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Late Repair of the Native Pulmonary Valve in Patients With Pulmonary Insufficiency After Surgery for Tetralogy of Fallot
Late Repair of the Native Pulmonary Valve in Patients With Pulmonary Insufficiency After Surgery for Tetralogy of Fallot Richard D. Mainwaring, MD, Timothy Pirolli, MD, Rajesh Punn, MD, and Frank L. Hanley, MD Divisions of Pediatric Cardiac Surgery and Pediatric Cardiology, Lucile Packard Children’s Hospital at Stanford, Palo Alto, California
Pulmonary regurgitation developing late after tetralogy of Fallot repair is now recognized as a serious threat to the long-term welfare of these patients. This article summarizes our experience with 5 patients who underwent reoperations for treatment of severe pulmonary regurgitation after transannular patch repair of tetralogy
of Fallot. In each case, the intraoperative findings revealed anatomy favorable for valve repair and enabled preservation of the native pulmonary valves.
T
undergone repair of tetralogy of Fallot. These reoperations were performed between March 2009 and April 2011. The age at the time of pulmonary valvuloplasty ranged from 7 to 14 years (median, 9 years), and the interval between the primary repair and valvuloplasty was 6 to 13 years.
Technique We performed pulmonary valve repair in 5 patients (2 male patients and 3 female patients) who had previously Accepted for publication Sept 2, 2011. Address correspondence to Dr Mainwaring, Division of Pediatric Cardiac Surgery, Lucile Packard Children’s Hospital, Stanford University School of Medicine, 300 Pasteur Dr, Falk CVRC, Stanford, CA 94305; e-mail: [email protected].
© 2012 by The Society of Thoracic Surgeons Published by Elsevier Inc
Case Report A 14-year-old boy underwent tetralogy of Fallot repair in infancy. This surgery included transannular patch reconstruction of the right ventricular outflow tract, dividing the annulus at the anterior commissure of the bicuspid valve and retaining the leaflets (Fig 1), with the thought that this might limit the degree of pulmonary insufficiency. He had done well in the interim but had evidence of free pulmonary regurgitation (Fig 2) and a dilatated right ventricle. He was scheduled for elective pulmonary valve replacement. At the time of operation, it was recognized that the 2 native pulmonary valve leaflets had grown and were well developed and mobile (Fig 3). The transannular patch was excised and a valvuloplasty was performed by bringing the anterior commissure together with a 3– 0 polypropylene suture (Fig 4). The remainder of the right ventricular outflow tract was reapproximated primarily (Fig 5). Transesophageal echocardiography demonstrated a peak systolic gradient of 10 mm Hg with trace pulmonary insufficiency.
Results The 5 patients underwent pulmonary valvuloplasty with no resulting mortality. Eight concomitant procedures were performed. There was 1 patient who required repeated repair of the pulmonary valve and subsequently had an uneventful recovery. There were no other postoperative complications. The preoperative degree of pulmonary valve insufficiency was graded as severe in all 5 patients. This was consistent with preoperative right ventricular enddiastolic volumes of 130 to 165 mL/m2 squared. Postop0003-4975/$36.00 doi:10.1016/j.athoracsur.2011.09.016
FEATURE ARTICLES
etralogy of Fallot is a common congenital heart defect that is frequently associated with hypoplasia of the infundibulum, pulmonary valve, and main pulmonary artery trunk. Through the early 1990s, the standard surgical treatment of tetralogy of Fallot included transannular patch augmentation of the right ventricular outflow tract. It eventually became evident that most patients in this cohort experience progressive pulmonary insufficiency with right heart dilatation leading to right heart dysfunction, arrhythmias, and sudden death. It was concluded that establishing competency of the pulmonary valve was important before manifestation of late sequelae. A wide variety of pulmonary valve replacement options have been developed over the past 30 years. Homografts and stented porcine or bovine valves have been the principal options for reconstruction of the right ventricular outflow tract. These valves work well in children but have limited longevity and thus require multiple reoperations. Recently several groups have reported on the use of polytetrafluoroethylene valved conduits as an alternative method for right ventricular outflow tract reconstruction [1, 2]. We treated several patients who had undergone transannular repair for tetralogy of Fallot and returned with severe pulmonary insufficiency and in whom the intraoperative findings suggested the feasibility of valve repair. This report summarizes this experience with late repair of the native pulmonary valve.
(Ann Thorac Surg 2012;93:677–9) © 2012 by The Society of Thoracic Surgeons
678
HOW TO DO IT MAINWARING ET AL PULMONARY VALVE REPAIR
Fig 1. Preoperative anatomy. The native pulmonary valve was bicuspid. The anterior commissure had been split during the original repair, and a transannular patch was placed with retention of the pulmonary valve leaflets. (Ao ⫽ aorta.)
FEATURE ARTICLES
eratively, the degree of insufficiency was graded as none or trace in 3 patients and mild in 2 patients. A comparison of the preoperative to postoperative right ventricular outflow tract hemodynamics are summarized in Table 1. The 5 patients have been followed for 4 to 30 months
Ann Thorac Surg 2012;93:677–9
Fig 3. The pulmonary valve leaflets are well developed and mobile, with a gap located anteriorly along the margin of the patch. (Ao ⫽ aorta.)
postoperatively, and there has been no change in the degree of pulmonary insufficiency during this interval.
Comment This article summarizes our experience with 5 patients who were appropriate for pulmonary valvuloplasty for the treatment of severe pulmonary valve insufficiency after tetralogy of Fallot repair. The conditions for valve repair rather than valve replacement were unique; specifically, these 5 patients had bicuspid native pulmonary valves, had undergone transannular patch repair splitting through the anterior commissure, had retention of the native leaflets at the primary operation, and had a minimal gradient across the existing pulmonary annulus. These circumstances define a narrow set of conditions in which pulmonary valve repair may be applicable. Our experience indicates that the surgical technique of “bicuspidization” resulted in a dramatic decrease in the
Table 1. Hemodynamics Across the Right Ventricular Outflow Tract Preoperative
Fig 2. Preoperative echocardiogram demonstrating severe pulmonary valve regurgitation located anteriorly through the gap created by the transannular patch. (AOV ⫽ aortic valve; MPA ⫽ main pulmonary artery; PR ⫽ pulmonary regurgitation; PV ⫽ pulmonary valve; RVOT ⫽ right ventricular outflow tract.)
Case
Pulmonary Regurgitation
1 2 3 4 5
Severe Moderate/severe Severe Severe Severe
a
Postoperative
Pressure Pulmonary Pressure Gradienta Regurgitation Gradienta
By echocardiography (mm Hg).
12 9 6 5 27
None Trace/mild Mild Trace Trace
10 16 7 16 16
Fig 4. The transannular patch has been excised and the anterior commissure reapproximated. The result is restoration of a bicuspid pulmonary valve. (Ao ⫽ aorta.)
amount of pulmonary insufficiency without creating significant stenosis. It is reasonable to think that valve repair with the native pulmonary leaflets will result in a very durable solution for these patients. Two previous articles reported on the feasibility of pulmonary valve repair for the late treatment of pulmonary valve insufficiency. Papadopoulos and associates [3]
HOW TO DO IT MAINWARING ET AL PULMONARY VALVE REPAIR
679
reported their experience with 7 patients who underwent secondary repair of incompetent pulmonary valves. One patient in their series had anatomy identical to that described in our series, and their operative solution was similar in principal, although not identical in technical detail. Three additional patients in their series had normal, well-developed leaflets amenable to repair. In a separate report, Park and colleagues [4] reported on 20 patients who underwent late pulmonary valve repair after right ventricular outflow tract reconstruction. All of their patients had sufficient remaining native pulmonary valve leaflet tissue to support valve repair. Their approach includes creation of an additional prosthetic leaflet from either polytetrafluoroethylene or fresh autologous pericardium to coapt with the remaining native leaflets. The results indicate that this approach was quite successful in reducing or eliminating pulmonary insufficiency. The prerequisite for undergoing the pulmonary valve repair as described in this article relies on several specific criteria, which include (1) a native bicuspid pulmonary valve, (2) preservation of the native pulmonary valve leaflets at the time of the original tetralogy of Fallot repair, (3) subsequent growth of the native leaflets, with maintenance of mobility of the leaflets, and 4) minimal gradient across the pulmonary annulus. Using these selection criteria, our experience has demonstrated that the postoperative transannular gradient is typically quite low (10 mm Hg), with no palpable thrill. We have attempted valve repair in 2 additional patients in whom the gradients after repair were deemed excessive (⬎ 40 mm Hg) who then underwent pulmonary valve replacement. In summary, the technique for pulmonary valvuloplasty that we describe in this article is applicable to a very discrete set of circumstances in patients who have previously undergone repair of tetralogy of Fallot. The surgical technique is straightforward to perform and provides reproducible results. It is our hypothesis that this technique should result in an extremely durable solution for this discrete set of patients.
Illustrations are by Erin Anne Mainwaring.
References
Fig 5. Completed repair with linear closure of the right ventricular outflow tract. (Ao ⫽ aorta.)
1. Miyazaki T, Yamagishi M, Nakashima A, et al. Expanded polytetrafluoroethylene valved conduit and patch with bulging sinuses in right ventricular outflow tract reconstruction. J Thorac Cardiovasc Surg 2007;134:327–32. 2. Yoshida M, Weardon PD, Dur O, Pekkam K, Morrell VO. Right ventricular outflow tract reconstruction with bicuspid valved polytetrafluoroethylene conduit. Ann Thorac Surg 2011;91:1235–9. 3. Papadopoulos N, Esmaeili A, Zierer A, Bakhtiary F, Ozaslan F, Moritz A. Secondary repair of incompetent pulmonary valves. Ann Thorac Surg 2009;87:1879 – 84. 4. Park CS, Lee C-H, Lee YO, Kim G-B, Kim J-T, Kim YJ. Pulmonary valve repair late after right ventricular outflow tract reconstruction in children and adolescents. Interact Cardiovasc Thorac Surg 2010;10:906 –9.
FEATURE ARTICLES
Ann Thorac Surg 2012;93:677–9
Pulmonary regurgitation developing late after tetralogy of Fallot repair is now recognized as a serious threat to the long-term welfare of these patients. This article summarizes our experience with 5 patients who underwent reoperations for treatment of severe pulmonary regurgitation after transannular patch repair of tetralogy
of Fallot. In each case, the intraoperative findings revealed anatomy favorable for valve repair and enabled preservation of the native pulmonary valves.
T
undergone repair of tetralogy of Fallot. These reoperations were performed between March 2009 and April 2011. The age at the time of pulmonary valvuloplasty ranged from 7 to 14 years (median, 9 years), and the interval between the primary repair and valvuloplasty was 6 to 13 years.
Technique We performed pulmonary valve repair in 5 patients (2 male patients and 3 female patients) who had previously Accepted for publication Sept 2, 2011. Address correspondence to Dr Mainwaring, Division of Pediatric Cardiac Surgery, Lucile Packard Children’s Hospital, Stanford University School of Medicine, 300 Pasteur Dr, Falk CVRC, Stanford, CA 94305; e-mail: [email protected].
© 2012 by The Society of Thoracic Surgeons Published by Elsevier Inc
Case Report A 14-year-old boy underwent tetralogy of Fallot repair in infancy. This surgery included transannular patch reconstruction of the right ventricular outflow tract, dividing the annulus at the anterior commissure of the bicuspid valve and retaining the leaflets (Fig 1), with the thought that this might limit the degree of pulmonary insufficiency. He had done well in the interim but had evidence of free pulmonary regurgitation (Fig 2) and a dilatated right ventricle. He was scheduled for elective pulmonary valve replacement. At the time of operation, it was recognized that the 2 native pulmonary valve leaflets had grown and were well developed and mobile (Fig 3). The transannular patch was excised and a valvuloplasty was performed by bringing the anterior commissure together with a 3– 0 polypropylene suture (Fig 4). The remainder of the right ventricular outflow tract was reapproximated primarily (Fig 5). Transesophageal echocardiography demonstrated a peak systolic gradient of 10 mm Hg with trace pulmonary insufficiency.
Results The 5 patients underwent pulmonary valvuloplasty with no resulting mortality. Eight concomitant procedures were performed. There was 1 patient who required repeated repair of the pulmonary valve and subsequently had an uneventful recovery. There were no other postoperative complications. The preoperative degree of pulmonary valve insufficiency was graded as severe in all 5 patients. This was consistent with preoperative right ventricular enddiastolic volumes of 130 to 165 mL/m2 squared. Postop0003-4975/$36.00 doi:10.1016/j.athoracsur.2011.09.016
FEATURE ARTICLES
etralogy of Fallot is a common congenital heart defect that is frequently associated with hypoplasia of the infundibulum, pulmonary valve, and main pulmonary artery trunk. Through the early 1990s, the standard surgical treatment of tetralogy of Fallot included transannular patch augmentation of the right ventricular outflow tract. It eventually became evident that most patients in this cohort experience progressive pulmonary insufficiency with right heart dilatation leading to right heart dysfunction, arrhythmias, and sudden death. It was concluded that establishing competency of the pulmonary valve was important before manifestation of late sequelae. A wide variety of pulmonary valve replacement options have been developed over the past 30 years. Homografts and stented porcine or bovine valves have been the principal options for reconstruction of the right ventricular outflow tract. These valves work well in children but have limited longevity and thus require multiple reoperations. Recently several groups have reported on the use of polytetrafluoroethylene valved conduits as an alternative method for right ventricular outflow tract reconstruction [1, 2]. We treated several patients who had undergone transannular repair for tetralogy of Fallot and returned with severe pulmonary insufficiency and in whom the intraoperative findings suggested the feasibility of valve repair. This report summarizes this experience with late repair of the native pulmonary valve.
(Ann Thorac Surg 2012;93:677–9) © 2012 by The Society of Thoracic Surgeons
678
HOW TO DO IT MAINWARING ET AL PULMONARY VALVE REPAIR
Fig 1. Preoperative anatomy. The native pulmonary valve was bicuspid. The anterior commissure had been split during the original repair, and a transannular patch was placed with retention of the pulmonary valve leaflets. (Ao ⫽ aorta.)
FEATURE ARTICLES
eratively, the degree of insufficiency was graded as none or trace in 3 patients and mild in 2 patients. A comparison of the preoperative to postoperative right ventricular outflow tract hemodynamics are summarized in Table 1. The 5 patients have been followed for 4 to 30 months
Ann Thorac Surg 2012;93:677–9
Fig 3. The pulmonary valve leaflets are well developed and mobile, with a gap located anteriorly along the margin of the patch. (Ao ⫽ aorta.)
postoperatively, and there has been no change in the degree of pulmonary insufficiency during this interval.
Comment This article summarizes our experience with 5 patients who were appropriate for pulmonary valvuloplasty for the treatment of severe pulmonary valve insufficiency after tetralogy of Fallot repair. The conditions for valve repair rather than valve replacement were unique; specifically, these 5 patients had bicuspid native pulmonary valves, had undergone transannular patch repair splitting through the anterior commissure, had retention of the native leaflets at the primary operation, and had a minimal gradient across the existing pulmonary annulus. These circumstances define a narrow set of conditions in which pulmonary valve repair may be applicable. Our experience indicates that the surgical technique of “bicuspidization” resulted in a dramatic decrease in the
Table 1. Hemodynamics Across the Right Ventricular Outflow Tract Preoperative
Fig 2. Preoperative echocardiogram demonstrating severe pulmonary valve regurgitation located anteriorly through the gap created by the transannular patch. (AOV ⫽ aortic valve; MPA ⫽ main pulmonary artery; PR ⫽ pulmonary regurgitation; PV ⫽ pulmonary valve; RVOT ⫽ right ventricular outflow tract.)
Case
Pulmonary Regurgitation
1 2 3 4 5
Severe Moderate/severe Severe Severe Severe
a
Postoperative
Pressure Pulmonary Pressure Gradienta Regurgitation Gradienta
By echocardiography (mm Hg).
12 9 6 5 27
None Trace/mild Mild Trace Trace
10 16 7 16 16
Fig 4. The transannular patch has been excised and the anterior commissure reapproximated. The result is restoration of a bicuspid pulmonary valve. (Ao ⫽ aorta.)
amount of pulmonary insufficiency without creating significant stenosis. It is reasonable to think that valve repair with the native pulmonary leaflets will result in a very durable solution for these patients. Two previous articles reported on the feasibility of pulmonary valve repair for the late treatment of pulmonary valve insufficiency. Papadopoulos and associates [3]
HOW TO DO IT MAINWARING ET AL PULMONARY VALVE REPAIR
679
reported their experience with 7 patients who underwent secondary repair of incompetent pulmonary valves. One patient in their series had anatomy identical to that described in our series, and their operative solution was similar in principal, although not identical in technical detail. Three additional patients in their series had normal, well-developed leaflets amenable to repair. In a separate report, Park and colleagues [4] reported on 20 patients who underwent late pulmonary valve repair after right ventricular outflow tract reconstruction. All of their patients had sufficient remaining native pulmonary valve leaflet tissue to support valve repair. Their approach includes creation of an additional prosthetic leaflet from either polytetrafluoroethylene or fresh autologous pericardium to coapt with the remaining native leaflets. The results indicate that this approach was quite successful in reducing or eliminating pulmonary insufficiency. The prerequisite for undergoing the pulmonary valve repair as described in this article relies on several specific criteria, which include (1) a native bicuspid pulmonary valve, (2) preservation of the native pulmonary valve leaflets at the time of the original tetralogy of Fallot repair, (3) subsequent growth of the native leaflets, with maintenance of mobility of the leaflets, and 4) minimal gradient across the pulmonary annulus. Using these selection criteria, our experience has demonstrated that the postoperative transannular gradient is typically quite low (10 mm Hg), with no palpable thrill. We have attempted valve repair in 2 additional patients in whom the gradients after repair were deemed excessive (⬎ 40 mm Hg) who then underwent pulmonary valve replacement. In summary, the technique for pulmonary valvuloplasty that we describe in this article is applicable to a very discrete set of circumstances in patients who have previously undergone repair of tetralogy of Fallot. The surgical technique is straightforward to perform and provides reproducible results. It is our hypothesis that this technique should result in an extremely durable solution for this discrete set of patients.
Illustrations are by Erin Anne Mainwaring.
References
Fig 5. Completed repair with linear closure of the right ventricular outflow tract. (Ao ⫽ aorta.)
1. Miyazaki T, Yamagishi M, Nakashima A, et al. Expanded polytetrafluoroethylene valved conduit and patch with bulging sinuses in right ventricular outflow tract reconstruction. J Thorac Cardiovasc Surg 2007;134:327–32. 2. Yoshida M, Weardon PD, Dur O, Pekkam K, Morrell VO. Right ventricular outflow tract reconstruction with bicuspid valved polytetrafluoroethylene conduit. Ann Thorac Surg 2011;91:1235–9. 3. Papadopoulos N, Esmaeili A, Zierer A, Bakhtiary F, Ozaslan F, Moritz A. Secondary repair of incompetent pulmonary valves. Ann Thorac Surg 2009;87:1879 – 84. 4. Park CS, Lee C-H, Lee YO, Kim G-B, Kim J-T, Kim YJ. Pulmonary valve repair late after right ventricular outflow tract reconstruction in children and adolescents. Interact Cardiovasc Thorac Surg 2010;10:906 –9.
FEATURE ARTICLES
Ann Thorac Surg 2012;93:677–9