A prenatal ultrasound study of cerebral sulci and gyrus development in. Approximately 90% of patients with a right aortic arch and mirrorimage branching have tetralogy of fallot. The child or adolescent with repaired tetralogy of fallot. Numbers above the curves show the number of living patients 25 and 43 years after corrective operation. Cardiac surgeons perform an open heart surgery during a childs first year to treat tetralogy of fallot. How is surgical treatment of tetralogy of fallot tof. Although in the widest definition, tetralogy of fallot includes pulmonary atresia and ventricular septal defect vsd, only the simple form i. 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. Transannular patching for tetralogy of fallot with an. Transventricular repair of tetralogy of fallot operative. Enlarged aortic root a normal aortic diameter does not exclude tetralogy of fallot, there is a 0. To assess outcomes of prenatally diagnosed tetralogy of fallot and determine factors associated with the choice to undergo a valvesparing repair versus transannular patch, and the use of prostaglandins at birth.
Approach based on understanding of surgical procedures. Opinions differ regarding the timing of surgeryincluding the need for a palliative systemictopulmonary artery shunt prior to complete repair, the. For decades, repair of tetralogy of fallot tof has been performed successfully. Transannular patching is a valid alternative for tetralogy. Tetralogy of fallot tof is the third most common congenital heart defect and the most common form of congenital heart disease to cause cyanosis.
Most children with the simpler versions of tetralogy of fallot wont need a cardiac catheterization, ct scan or mri. Pr, the most common sequela of transannular or rv outflow tract patch repair, is present in nearly all tof patients and can be accurately quantified with phasecontrast imaging. Implications for valvesparing repair versus transannular patch. Pulmonary stenosis congenital heart disease cove point. Of patients with tetralogy of fallot, 25% have a right aortic arch, and most have a mirrorimage branching pattern. Some patients may require earlier correction in the setting of severe cyanosis. Since the first procedures in the 1950s, advances in the diagnosis, perioperative and surgical treatment, and postoperative care have been such that almost all those born with tetralogy of fallot can now expect to survive to adulthood. Overall mortality following the repair of tetralogy of fallot is low. Cardiac mri is a valid alternative to echocardiography when ultrasound images do. Surgical repair was first introduced in the 1950s and there is now a large population of adults with repaired tetralogy. One provides temporary improvement by a shunt to give more blood flow to the lungs. Valvesparing surgery for tetralogy of fallot procedure. Discuss the technical aspects of cardiac mr imaging and the benefits of using this modality in patients who undergo repair of tetralogy of fallot.
Name deepak age 4 years informant mother place bihar presenting complaints bluish discolouration of lips since 1 month of age breathlessness on exertion since 6 months of age. 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. Transannular patching is used to relieve significant pulmonary annular stenosis during tetralogy of fallot repair. 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. Prenatal and postnatal survival of fetal tetralogy of fallot zhao. In addition, the effects of pr on rv size and function can be serially measured. For the remainder of her pregnancy, kelly went to chop for monthly ultrasounds to monitor her babys heart. The other is a complete repair of the two most important abnormalities that make up tetralogy of fallot. Patients might have one or both surgeries in their. Depending upon the precise nature of the condition, surgery to repair it can include ventricular septal defect vsd closure, removal of muscle from the right ventricle, repair of the pulmonic valve, or pulmonary artery enlargement. When a transannular patch has been inserted, it is usually much less. Cardiovascular mr imaging after surgical correction of 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 term. Ventricular interaction in children after repair of tetralogy of fallot.
Tetralogy of fallot is a heart defect made up of four different heart problems. Current strategy of repair of tetralogy of fallot in children and adults. The transatrial repair, the palliation with a btshunt, the redo surgery and the repair of tofpa with main aortopulmonary collateral arteries mapcas, tofavsd and absent pulmonary valve syndrome are. Tetralogy of fallot is a conotruncal cardiac anomaly with a combination of 4 anatomic heart abnormalities. Prenatal and postnatal survival of fetal tetralogy of fallot. An analysis was performed on the clinical variables, morbidity and mortality. In what im going to call the usual or more typical form of tetralogy of fallot, weve got our ventricular. Anterocephalad deviation of the outlet septum arrow is the key anatomical feature leading to subpulmonary stenosis, ventricular septal defect, aortic override and right ventricular hypertrophy. 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. Management strategies, by peter lang, md, for openpediatrics. The total predicted post repair pa,, without a transannular patch is the sum of the predicted prv,lv in figs 1 and 3. Consecutive 83 patients with repaired tof who did not undergo.
A novel predictive value for the transannular patch. Cardiovascular mr imaging after surgical correction of. The cove point foundation congenital heart resource center is the worlds largest resource for information on pediatric and adult congenital heart disease. Use of a pulmonary neovalve with a transannular patch for. Jun 29, 20 cardiovascular mr imaging after surgical correction of tetralogy of fallot. The study included patients who had complete repair of fallot tetralogy with transannular patch from january 2000 to december 2009. Tetralogy of fallot is a congenital heart defect that is made up of 4 problems and results in not enough blood flow to the lungs. Feb, 2018 tetralogy of fallot is a heart defect made up of four different heart problems. Tetralogy of fallot tof is a congenital heart defect that can be diagnosed either before or after a baby is born. The degree of pulmonary insufficiency after transannular patch repair. Dr bhagirath s n tetralogy of fallot case presentation 2. Cardiac mr imaging assessment following tetralogy of fallot. Our main goal of management of symptomatic neonatal tof is to avoid transannular patch as much as possible, because transannular patch in tof could have an adverse impact on longterm results. Pdf outcomes of prenatally diagnosed tetralogy of fallot.
Image a depicts the underlying anatomical substrate of tetralogy of fallot. Corrective surgery for tetralogy of fallot requires enlargement of the rvot and frequently a transannular patch, which results in pulmonary regurgitation. Low incidence of arrhythmias in the right ventricular infundibulum sparing approach to tetralogy of fallot repair. Tetralogy of fallot with atrioventricular canal defect.
The aim of this study was to detect ventricular arrhythmia incidence and to find out the relationship between ventricular. Tetralogy of fallot video series childrens hospital of. Tetralogy of fallot is the most common form of cyanotic congenital heart disease, and one of the first to be successfully repaired by congenital heart surgeons. Tetralogy of fallot repair assumes vsd closure and relief of pulmonary stenosis at one or more levels, with use of a ventriculotomy incision and placement of a transpulmonary annulus patch. Late homograft valve insertion after transannular patch repair of tetralogy of fallot. The procedure completely relieved recurrent stenosis.
One patient required a transannular patch repair after the initial valvesparing repair. Iorio, gianluca oricchio, roberta iacobelli, antonio amodeo, roberto m. In the smaller picture survival of patients who survived over 30 days after surgery, i. Total repair of tetralogy of fallot radiology reference. Tetralogy of fallot tof with pulmonary stenosis is the common form of tetralogy of fallot, and it is the focus of this article. Openheart surgery is required to repair tetralogy of fallot tof, a complex congenital heart condition.
Ventricular arrhythmia and tetralogy of fallot repair with. Tetralogy of fallot tof is the most common cyanotic congenital heart disease and the population of tof repair survivors is growing rapidly. Effect of transannular patching on outcome after repair of. Longterm followup in adults after tetralogy of fallot repair. Reoperations are no more frequent that with the use of transannular patches. Treatment of fallot tetralogy with a transannular patch. Need of transannular patch in tetralogy of fallot surgery. To assess outcomes of prenatally diagnosed tetralogy of fallot and determine factors associated with the choice to undergo a valvesparing repair versus transannular patch, and the use of. Backgroundin tetralogy of fallot, transannular patching is suspected to be responsible for late right ventricular dilatation methods and resultsin our institution, 191 patients survived a tetralogy of fallot repair between 1964 and 1984. Surgical results of monocusp implantation with transannular patch angioplasty in tetralogy of fallot repair. Complete repair of tetralogy of fallot in the neonate.
Mar 29, 2015 tetralogy of fallot case presentation 1. The blood leaving the heart has less oxygen than is needed by the organs and. Pulmonary valve restitution following transannular patch. This chapter will focus on the surgical transventricular repair of tetralogy of fallot tof, including pulmonary atresia pa without mapcas. Chronic pulmonary insufficiency following transannular patch repair of tetralogy of fallot may mandate restoration of a competent pulmonary valve. Tetralogy of fallot tof is the most common cyanotic congenital heart defect, affecting 3. These problems are treated with medicines, procedures, and surgery. It has been classically characterized by the combination of ventricular septal defect vsd, right ventricular o. Biventricular repair of tetralogy of fallot has been performed for almost four decades, with a favorable longterm outcome in most patients. Tof repair, ventriculotomy, transannular patch 370. Les consequences dune refection epargnant les valves par rapport a une piece transannulaire. Tetralogy of fallot was initially described in 1671 by niels stensen. Transannular patch pericardial unicuspfortotal correction. Tetralogy of fallot tof is a cyanotic congenital cardiac defect that was first.
Pregnancy in repaired tetralogy of fallot thoracic key. Tetralogy of fallot may be diagnosed with fetal echocardiogram ultrasound before your baby is born. Tetralogy of fallot, pediatric cardiac center delaware. 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. Most patients with tetralogy of fallot tof undergo elective surgical repair between 3 and 6 months of age 15. Total repair of tetralogy of fallot requires cardiopulmonary bypass and aortic. But some children with more complex versions may need these additional tests before treatment. A 7yearold girl underwent transannular patching under the mobilized anomalous right coronary artery across the right ventricular outflow to treat progressing infundibular stenosis 6 years after total repair of the tetralogy of fallot. Tetralogy of fallot tof is one of the most common congenital heart. Should patients with transannular patch repair of tetralogy of fallot ha. If the predicted prvjlv is higher than the acceptable value selected by the surgeon, a transannular patch is constructed. Total surgical repair is the only anatomically corrective treatment for. Pediatric and adult interventional cardiac symposium. Tetralogy of fallot tof is a congenital heart defect chd.
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. Right ventricular diastolic function 15 to 35 years after. Teenagers and adults who had surgery to repair tetralogy of fallot may have longterm heart problems, such as heart function problems, arrhythmias irregular heartbeats, or problems resulting from the original repair. Tetralogy of fallot tof is a congenital heart defect. The first anatomic repair of tof was performed in 1954. Simultaneous angiography of the coronary artery and right ventricle were useful in precisely evaluating.
Although recent literature has focused on the deleterious effects of pulmonary regurgitation, inadequate relief of stenosis may increase postoperative mortality and the reintervention rate. Our fetal heart program will then prepare a plan for delivery and care immediately after birth doctors might also make the tof diagnosis before your newborn leaves the hospital if they hear a murmur or see a blue tint to the skin. Tetralogy of fallot is most often diagnosed in the first few weeks of life due to either a loud murmur or cyanosis. The overall outcome after surgical repair of tetralogy of fallot. Tetralogy of fallot tof is the most common cyanotic congenital heart condition with many cases presenting after the newborn period. The condition causes mixing of oxygenpoor blood with the oxygenrich blood being pumped out of the heart and into the circulatory system of blood vessels. Tetralogy of fallot represents 15% of all the congenital heart disease. Outcomes of prenatally diagnosed tetralogy of fallot. A patch across the pulmonary valve annulus a transannular patch is often.
If your baby has pulmonary atresia, a severe obstruction of the pulmonary valve, the surgeon may not be able to remove the obstruction as described above. Echocardiographic assessment after surgical repair of tetralogy of. The incidence of tetralogy of fallot tof among patients with atrioventricular septal defect avsd is estimated to be about 6% to 10%. The ventricular septal defect vsd is also closed with a patch. Enlargement of the rvot, relieving obstructed pulmonary flow. When we repair tetralogy of fallot, there are two major things that we need to do. A novel predictive value for the transannular patch enlargement in repair of tetralogy of fallot.
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. The first surgical repair was carried out in 1944 at johns hopkins. Tetralogy of fallot tof occurs in approximately 1 in 3600 live births and accounts for 3. Significant resection of right ventricular muscle, coupled with pulmonary insufficiency resulting from transannular patch repair can lead to early postoperative hemodynamic instability. Transannular patching was used in 99 patients 52%, patch closure of a right ventriculotomy in 35, and direct closure of a right ventriculotomy in 55. Dec 26, 2015 tetralogy of fallot is the most common congenital heart disease associated with a right aortic arch. Comparison of pulmonary regurgitation and rv size after repair of tetralogy of fallot. 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. A further description was published in 1888 by the french physician etiennelouis arthur fallot, after whom it is named. Tetralogy of fallot is the most common heart defect in children. Lifethreatening arrhythmias and sudden death remain to be serious late complications after correction of tetralogy of fallot. Mitral valve imaging as the road map for successful valve repair feat. 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.
Adults with repaired tof develop late complications. Most kids get definitive repair in 1st year of life. Tetralogy of fallot tof is an important lesion for all pediatric and congenital heart surgeons. An echo is a completely safe and painless test that uses ultrasound sound waves to build a series of pictures of the heart. Tetralogy of fallot, pediatric cardiac center delaware valley. 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. Tetralogy of fallot is a conotruncal defect resulting from anterior malalignment of the infundibular septum. Tetralogy of fallot with pulmonary stenosis treatment. Unedited hd video of tetralogy of fallot repair with a limited transannular patch and transatrial vsd closure in a 5 moth old child.
In older children four years and adults the strategy of repair of tetralogy of fallot tof should have a low rate of transannular patch to avoid pulmonary insufficiency pi, and in addition, we developed a new method of reconstruction of the right ventricular outflow tract rvot to reduce pi. The aim of this study was to describe and analyze longterm followup of patients with repaired tof. The reported success rate for surgical repair of an absent. The most common physical finding for a child or adolescent with repaired tetralogy of fallot tof is a residual heart murmur. Tetralogy of fallot is treated with two kinds of surgery. Cardiac mr imaging assessment following tetralogy of. Echocardiographic assessment after surgical repair of tetralogy of fallot. Pdf echocardiographic assessment after surgical repair of.
Tetrology of fallot is usually diagnosed by echocardiogram ultrasound pictures of the heart. Tetralogy of fallot university of maryland medical system. Jan 01, 2006 describe the surgical procedures used to repair tetralogy of fallot. Ultrasound in obstetrics and gynecology 2007 29 625627. Although transannular patch repair has a good shortterm outcome, there is mounting concern that longstanding severe pulmonary insufficiency may lead to progressive rightsided heart failure, reduced functional status and reduced life expectancy. Current strategy of repair of tetralogy of fallot in. Unedited hd video of tetralogy of fallot repair with a. In 1924, maude abbott coined the term tetralogy of fallot. Tetralogy of fallot is a combination of four related heart defects. Tetralogy of fallot is the most common congenital heart defect. B survival after repair of tetralogy of fallot with or without a transannular patch tap or no tap in different decades. Pulmonary valve leaflets that are preserved at initial surgery may grow and develop normal morphology and subsequent valve repair may be possible. Use of a pulmonary neovalve with a transannular patch for repair. Echocardiography, fetal, prenatal, tetralogy of fallot, valve.