The pulmonary valvesparing approach to repairing tetralogy of fallot can be performed successfully in as many as 80% of the patients in which its attempted. Tetralogy of fallot tof is the third most common congenital heart defect and the most common form of congenital heart disease to cause cyanosis. Use of a pulmonary neovalve with a transannular patch for. Background tof is a rare congential defect affecting the heart, that results. 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. Pdf ventricular arrhythmia and tetralogy of fallot. In total, 21 patients were treated between september 2008 and february 2010. The natural history of tetralogy of fallot depends on whether a transannular pulmonary valve patch or shunt surgery was necessary in infancy. Advantages to early complete repair include removal of right ventricular outflow tract obstruction, alleviation of systemic. 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. Complete repair of tetralogy of fallot tof remains one of the most commonly performed operations for congenital heart diseases. Some children can have other heart defects along with tetralogy of fallot. Late homograft valve insertion after transannular patch repair of tetralogy of fallot. Treatment options for tetralogy of fallot tof animated.
Muscular right ventricular outflow obstruction has been cut away as part of the repair. Despite the excellent operative survival for tetralogy of fallot tof repair, welldocumented longterm complications and reduced life expectancy remain challenges for these patients and their clinicians. Our objective is to evaluate early and midterm results of pulmonary leaflets sparing with infundibular preservation and tricuspid valve repair in. In an attempt to change the natural history of repaired tof, we at texas childrens hospital houston, tx have developed a management strategy not focused on age, but rather focused on. This animation, fourth in a set of four, depicts blood flow through the right atrium, into the right ventricle and out the pulmonary artery. Transannular patching is used to relieve significant pulmonary annular stenosis during tetralogy of fallot repair. If the predicted prvjlv is higher than the acceptable value selected by the surgeon, a transannular patch is constructed. Ventricular arrhythmia and tetralogy of fallot repair with transannular patch. Tetralogy of fallot is the most common congenital heart defect. Total repair of the tetralogy of fallot generally includes a vsd patch and usually an enlarging rv outflow tract patch, as well as a takedown of any prior arterialpulmonary artery shunt. A new technique of transannular monocusp patchrepair of the. In most children, the cause of tetralogy of fallot isnt known. Late pulmonary valve replacement after repair of tetralogy of.
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. As a result, i got hold of the most interesting ecg i have recorded to date. 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. Some patients may require earlier correction in the setting of severe cyanosis. Treatment options for tetralogy of fallot tof the upper chamber of the heart is opened, and working through the valve that enters the right side of the heart, the hole between the two pumping chambers of the heart is closed with a patch. Texas childrens heart centers animation videos are designed to help children with heart problems better understand their diagnosis and potential treatment options. This animation, third in a set of four, depicts the ventricular septal defect repair with a dacron patch and the stenotic right ventricular outflow repair with a tetralogy of fallot animation part 3 surgical repair on vimeo. Valvesparing repairs have recently gained recognition. B survival after repair of tetralogy of fallot with or without a transannular patch tap or no tap in different decades. Mitral valve imaging as the road map for successful valve repair feat. Jul 31, 2016 recently, in a holter clinic, i dealt with an 8 year old patient who was on the road to recovery after a diagnosis of congenital defect, tetralogy of fallot. Repair of tetralogy of fallot in nearly 400 patients, a. A retrospective analysis of 122 pts who had undergone tof repair with or without tape was performed.
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. In that case, repair is done with a conduit tube from right ventricle to main pulmonary artery adolescents and adults progressive leaking of the pulmonary valve and. Tetralogy of fallot with atrioventricular canal defect. The abnormal superior, anterior, and leftward position of the infundibular sep. Enlargement of the rvot, relieving obstructed pulmonary flow. Chronic pulmonary insufficiency following transannular patch repair of tetralogy of fallot may mandate restoration of a competent pulmonary valve. In patients with severe stenosis of the pulmonary annulus in addition to the muscular stenosis at the right ventricular out flow tract rvot, transannular repair may be inevitable but may lead to pulmonary insufficiency pi featured by pulmonary regurgitation pr. However, neonatal repair for babies with tetralogy of fallot who are intolerably blue and have no contraindications such as anomalous lad or complex dorv or additional avsd, then we would just go in and we would fix it. Total repair of tetralogy of fallot is a corrective surgical procedure that involves closure of the ventricular septal defect vsd and relief of right ventricular outflow tract rvot obstruction. Experts from the cardiac center at the childrens hospital of philadelphia describe tetralogy of fallot tof, a congenital heart disease characterized by four associated features, one of which is a ventricular septal defect a hole in the heart. Transatrial approach with a mini transannular patch 160.
The study included patients who had complete repair of fallot tetralogy with transannular patch from january 2000 to december 2009. The authors determined the need for transannular patch enlargement tape in management of tof by measuring the ratio between pulmonary annulus size to aortic valve annulus size ga ratio. It may be seen more commonly in children with down syndrome or digeorge syndrome. Significant resection of right ventricular muscle, coupled with pulmonary insufficiency resulting from transannular patch repair can lead to early postoperative hemodynamic instability. Repair of tetralogy of fallot in neonates and young infants. Opinions differ regarding the timing of surgeryincluding the need for a palliative systemictopulmonary artery shunt prior to complete repair, the. Cardiac surgeons perform an open heart surgery during a childs first year to treat tetralogy of fallot. Recently, in a holter clinic, i dealt with an 8 year old patient who was on the road to recovery after a diagnosis of congenital defect, tetralogy of fallot. Should patients with transannular patch repair of tetralogy of fallot have pvr by 10yo debate. From the department of cardiac surgery, boston childrens hospital, boston, mass. Lifethreatening arrhythmias and sudden death remain to be serious late complications after correction of tetralogy of fallot. Some degree of right ventricular failure is nearly universal following repair of tetralogy of fallot. Tetralogy of fallot is a rare, complex heart defect that occurs in about 5 out of every 10,000 babies. I think the vast majority of patients with tetralogy of fallot can be repaired electively between 3 and 6 months of age.
Transventricular repair of tetralogy of fallot operative. Approach based on understanding of surgical procedures. Tetralogy of fallot is a conotruncal defect resulting from anterior malalignment of the infundibular septum. The first surgical repair was carried out in 1944 at johns hopkins. Tetralogy of fallot animation part 4 anatomical justice. Aiming to preserve pulmonary valve function in tetralogy of.
Mar 30, 2017 tetralogy of fallot tof with pulmonary stenosis is the common form of tetralogy of fallot, and it is the focus of this article. Ylitalo p1, nieminen h1, pitkanen om1, jokinen e1, sairanen h2. 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. Right ventricular outflow patch to address all levels of obstruction. Historically, the results of repair in infancy were poor and surgeons tended to palliate infants requiring surgery and undertake correction later e. 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. The zscore of pulmonary annulus is most commonly used as a predictor of the need for tape.
Tetralogy of fallot signs, symptoms, diagnosis, treatment, results, provided by cincinnati childrens hospital medical center. A further description was published in 1888 by the french physician etiennelouis arthur fallot, after whom it is named. Primary surgical repair of tetralogy of fallot at under three months of age. Cardiovascular mr imaging after surgical correction of tetralogy of fallot. 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. Tetralogy of fallot is most often diagnosed in the first few weeks of life due to either a loud murmur or cyanosis. An analysis was performed on the clinical variables, morbidity and mortality. Numbers above the curves show the number of living patients 25 and 43 years after corrective operation. Tetralogy tehtraloje of fallot fahlo is a congenital heart defect. Ho ab, bharucha t, jones e, thuraisingham j, kaarne m, viola n. 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. Tetralogy of fallot animation part 4 rupture of bovine. Pulmonary regurgitation appears to be well tolerated early after repair of tetralogy of fallot. 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 patch is made out of a sort of fuzzy dacron material. The aim of this study was to detect ventricular arrhythmia incidence and to find out the relationship between ventricular. Need of transannular patch in tetralogy of fallot surgery carries a higher risk of reoperation but has no impact on late survival. When the infundibular septum is absent, the ventricular septal defect is subarterial. Iorio, gianluca oricchio, roberta iacobelli, antonio amodeo, roberto m. Most patients with tetralogy of fallot tof undergo elective surgical repair between 3 and 6 months of age 15. Tetralogy of fallot animation part 3 surgical repair on vimeo. Surgical strategies like pulmonary leaflets sparing and tricuspid valve repair at time of primary repair may decrease rv overload. Longterm outcome in patients undergoing surgical repair. Although recent literature has focused on the deleterious effects of.
In the smaller picture survival of patients who survived over 30 days after surgery, i. Although in the widest definition, tetralogy of fallot includes pulmonary atresia and ventricular septal defect vsd, only the simple form i. 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. Previously a routine technique, making a large incision in the right ventricle ventriculotomy and using a large transannular patch to repair tof, have been abandoned. Unedited hd video of tetralogy of fallot repair with a limited. The aim of this study was to detect ventricular arrhythmia incidence and to find out the relationship between ventricular arrhythmia and the transannular and infundibular patch repair techniques to correct tetralogy of fallot. Mar 29, 2019 unedited hd video of tetralogy of fallot repair with a limited transannular patch and transatrial vsd closure in a 5 moth old child. Transannular patching is a valid alternative for tetralogy. Valvesparing surgery for tetralogy of fallot procedure. Ventricular arrhythmia and tetralogy of fallot repair with. Elective primary repair of tetralogy of fallot with pulmonary stenosis in. Overall mortality following the repair of tetralogy of fallot is low. Effect of transannular patching on outcome after repair of. Surgical strategies protecting against right ventricular.
Original article from the new england journal of medicine longterm outcome in patients undergoing surgical repair of tetralogy of fallot. The incidence of tetralogy of fallot tof among patients with atrioventricular septal defect avsd is estimated to be about 6% to 10%. In a case of tetralogy of fallot, blood flow to the lungs and the body is affected. Right ventricular rv volume overload increases morbidity and mortality after tetralogy of fallot tof repair. In tetralogy of fallot, whether relieving right ventricular outflow tract obstruction requires transannular patch enlargement tape of the pulmonary valve depends on pulmonary valvular annulus size. A novel predictive value for the transannular patch. If the rv outflow tract patch extends through the pulmonary valve into the pa transannular patch, varying degrees of pulmonary valve. The first anatomic repair of tof was performed in 1954. Pulmonary valve leaflets that are preserved at initial surgery may grow and develop normal morphology and subsequent valve repair may be possible. When a transannular patch has been inserted, it is usually much less. 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.
The anatomic feature of the tetralogy of fallot is underdevelopment of the. Ga ratios were smaller in patients undergoing tape. We sought to determine whether a repair that increases the pv annulus and augments. Corrective repair of tetralogy of fallot involves closure of the ventricular septal defect with a synthetic dacron patch so that the blood can flow normally from the left ventricle to the aorta. Should patients with transannular patch repair of tetralogy. In this video series, you will learn how our experts diagnose tof before birth, monitor babies during pregnancy, plan for delivery and care after. 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. Tetralogy of fallot msd manual professional edition. Treatment of fallot tetralogy with a transannular patch. Tetralogy of fallot was initially described in 1671 by niels stensen. Oct 18, 2016 most of these adult congenital heart disease patients underwent tetralogy of fallot repair in childhood.
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. Pediatric and adult interventional cardiac symposium. Backgroundthe timing of repair of tetralogy of fallot tof remains controversial. When we repair tetralogy of fallot, there are two major things that we need to do. Total repair of tetralogy of fallot radiology reference. Tetralogy of fallot tof is an important lesion for all pediatric and congenital heart surgeons.
Oct 17, 2007 5 month old baby with cyanotic heart disease, tetralogy of fallot. In 1924, maude abbott coined the term tetralogy of fallot. Tetralogy of fallot with pulmonary stenosis treatment. A congenital heart defect is a problem with the hearts structure thats present at birth. Currently, the mid and longterm outcomes reported following tof repair are often from single centers, or even single surgeons, which may not be largely. Coronary artery anomalies are seen in approximately 57% of the cases. Pulmonary valve pv incompetence following transannular patch tap repair of tetralogy of fallot tof results in longterm morbidity and mortality. Should patients with transannular patch repair of tetralogy of fallot. This type of heart defect changes the normal flow of blood through the heart. Most kids get definitive repair in 1st year of life. Tetralogy of fallot video series childrens hospital of. In designing the most appropriate operation for children with tof, the postoperative physiology should be taken into account, both in the short and long. The heart program at nicklaus childrens hospital remains dedicated to providing the very best and safest experience for patients undergoing congenital heart surgery. Reoperations are no more frequent that with the use of transannular patches.
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