Tetralogy of fallot with pulmonary atresia treatment. The abnormal superior, anterior, and leftward position of the infundibular sep. Pulmonary valve pv incompetence following transannular patch tap repair of tetralogy of fallot tof results in longterm morbidity and mortality. In fact, in type iib patients, the infundibulum nearly always has to be incised and augmented with patch ventriculotomy. Ventricular septal defect a hole in the wall of the heart septum. We changed our approach from one of initial palliation in the infant to one of primary repair around the age of 6 months, or earlier if clinically indicated. B unifocalization using patch material to reconstruct central pulmonary artery pa confluence.
We sought to determine whether a repair that increases the pv annulus and. B survival after repair of tetralogy of fallot with or without a transannular patch tap or no tap in different decades. Treatment of fallot tetralogy with a transannular patch. Cove point contains comprehensive information on all congenital heart defects, including atrial septal defect asd, ventricular septal defect vsd, hypoplastic left heart syndrome hlhs, and tetralogy of fallot tof. We study the valve first, just like carpentier taught us to study the mitral valve before you do anything, we study the pulmonary valve, typically open the main pa and a.
Although tof was once uniformly lethal, current operative mortality is less 2%, and it is estimated that more tha. The anterior wall of the rvot is opened through the annulus of the pulmonary valve. Routine primary repair of tetralogy of fallot in neonates and infants less than three months of age. Total repair of tetralogy of fallot radiology reference. Complete repair of tetralogy of fallot in the neonate ncbi. For patients who receive a transannular rvot patch as part of the tof repair, the. Tetralogy of fallot with common av canal av septal defect avsd is the presence of both tetralogy of fallot and complete avsd.
Cardiac surgeons perform an open heart surgery during a childs first year to treat tetralogy of fallot. Need of transannular patch in tetralogy of fallot surgery. Children and adolescents with repaired tetralogy of fallot are often. Valvesparing surgery for tetralogy of fallot procedure details. Elective primary repair of acyanotic tetralogy of fallot. Surgical repair of tetralogy of fallot with complete atrioventricular canal. Pulmonary valve replacement in adult congenital cardiac surgery. So i have actually not done a transannular patch, a classic transannular patch, in the last 3 or 4 years now. The narrowing in the pulmonary valve is also repaired with the transannular patch to improve blood flow to the lungs. Ann thorac surg how i teach it mainwaring and hanley 1779 2016.
To assess diastolic right ventricular rv physiology after tetralogy of fallot repair in infancy. Tetralogy of fallot is the most common congenital heart disease manifesting with cyanosis. The incidence of tetralogy of fallot tof among patients with atrioventricular septal defect avsd is estimated to be about 6% to 10%. Early primary repair of tetralogy of fallot sciencedirect. Exercise capacity after complete repair of tetralogy of fallot. Although recent literature has focused on the deleterious effects of pulmonary regurgitation, inadequate relief of stenosis may increase postoperative mortality and the reintervention rate. In total, 21 patients were treated between september 2008 and february 2010. Backgroundin tetralogy of fallot, transannular patching is suspected to be. The principles of surgical correction of tof involve closure of the vsd and relief of the rvoto. Pulmonary valve stenosis a narrowed or completely blocked pulmonary valve, which restricts blood flow from the valve to the lungs. The longterm outcome of children after complete repair of tetralogy of fallot tof has been well described. Tetralogy of fallot congenital heart defects treatment. Early palliation for hypercyanotic spells or severe cyanosis can be provided by a modified blalocktaussig shunt.
One is to close the ventricular septal defect so that theres no mixing between the two pumping chambers, and the second is to relieve the blockage to the lungs. Read elective primary repair of acyanotic tetralogy of fallot in early infancy. Need of transannular patch in tetralogy of fallot surgery carries a. Valvesparing repairs have recently gained recognition. If placing a transannular patch, can make a monocusp pericardial valve to reduce pr but it might not really impact mortality, length of stay, post op hemodynamics some propose a complete transatrial repair so theres no ventriculotomy unless pt needs a transannular patch.
While surgical repair for tetralogy of fallot gives excellent short and longterm results, adult survivors often fall prey to arrhythmia and sudden cardiac death. Most patients with tetralogy of fallot tof undergo elective surgical repair between 3 and 6 months of age 15. Tetralogy of fallot tof childrens hospital of philadelphia. Adapted from 6, by permission of the society of thoracic surgeons. 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. N in legend is the number of patients operated during the era in each group. Tetralogy of fallot is treated by surgical repair of the defects.
Should patients with transannular patch repair of tetralogy of fallot have pvr by 10yo debate by pediatric and adult interventional cardiac symposium featuring jamil aboulhosn, lee benson. Tetralogy of fallot with and without pulmonary atresia. Left pulmonary artery kinking caused by outflow tract. Indication and technique of total correction of tetralogy of fallot in. The word tetralogy means a group of 4, and in tetralogy of fallot, there are 4 heart defects that occur in combination. To create an unobstructed way out of the right ventricle, pulmonary valvotomy, the insertion of an outflow tract patch or a transannular patch are often required. Consequently, most patients acquire pulmonary regurgitation as a result of the repair. Backgroundin tetralogy of fallot, transannular patching is suspected to be responsible for late right ventricular dilatation. Total repair of tetralogy of fallot requires cardiopulmonary bypass and aortic crossclamping.
Methods and resultsin our institution, 191 patients survived a tetralogy of fallot repair between 1964 and 1984. Opinions differ regarding the timing of surgeryincluding the need for a palliative systemictopulmonary artery shunt prior to complete repair, the. Early outcome of fallot repair with preservation of the. Tetralogy of fallot tof is a complex congenital cardiac abnormality representing. Tetralogy of fallot is most often diagnosed in the first few weeks of life due to either a loud murmur or cyanosis. Transannular patching is used to relieve significant pulmonary annular stenosis during tetralogy of fallot repair. Early age at repair prevents restrictive right ventricular. The obstruction to flow into the lungs leads to blood being diverted through the vsd to the aorta. The anatomic feature of the tetralogy of fallot is underdevelopment of the. Babies with tetralogy of fallot usually have a patent ductus arteriosus at birth that provides additional blood flow to the lungs, so severe cyanosis is rare early after birth. This rare lesion represents only 2% of all cases of tetralogy of fallot. Tetralogy tehtraloje of fallot fahlo is a congenital heart defect.
Exercise capacity after repair of tetralogy of fallot in. Some patients may require earlier correction in the setting of severe cyanosis. Correction with transannular patch was performed in 80%, as reported by seddio et al. Repair of tetralogy of fallot with pulmonary atresia. Tetralogy of fallot is the most common congenital heart defect. We aim to report contemporary results using these treatment options in a comparative study. Tetralogy of fallot is composed of a malaligned ventricular septal defect vsd. Transannular patching was used in 99 patients 52%, patch closure of a right ventriculotomy in 35. Pulmonary valve stenosis a narrowed or completely blocked pulmonary valve, which restricts blood flow from the.
Timing of complete repair of tetralogy of fallot varies. Gore, newark, del patch is then sutured to both margins of the incision, increasing the. The first complete description of tetralogy of fallot tof is credited to the french. Hospital, transannular patch repair was avoided whenever judged possible, resulting in a low rate of tape only editorial the problems related with primary repair for tetralogy of fallot, especially about transannular patch repair atsushi mizuno, koichiro niwa department of cardiology, st.
In a case of tetralogy of fallot, blood flow to the lungs and the body is affected. Before the time of corrective surgery the survival rate was 50% over the first years of life and only very few patients reached an adult age. 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. Pulmonary valve preservation pvp has recently gained recognition even in repair of patients with z score 3 or less.
Restrictive rv physiology after tetralogy of fallot repair is related to type of repair, pulmonary regurgitation, and late arrhythmias. In many cases, the repair is made at around 6 months of age, or even a little earlier. Tetralogy of fallot with atrioventricular canal defect. Muscular right ventricular outflow obstruction has been cut away as part of the repair. Aiming to preserve pulmonary valve function in tetralogy. Background early primary repair of tof normalizes intracardiac flow patterns, which may allow subsequent normal rvot growth. Fact sheet on fallot s tetralogy the combination of a vsd with pulmonary stenosis, with the aorta overriding sitting astride the vsd and with rv hypertrophy is termed tetralogy of fallot. In designing the most appropriate operation for children with tof, the postoperative physiology should be taken into account, both in the short and long. A team of cardiac surgeons performs the surgery, usually before an infant is 1 year old. Right ventricular outflow patch to address all levels of obstruction. Oct 18, 2016 most of these adult congenital heart disease patients underwent tetralogy of fallot repair in childhood. Pulmonary regurgitation is almost universal after corrective repair of tetralogy of fallot, more so in those who require a transannular patch for widening of the right ventricular outflow tract. Results of total correction of tetralogy of fallot with complete atrioventricular canal. No significant differences were found between patients with and without a transannular patch.
C completed repair with ventricular septal defect patch and right ventricle to pa valved conduit. Numbers above curves show the number of patients alive after 25 years of the followup in the 1970s and the 1980s. Tetralogy of fallot, survival, transannular patch, late results, populationbased. What is the optimal age for repair of tetralogy of fallot. Because of the wide variability of pulmonary blood supply, diagnosis and surgical management of tetralogy of fallot tof with pulmonary atresia pa is more difficult than that of classic tetralogy of fallot, and therefore, it is worthy of separate consideration. Fortynine patients underwent repair with a transannular patch and 12 with an. Typically in the first few months of life we will perform open heart surgery to patch the hole vsd and widen the pulmonary valve or artery. Jun 29, 20 transannular patch repair is performed when there is marked stenosis of the rvot or pulmonary annulus.
The fact of having 3 holding stitches allowed us to use the beatingheart technique. Objectives we sought to determine if early primary repair of acyanotic tetralogy of fallot tof can be performed safely with low requirement for transannular patching tap and thereafter allow normal right ventricular outflow tract rvot growth. Backgroundcontroversy regarding the timing for the repair of tetralogy of fallot centers around initial palliation versus primary repair for the symptomatic neonateyoung infant and the optimal age for repair of the asymptomatic child. Use of a pulmonary neovalve with a transannular patch for. 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 artery conduit in 11 33%. Primary repair versus surgical and transcatheter palliation. Mar 30, 2017 tetralogy of fallot with common av canal av septal defect avsd is the presence of both tetralogy of fallot and complete avsd. The transannular patch technique was used to relieve the obstruction at the level of the annulus, which lead to the wellknown, longterm problems associated with free pulmonary valve regurgitation pr, including right ventricular dilation. Stenosis of the branch pulmonary arteries after tetralogy of fallot repair can result from several mechanisms. When we repair tetralogy of fallot, there are two major things that we need to do. Comparison of pulmonary regurgitation and rv size after repair of tetralogy of fallot. The problems related with primary repair for tetralogy of.
Repairing the heart defects will allow oxygenpoor blue blood to travel its normal route through the. Outcome after surgical repair of tetralogy of fallot. In fact, most centers prefer to complete tof repair between 3 and 6 months of age. A total of 600 patients underwent surgical repair of tof before the. Key words tetralogy, ventricular septal defect, pulmonary stenosis, pulmonary atresia, jet. Tetralogy of fallot tof is an important lesion for all pediatric and congenital heart surgeons. You may need to limit your activity if there is leftover obstruction or a pulmonary valve leak, which is common after repair. Tetralogy of fallot is a rare, complex heart defect that occurs in about 5 out of every 10,000 babies. Cardiovascular mr imaging after surgical correction of. This type of heart defect changes the normal flow of blood through the heart. The combination of a vsd with pulmonary stenosis, with the aorta overriding sitting astride the vsd and with rv hypertrophy is termed tetralogy of fallot. B the incision is extended to join the infundibulotomy.
Transannular patching is a valid alternative for tetralogy. Tetralogy of fallot video series childrens hospital of. A congenital heart defect is a problem with the hearts structure thats present at birth. T he first completed repair of tetralogy of fallot was successfully. In fact, since the aggressive attention to the ductus insertion site, we have. Intracardiac corrective surgery of tof was performed for the first time in 1955. Apr 23, 2012 pulmonary regurgitation is almost universal after corrective repair of tetralogy of fallot, more so in those who require a transannular patch for widening of the right ventricular outflow tract.
Fortyseven patients were investigated, 27 and 20 patients in lund and london, respectively. Right ventricular function and exercise performance late after primary repair of tetralogy of fallot with the transannular patch in infancy. Pulmonary stenosis congenital heart disease cove point. Tetralogy of fallot tof is the most common cyanotic congenital heart disease in all age groups, constituting approximately 8% of congenital heart disease overall. Should patients with transannular patch repair of tetralogy. Transannular patching is a valid alternative for tetralogy of. The aim of the study was to evaluate the results with pulmonary valve preservation versus transannular patch in selected patients with tetralogy of fallot, according to pulmonary valve annulus z score. In some cases, depending on the unique needs of the patient, we will perform a temporary repair until a complete repair can be done.
Neonatal repair of tetralogy of fallot with and without pulmonary atresia. A pericardial or synthetic polytetrafluoroethylene goretex. Babies with tetralogy of fallot usually have a patent ductus arteriosus at birth that provides additional blood flow to the lungs, so severe cyanosis is rare early after birth as the ductus arteriosus closes, which it typically will in the first days of life, cyanosis can. D the transannular patch is narrowed with a series of clips. Complete surgical repair of this lesion is riskier than repair of tetralogy of fallot or avsd alone. Use of a pulmonary neovalve with a transannular patch for repair. A patch across the pulmonary valve annulus a transannular patch is often. Total repair of tetralogy of fallot requires cardiopulmonary bypass and aortic. If tetralogy of fallot has been repaired with surgery, and theres no obstruction or leak in the pulmonary valve, you may be able to participate in normal activities without much increased risk. Need of transannular patch in tetralogy of fallot surgery carries a higher risk of reoperation but has no impact on late survival. Pitkanen, eero jokinen, heikki sairanen, need of transannular patch in tetralogy of fallot surgery carries a higher risk of reoperation but has no impact on late survival. Ylitalo p1, nieminen h1, pitkanen om1, jokinen e1, sairanen h2.
Transanular patch the incidence of transannular patch use at the time of complete repair in younger patients ranges from 90 to 100% touati et al. Objectives treatment of infants with tetralogy of fallot tof has evolved in the last two decades with increasing use of primary surgical repair prr and transcatheter right ventricular outflow tract palliation rvotd, and fewer systemictopulmonary shunts sps. In patients with free pulmonary regurgitation and right ventricular dilatation after transannular patch repair, we have observed that the pulmonary outflow tract can dilate and elongate craniad and rotate to the left, resulting in kinking and obstruction of the previously. The treatment of tetralogy of fallot tof exemplifies both the remarkable success and the questions and challenges associated with successful repair and longterm survival. The cove point foundation congenital heart resource center is the worlds largest resource for information on pediatric and adult congenital heart disease. Hence an early diastolic murmur along the left sternal edge following repair of tetralogy of fallot is most often due to pulmonary regurgitation.
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