Ventricular septal defect a hole in the wall of the heart septum. Pulmonary valve preservation strategies for tetralogy of fallot repair constantine mavroudis, md t he optimal repair for tetralogy of fallot is challenged by the management of the small right ventricular out. However, ensuing chronic pulmonary insufficiency may lead to right ventricular dilation, dysfunction and arrhythmias, eventually requiring pulmonary valve replacement. In total, 21 patients were treated between september 2008 and february 2010. Pulmonary valve restitution following transannular patch repair of. One patient with pulmonary gradient underwent reoperation at postoperative 15th month and died due to multiple organ failure. Most patients with tetralogy of fallot tof undergo elective surgical repair between 3 and 6 months of age 15. Pulmonary allograft conduit repair of tetralogy of fallot. Tetralogy of fallot tof is an important lesion for all pediatric and congenital heart surgeons.
Pulmonary valvesparing techniques for tetralogy of fallot. The initial efforts at repair focused on the complete relief of obstruction across the right ventricular outflow tract rvot. The most common physical finding for a child or adolescent with repaired tetralogy of fallot tof is a residual heart murmur. Treatment of fallot tetralogy with a transannular patch. Symptoms of fatigue, especially with exercise, may develop. This is due to a mixing of oxygenated and deoxygenated blood in the left ventricle via the ventricular septal defect vsd and preferential flow of the mixed blood from both ventricles through the aorta because of the obstruction to flow through the pulmonary valve.
A functioning pulmonary valve does not improve immediate postsurgical outcomes. However, if there is also a need to widen the outflow tract of the right ventricle, a transannular patch may be required. Pulmonary valve stenosis a narrowed or completely blocked pulmonary valve, which restricts blood flow from the. Second degree pulmonary insufficiency that was developed in 12 patients who underwent transannular patch closure was followed up to day. Primary repair of fallot tetralogy has been performed successfully for the last 45. Transannular patching is a valid alternative for tetralogy of fallot and complete atrioventricular septal defect repair gianluca brancaccio, guido michielon, sergio filippelli, gianluigi perri, duccio di carlo, fiore s. The natural history of tetralogy of fallot depends on whether a transannular pulmonary valve patch or shunt surgery was necessary in infancy. Pulmonary valve pv incompetence following transannular patch tap repair of tetralogy of fallot tof results in longterm morbidity and mortality. Some patients may require earlier correction in the setting of severe cyanosis. Transannular patch of the pulmonary artery simulation on a. The transannular patch tap repair used in the correction of tetralogy of fallot tof inevitably causes pulmonary regurgitation. In designing the most appropriate operation for children with tof, the postoperative physiology should be taken into account, both in the short and long term.
A total of 600 patients underwent surgical repair of tof before the age of 15 years during. Tetralogy of fallot tof is the third most common congenital heart defect and the most common form of congenital heart disease to cause cyanosis. Pulmonary effects on exercise testing in tetralogy of. Valvesparing surgery for tetralogy of fallot procedure. Need of transannular patch in tetralogy of fallot surgery carries a. Longterm outcome in patients undergoing surgical repair. However, when distal pulmonary artery problems are added as a risk factor this rate rises to 21%, and valved conduit insertion increases the mortality rate kirklin and barrattboyes, 1986. Tetralogy of fallot tof with pulmonary stenosis is the common form of tetralogy of fallot, and it is the focus of this article. Transesophageal echocardiography is utilized to help assess adequacy of repair. Precisely evaluating the need for transannular patch tap placement is very important in the surgical treatment of tetralogy of fallot. Pulmonary valve restitution following transannular patch repair of tetralogy of fallot. The characteristic feature of rvrp is the presence of a direct enddiastolic flow edff during atrial contraction in the main pulmonary artery. This can lead to significant pulmonary insufficiency and increased right ventricular. Background a transannular patch is often used in the contemporary surgical repair of tetralogy of fallot.
Valvesparing surgery for tetralogy of fallot procedure details. The word tetralogy means a group of 4, and in tetralogy of fallot, there are 4 heart defects that occur in combination. Tetralogy of fallot patients repaired with a transannular patch without pulmonary valve replacement were compared. The problems related with primary repair for tetralogy of. Monocusp valve placement in children with tetralogy of. To report on our initial experience with the implantation of a pulmonary valve using nunns technique in association with a transannular patch for the complete repair of the tetralogy of fallot. Patients who have had repair of tetralogy of fallot can also redevelop a narrowing at the. Total repair of tetralogy of fallot requires cardiopulmonary bypass and aortic. The most frequent problem that occurs after tetralogy of fallot repair is pulmonary backflow, or leaking from the pulmonary valve. Pulmonary stenosis congenital heart disease cove point. Although recent literature has focused on the deleterious effects of pulmonary regurgitation, inadequate relief of stenosis may increase postoperative mortality and the reintervention rate.
Cardiovascular mr imaging after surgical correction of. Our surgical results of tetralogy of fallot selcuk. Chronic pulmonary insufficiency following transannular patch repair of tetralogy of fallot may mandate restoration of a competent pulmonary valve. At present, the majority of fallot patients who undergo transannular patch tap repair are not candidates for tpvr. Transannular patching is a valid alternative for tetralogy. Value of pulmonary annulus area index in predicting. The problems related with primary repair for tetralogy of fallot. We hypothesized that the pulmonary annulus area index paai, the pulmonary. Tetralogy of fallot tof is one of the most common congenital heart diseases for which patients are referred for postoperative magnetic resonance mr imaging evaluation. Total repair of tetralogy of fallot radiology reference. The most common surgical procedures for tof repair include infundibulectomy, transannular pulmonary artery patch repair, and right ventriclepulmonary artery conduit placement.
Original article from the new england journal of medicine longterm outcome in patients undergoing surgical repair of tetralogy of fallot. A patch across the pulmonary valve annulus a transannular patch is often. Tetralogy of fallot, survival, transannular patch, late results, populationbased. Transannular patching is used to relieve significant pulmonary annular stenosis during tetralogy of fallot repair. We report our experience with repair of tetralogy of fallot associated with. The study included patients who had complete repair of fallot tetralogy with transannular patch from january 2000 to december 2009. Patch enlargement in repair of tetralogy of fallot. The child or adolescent with repaired tetralogy of fallot. Backflow from the tricuspid valve and aortic valve also can occur. Valvesparing repairs have recently gained recognition. Surgical strategies protecting against right ventricular.
Following adequate rewarming, the patient is weaned from cardiopulmonary bypass. Pulmonary valve restitution following transannular patch. Complete repair of tetralogy of fallot consists of patch closure of the ventricular septal defect, widening of the right ventricular outflow tract with muscle resection, pulmonic valvuloplasty and, when warranted, patch augmentation of the main pulmonary artery. Among 814 patients undergoing repair of tetralogy of fallot with pulmonary stenosis between 1967 and may 1986, transannular patching in the current era was a weak risk factor for death early postoperatively predicted 30day mortality, 4% with a transannular patch and 1. The first anatomic repair of tof was performed in 1954. Aiming to preserve pulmonary valve function in tetralogy.
Effect of transannular patching on outcome after repair of. Monocusp valve placement in children with tetralogy of fallot undergoing repair with transannular patch. Use of a pulmonary neovalve with a transannular patch for repair. A patch across the pulmonary valve annulus a transannular patch is often required in order to adequately relieve right ventricular outflow tract obstruction. Tetralogy of fallot msd manual professional edition. Right ventricular outflow tract obstruction was relieved by transannular patch in 14 cases 42%, infundibular patch with preservation of the pulmonary valve in 7 21%, and right ventricletopulmonary. Etiology of right ventricular restrictive physiology early.
Recent evidence indicates, however, that use of a transannular patch can cause pulmonary insufficiency, or weakness in the pulmonary valve that allows backflow of blood into the right ventricle. The operative mortality rate from a number of series for transannular patch repair of tetralogy of fallot averages 16%, ranging from 3% to 63%. Tetralogy of fallot is a conotruncal defect resulting from anterior malalignment of the infundibular septum. The problems related with primary repair for tetralogy of fallot, especially about transannular patch repair since the initial surgical correction of tetralogy of fallot tof in 1954, advances in management have helped reduce early surgical mortality to less than 2% 1. The most common surgical procedures for tof repair include infundibulectomy, transannular pulmonary artery patch repair, and right ventriclepulmonary artery conduit.
Tetralogy of fallot tof repair often includes transannular patch augmentation of the right ventricular outflow tract. The narrowing in the pulmonary valve is also repaired with the transannular patch to improve blood flow to the lungs. Surgery is needed to repair or replace the leaking valve. A novel predictive value for the transannular patch enlargement in. The longterm influence of pulmonary valve regurgitation. Although the society of thoracic surgeons congenital database has been instrumental in the understanding of hospital outcomes following the repair of tetralogy of fallot tof, insights into the mid and longterm results are less concrete.
Valvesparing techniques for the treatment of tetralogy of fallot with pulmonary stenosis have evolved over the past few decades. Methods to repair tetralogy of fallot have evolved from large ventriculotomy, large transannular patch placement techniques to smaller, transatrial approaches with valvesparing strategies in select patients. The incidence of tetralogy of fallot tof among patients with atrioventricular septal defect avsd is estimated to be about 6% to 10%. Currently, the mid and longterm outcomes reported following tof repair are often from single centers, or even single surgeons, which may not be largely. Initial results using a transannular patch with a modified monocusp valve to repair the outflow tract in the tetralogy of fallot were promising. Associated risk factors were severe pulmonary insufficiency p0.
Development of a novel hybrid strategy for transcatheter. We sought to determine whether a repair that increases the pv annulus and augments. Right ventricular restrictive physiology rvrp is a common finding after repair of tetralogy of fallot tof. In these cases, replacement of the pulmonary valve is often recommended.
Pulmonary valve leaflets that are preserved at initial surgery may grow and develop normal morphology and subsequent valve repair may be possible. Native pulmonary valve restoration after remote tetralogy. Transatrial repair of tetralogy of allot with limited. Recent trends revealed many centers used the pulmonary valve annulus. Tetralogy of fallot results in low oxygenation of blood. Arrhythmias ahrithmeahs are another complication that may develop. Pulmonary valve preservation strategies for tetralogy of. Enlarging the right ventricular outflow tract the region from the right ventricular muscle to the pulmonary artery which allows blood to flow to the lungs often requires an incision across the pulmonary valve and placement of a patch to widen the outflow tract called a transannular patch. This case illustrates the feasibility of cardiac rest on extracorporeal membranous oxygenation for a very ill adult with conduit endocarditis who received a right ventricletopulmonary artery valveless conduit for later transcatheter pulmonary valve. The initial efforts at repair focused on the complete relief of obstruction across the right ventricular outflow tract rvot, usually including an aggressive resection of right ventricular. Iorio, gianluca oricchio, roberta iacobelli, antonio amodeo, roberto m. Volume overload of the right ventricle is aggravated with any associated tricuspid regurgitation and it is considered the major cause of function impairment and increased morbidity and mortality in those patients 1,2,3.
Transcatheter pulmonary valve replacement tpvr is an accepted therapy for treatment of dysfunctional right ventricular outflow tract rvot conduits. We report herein the results of a longterm followup study conducted on 50 patients who had undergone a tap repair 2029 years earlier to evaluate the influence of pulmonary regurgitation on their late outcome and quality of life. In a small percentage of children, this pulmonary insufficiency can lead to diminished function of the right ventricle. Severe pulmonary valve regurgitation following tetralogy of fallot tof repair leads to right ventricular rv volume overload.
Tetralogy of fallot, pediatric cardiac center delaware. They finally concluded that rvot reconstruction in tof, using only a limited transannular incision and a stiff dacron patch, that restricts the pulmonary annulus. An analysis was performed on the clinical variables, morbidity and mortality. Tetralogy of fallot with atrioventricular canal defect. Use of a pulmonary neovalve with a transannular patch for. Tetralogy of fallot with pulmonary stenosis treatment. If a patch is inserted, it may be used to widen the pulmonary artery from the valve upward. There were no complet atrioventricular block and disrhythm in long term. Pulmonary valve stenosis a narrowed or completely blocked pulmonary valve, which restricts blood flow from the valve to the lungs. Opinions differ regarding the timing of surgeryincluding the need for a palliative systemictopulmonary artery shunt prior to complete repair, the. This enddiastolic forward flow is caused by increased right ventricular enddiastolic pressure due to right ventricular myocardial. Complete repair tetralogy of fallot without pulmonary atresia. This patch covers part of the wall of the right ventricle as well as widening the pulmonary artery and pulmonary valve.
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