36 years old at the redo procedure. A resuspension of the pulmonary valve, similar to the David technique, was performed. At the initial operation, a valvotomy of a bicuspid valve had been performed. The remaining valve leaflets were sufficient to cover the orifice after reanastomosing a rupture and plication of the elongated free margin (Fig 1). The dilated pulmonary root, leading to a significant PR, was stabilized by resuspension within a tubular Dacron (DuPont, Wilmington, DE) prosthesis analogous to the David procedure (Fig 2). A mitral repair and closure of a VSD aneurysm were performed as well. At the latest echocardiographic examination 6 years after the redo procedure, a mild PR could be detected with a mean transvalvular gradient of 20 mm Hg. The LVEF was 0.65, and RVEDD and LVEDD averaged 5.4 and 5.9 cm, respectively.


Figure 1
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  		Fig 1. Reanastomosed rupture and plication of the elongated free margin of the remaining pulmonary valve leaflets.

 

Figure 2
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  		Fig 2. The dilated pulmonary root has been stabilized by resuspension within a tubular Dacron prosthesis (DuPont, Wilmington, DE), analogous to the David procedure.

 
Patient 2 was 40 years old at the redo procedure. A trileaflet pulmonary valve was present, and the patient had undergone surgical pulmonary valvotomy and ASD closure. An occluder was implanted later for residual ASD. The main pathology was that the left and right posterior pulmonary leaflets had a deficit in leaflet height compared with the anterior leaflet, resulting a significant PR. Reconstruction was possible by leaflet extension with heterologous pericardial patches, by which good coaptation and a regular geometry of the pulmonary valve were achieved. This patient showed mild PR, with a mean transvalvular gradient of 25 mm Hg, a LVEF of 0.62, a RVEDD of 4.4 cm, and a LVEDD of 5.5 cm at the latest follow-up 7.5 years after the redo procedure.

Patient 3 was 6 years old at the redo procedure. After complicated repair of TOF, the patient was transferred primarily for correction of residual VSD. One of the pulmonary valve leaflets had been cut in half during the primary operation. It was not possible to reanastomose this leaflet directly, so a small piece of heterologous pericardium was used to build a normally sized valve leaflet. This patient had to undergo early reoperation for a significant VSD leak recurrence. A small dehiscence of the patch suture line was corrected simultaneously. An echocardiographic examination showed mild PR with a mean transvalvular gradient of 30 mm Hg 4 years after the redo procedure. The LVEF was 0.60, the RVEDD was 1.8 cm, and the LVEDD was 3.8 cm.

Patient 4 was 8 years old at the redo procedure. Severe PR had developed after correction of TOF with a transannular polytetrafluoroethylene (PTFE) patch. At reoperation, the pulmonary annulus was dilated and well-developed pulmonary leaflets were found. Distension at the posterior commissure was corrected, and some restrictions of the cusps were liberated. The pulmonary root was tailored over a Hegar dilator to a diameter of 14 mm. At the latest follow-up 3.5 years postoperatively, this patient showed a mild PR. The mean transvalvular gradient was 15 mm Hg, the LVEF was 0.57, the RVEDD was 3.2 cm, and the LVEDD was 3.8 cm.

Patient 5 was 5 years old at the redo procedure. The child had undergone primary repair of TOF with atresia of the left pulmonary artery. The annulus of the bicuspid valve was enlarged with an autologous pericardial patch, and the left pulmonary artery was reanastomosed and enlarged with the patch as well. At reoperation, the patch had dilated and caused significant pulmonary incompetence. Two structurally normal, well-developed pulmonary leaflets were found. A narrow strip of PTFE patch replaced the dilated pericardium and was tailored under echocardiographic guidance to minimize regurgitation at a minimal gradient. At the latest follow-up of 3 years and 9 months, this patient showed moderate PR, with a mean transvalvular gradient of 21 mm Hg. The LVEF was 0.42, the RVEDD was 1.38 cm, and the LVEDD was 3 cm.

Patient 6 was 13 years old at the redo procedure. After repair of TOF with a transannular pericardial patch, PR was mainly caused by patch dilatation. At reoperation, two structurally normal posterior valve leaflets were found, and one was geometrically adapted by a resuspension stitch at the commissure. To create a normal RVOT diameter width of 12 to 14 mm, a PTFE patch was used to cover the wall defect over an 18-mm Hegar dilator. The average follow-up time was 7 months. Moderate PR with a mean transvalvular gradient of 25 mm Hg could be detected. The LVEF was 0.74, the RVEDD was 2.5 cm, and the LVEDD was 3.4.

Patient 7 was 8 years old at the redo procedure. After shunt palliation for TOF with pulmonary atresia, corrective repair had been performed using a glutaraldehyde-fixed autologous pericardial patch. The remnants of the atretic valve membrane had been liberated and resuspended at the commissures. At reoperation, the pericardial patch had dilated and was resected. One of the leaflets was of good size, but the second smaller one had to be resuspended to create an adequate belly. To compensate for the deficit of leaflet tissue, a monocusp valve was shaped from glutaraldehyde-fixed autologous pericardium sewn to a strip of PTFE patch (Fig 3). At follow-up after 3 years and 2 months, this patient showed mild PR, with a mean transvalvular gradient of 19 mm Hg. The LVEF was 0.67, the RVEDD was 1.5 cm, and the LVEDD was 4.2 cm.


Figure 3
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  		Fig 3. A monocusp valve was shaped from glutaraldehyde-fixed autologous pericardium sewn to a strip of polytetrafluoroethylene patch.

 
Statistical Analysis
Data are presented as mean ± standard deviation. Data obtained preoperatively and postoperatively were compared using the paired t test. The analysis was done with SigmaStat 2.03 software (Systat Software, San Jose, CA).


   Results
Top
 Abstract
 Introduction
 Material and Methods
 Results
Comment
 References
 
Operative and Postoperative Course
The mean cross-clamp time was 55 ± 33.5 minutes, and cardiopulmonary bypass (CBP) time was 92 ± 25 minutes. All patients received secondary pulmonary valve repair. Associated procedures were ASD closure (n = 1), mitral valve annuloplasty (n = 1), and closure of VSD (n = 1). Two patients required resection of obstructive RVOT muscle bundles.

There was no operative or 30-day mortality. All patients but one were extubated within 24 hours after the operation. One patient required prolonged inotropic support due to low cardiac output, with the need for continuous venovenous hemofiltration for a few days. A complete atrioventricular block developed in 1 patient, and permanent pacemaker implantation was required.

The mean intensive care unit stay for our patient cohort was 43.2 ± 70 hours. The mean hospital stay was 17 ± 12 days.

Follow-Up
No late deaths occurred. We observed no neurologic events and no endocarditis or any other valve-related complications.

At the latest echocardiographic examination, PR was mild 5 patients and moderate in 2 patients. Figure 4 illustrates the degree of pulmonary insufficiency for each patient preoperatively and after the redo procedure. The mean RVDI decreased significantly (p = 0.045) from 0.8 ± 0.2 to 0.6 ± 0.2 for children and from 1.4 ± 0.01 to 0.9 ± 0.05 for the 2 adults. Figure 5 demonstrated the RVDI before and at the latest follow-up after the redo procedure. An improvement of the LVEF was also detected. The mean LVEF increased from 0.55 ± 0.10 to 0.60 ± 0.14 for children and from 0.575 ± 0.035 to 0.635 ± 0.02 for adults. The mean RVEDD decreased also significantly (p = 0.048), from 2.5 ± 0.3 to 2.1 ± 0.9 for children and from 7.4 ± 0.2 to 4.9 ± 0.7 for the adults.


Figure 4
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  		Fig 4. Pulmonary insufficiency grade is shown at the latest follow-up before and after secondary repair of the pulmonary valve. (PI = pulmonary insufficiency grade.)

 

Figure 5
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  		Fig 5. Right ventricular dilatation index (RVDI) is shown at last follow-up before and after the redo procedure.

 
Mean transvalvular gradients were 20 ± 4.2 mm Hg (maximum gradient, 25.6 ± 2.2 mm Hg) for children and 22.5 ± 3.5 mm Hg (maximum gradient, 24.4 ± 1.3 mm Hg) for adult patients. As calculated with the paired t test, RVDI (p = 0.045) and RVEDD (p = 0.048) improvement were significant.


   Comment
Top
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
References
 
Excellent long-term results are achieved after surgical correction of obstruction of the RVOT and the pulmonary valve, with most patients living almost normal lives [1, 13, 14]. The 30-year survival rate for patients who survived 30 days after complete TOF repair was reported to be higher than 85% [15, 16]. Besides this favorable long-term survival after operations on TOF, a substantial number of operated-on patients require redo procedures. These are mainly due to PR, with consecutive RV dilatation or dysfunction, or both, which frequently is associated with ventricular arrhythmias and reduced exercise tolerance [11]. PR after primary RVOT reconstruction has very individual dynamics, which leads to variable redo-free intervals [6].

The mean time between the first operation and the redo procedure in our series was 8 ± 2.7 years for children and 29 ± 7 years for adults. Other indications for redo procedures are hemodynamically significantly associated lesions, such as RV septal defect, aneurysm of the pulmonary outflow tract, RVOT stenosis, or branch pulmonary artery stenosis that need surgical management [15]. Murphy and colleagues [15] reported a reoperation probability of 88% during 30 years of follow-up in a series of 163 patients, with a mean 9-year interval to reoperation.

At the redo procedure, a valved conduit is usually implanted. A variety of prostheses can be selected: Heterografts, homografts, autologous pericardial valves, and mechanical prothesis have all been used for pulmonary valve replacement.

Caldarone and colleagues [12] showed that young age at the time of conduit implantation is the most important predictor of conduit failure in a large retrospective study after RVOT operations for congenital heart disease. They found in the same study that pulmonary homografts tended to be the best choice of conduit in children aged younger than 13 years, whereas the type of conduit did not affect conduit durability in older children and adolescents. The reported failure rate is 6.9% for heterograft prostheses and 1.75% to 3.7% for cryopreserved homografts after a midterm follow-up of 5 years [17–19].

Although homograft conduits remain the gold standard for pulmonary valve replacement, certain disadvantages, such as lack of sufficient availability, the requirement of sterilization and preservation, and late complications due to degeneration and calcification have been reported [20, 21]. An additional disadvantage in case of pulmonary valve replacement with homografts or xenografts in children is that they might be outgrown during adolescence [12, 22–27]. These disadvantages have led us to search for other alternatives for RVOT reconstruction in patients with a residual lesion after repair of pulmonary valve stenosis, mainly after transannular patch insertion.

In redo operations, we observed a considerable amount of remaining pulmonary leaflet tissue (Fig 6), sometimes even after pulmonary atresia. The regurgitant jet might be a stimulating factor in the development or growth of lunular structures. As with primary repair, a small coaptation deficit in the pulmonary valve does not lead to severe incompetence, at least with normal pulmonary artery pressures.


Figure 6
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  		Fig 6. A considerable amount of remaining pulmonary leaflet tissue was observed intraoperatively in redo operations.

 
Secondary repair is an infrequently reported technique to cure these patients and allows further growth in most instances; however, midterm and long-term durability have yet to be evaluated. The pathology leading to PR was variable in our patient cohort. A common feature in most of our patients treated initially by transannular patch reconstruction was the dilatation of the inserted patch with consecutive PR. Pulmonary valve competence could be restored by the 2 remaining pulmonary leaflets and resection of the aneurysmatic transannular patch, and significant diameter reduction of the RV could also be achieved [28, 29]. Surgical techniques varied in our series, but secondary repair of an incompetent pulmonary valve was possible due to sufficient remaining leaflet tissue in most of the patients or by using autologous or heterologous pericardium for leaflet extension. In one patient, a resuspension of a pulmonary valve, similar to the David technique, was performed.

Our results showed functional recovery of the RV after intervention, with RVDI reaching almost normal values (0.6 ± 0.2, p = 0.045) in children postoperatively. No significant regurgitation of the secondarily reconstructed pulmonary valve was observed during the follow-up period, which indicated reasonable durability of repair. Because most of the valve tissue is autologous, there is no reason for late degeneration. Even in the case of degeneration of the small pericardial extensions, the autologous part may be large enough to cover the annulus sufficiently.

Two main limitations of our study must be considered. Because we operate a congenital heart surgery program that is small, only a small number of patients were included in the study. The second is that the disease pattern of the patient cohort was inhomogeneous.

In conclusion, secondary valve reconstruction may offer an additional surgical option to solve the problem of progressive PR late after repair of pulmonary valve stenosis. Problems of degeneration or somatic outgrowth of homograft or xenograft conduits during adolescence can be avoided. The stability of the repair is satisfactory.


   References
Top
 Abstract
 Introduction
 Material and Methods
 Results
 Comment
 References
 

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