Transannular patching is a valid alternative for tetralogy of fallot and. Therefore, whether to repair tetralogy of fallot is not often debated. One is that there is a large hole in the wall of muscle septum that divides the 2 bottom pumping chambers ventricles of the heart. Pdf ventricular arrhythmia and tetralogy of fallot. Although the prognosis for patients who have undergone surgical repair is favorable early mortality rate prognosis improved considerably over time. Further, the need of a transannular patch was associated with a higher risk of reoperation, but had no impact on late survival. Tetralogy of fallot tof is a congenital heart defect. Backgroundin tetralogy of fallot, transannular patching is. We report our experience with repair of tetralogy of fallot associated. It is known, however, to have longterm detrimental effects, including exercise intolerance, progressive right ventricular rv dilatation and dysfunction, ventricular and. Surgical repair of tetralogy of fallot with complete atrioventricular canal. C, d systolic thickening and inward movement of the rvot in a patient without patch repair white arrows. Right ventricular function in adults with repaired.
Repair of tetralogy of fallot in neonates and young infants. Cardiac mr imaging assessment following tetralogy of. Need of transannular patch in tetralogy of fallot surgery carries a higher. 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. Primary repair of tetralogy of fallot predicts a lower mortality rate and longer freedom from reoperation when compared with twostage repair. 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. 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. Surgery to repair tetralogy of fallot improves blood flow to the lungs. Tetralogy of fallot the patient guide to heart, lung, and. Longterm outcome in patients undergoing surgical repair of.
Cardiovascular mr imaging after surgical correction of. Tetralogy of fallot causes, symptoms, surgery, treatment. Transesophageal echocardiography is utilized to help assess adequacy of repair. 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. People with tetralogy of fallot are more likely to also have other congenital defects. Longterm survival of patients with tetralogy of fallot with or without a patch up to or through the pulmonary annulus who survived the immediate postoperative period.
Pdf ventricular arrhythmia and tetralogy of fallot repair. The prognosis of patients with unrepaired tetralogy of fallot tof is inferior to the life expectancy of those undergoing repair. Pulmonary stenosis congenital heart disease cove point. Primary repair of tetralogy of fallot predicts a lower mortality rate and longer. Transatrialtranspulmonary repair of tof included closure. Tetralogy of fallot tof childrens hospital of philadelphia. Tetralogy of fallot is the most common heart defect in children. Survival after repair is excellent, but data 20 years are rare. Transannular patching is a valid alternative for tetralogy. Ventricular arrhythmia and tetralogy of fallot repair with transannular patch article pdf available in anadolu kardiyoloji dergisi. Tetralogy of fallot with pulmonary stenosis treatment. Right ventricular outflow tract obstruction and pulmonary insufficiency occur which require reintervention. Primary repair of fallot tetralogy has been performed successfully for the last 45.
Some degree of right ventricular failure is nearly universal following repair of tetralogy of fallot. Repair included transannular patch 61 tofps, 9 tofpa. Physical examination may be deceptive after classic tetralogy repair, with severe pulmonary valve regurgitation often present if a transannular patch was used. 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%. Jul 31, 2016 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. The most common signs and symptoms seen for a newborn with tetralogy of. In some repairs, the patch may extend across the pulmonary valve annulus transannular patch, making the. 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. One study of survivors of the first year after surgical repair showed actuarial survival rates of 97% at 10 years, 94% at 20 years, 89% at 30 years, and 85% at 36 years. When we repair tetralogy of fallot, there are two major things that we need to do. Transannular patch repair was associated with significantly lower reinterventionfree survival median 32. Tetralogy of fallot tof, the most common congenital heart defect associated with cyanosis and pulmonary undercirculation, accounts for approximately 6%10% of all cases of congenital heart disease, 1, 2.
Following adequate rewarming, the patient is weaned from cardiopulmonary bypass. The data are also relevant to the recent upsurge of interest in using semi lunar valve allografts rather than transannular patches when the pulmonary annulus is hypoplastic. Oct 18, 2016 most of these adult congenital heart disease patients underwent tetralogy of fallot repair in childhood. Need of transannular patch in tetralogy of fallot surgery carries a higher risk of reoperation but has no impact on late survival.
Transannular patching is used to relieve significant pulmonary annular stenosis during tetralogy of fallot repair. Prfree survival than those with a transannular patch 8. For patients who receive a transannular rvot patch as part of the tof repair, the. Use of a transannular patch does not reduce late survival and is associated with a lower incidence of right ventricular outflow tract obstruction. 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. Use of a pulmonary neovalve with a transannular patch for repair. Transannular patch repair was the commonest operation among patients born after 1985 43. 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. Nollert g, fischlein t, bouterwek s, bohmer c, klinner w, reichart b. Outcomesresolutions in most cases, tetralogy of fallot can be treated with surgery. 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.
The problems related with primary repair for tetralogy of. Tetralogy of fallot the patient guide to heart, lung. Longterm outcome in patients undergoing surgical repair. This rare lesion represents only 2% of all cases of tetralogy of fallot. The anterior wall of the rvot is opened through the annulus of the pulmonary valve. Adverse events were prospectively defined as the above andor endocarditis, documented atrial arrhythmia andor a need for permanent pacemaker or defibrillator implantation. One patient required a transannular patch repair after the initial valvesparing repair. From 1958 to 1977, 658 patients underwent correction of tof at our institution and were analyzed for survival. Outcomes of prenatally diagnosed tetralogy of fallot.
In some cases, depending on the unique needs of the patient, we will perform a temporary repair until a complete repair can be done. The present study evaluated the efficacy of reoperation following complete correction of tetralogy of fallot, the sites of recurrences and impact of techniques used at first operation. Tetralogy of fallot with common av canal av septal defect avsd is the presence of both tetralogy of fallot and complete avsd. Ppvi after repair of tet fallot with transannular patch for 12 yo patient. Pdf need of transannular patch in tetralogy of fallot. Early mortality was 3% 3 of 99, and actuarial survival rates were 94% at 1. 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. The outcome for patients with tetralogy of fallot has improved dramatically since the introduction of surgical repair.
Some patients may require earlier correction in the setting of severe cyanosis. According to the american heart association, congenital heart defects present at birth are the most common type of birth defect. Surgery also ensures that oxygenrich and oxygenpoor blood flow to the right places. 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. Most patients with tetralogy of fallot tof undergo elective surgical repair between 3 and 6 months of age 15.
Transannular patching is a valid alternative for tetralogy of. If a patch is inserted, it may be used to widen the pulmonary artery from the valve upward. The data indicate an increased risk of death in patients who had undergone a primary palliative procedure. Transannular patch repair is performed when there is marked stenosis of the rvot or pulmonary annulus. This patch covers part of the wall of the right ventricle as well as widening the pulmonary artery and pulmonary valve. Simultaneous angiography of the coronary artery and right ventricle were useful in precisely evaluating. Jun 29, 20 transannular patch repair is performed when there is marked stenosis of the rvot or pulmonary annulus. Transannular patching was used in 99 patients 52%, patch closure of a right ventriculotomy in 35. Should patients with transannular patch repair of tetralogy of fallot ha. Although recent literature has focused on the deleterious effects of pulmonary regurgitation, inadequate relief of stenosis may increase postoperative mortality and the reintervention rate. Type of surgical repair, use of prostaglandins and postnatal death were among the outcomes investigated. Mar 30, 2017 the prognosis of patients with unrepaired tetralogy of fallot tof is inferior to the life expectancy of those undergoing repair.
Right ventricular function in adults with repaired tetralogy. Tetralogy of fallot video series childrens hospital of. 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. Complete repair usually performed in the first year of life. Tetralogy of fallot is rare, but it is the most common form of cyanotic congenital heart disease. Backgroundthe timing of repair of tetralogy of fallot tof remains controversial. Longterm survival in patients with repair of tetralogy of. Should patients with transannular patch repair of tetralogy. Longterm survival in patients with repair of tetralogy of fallot. Effect of transannular patching on outcome after repair of. Prenatally diagnosed tetralogy of fallot has a good outcome with low short and midterm morbidity and mortality following valvesparing repair or transannular patch repair in fetuses and live born infants who are otherwise normal.
The blood leaving the heart has less oxygen than is needed by the organs and. Clinical outcomes of surgical pulmonary valve replacement. Significant resection of right ventricular muscle, coupled with pulmonary insufficiency resulting from transannular patch repair can lead to early postoperative hemodynamic instability. A patch across the pulmonary valve annulus a transannular patch is often required in order to adequately relieve right ventricular outflow tract obstruction. Comparison of pulmonary regurgitation and rv size after repair of tetralogy of fallot. Need of transannular patch in tetralogy of fallot surgery. The cause of most congenital heart defects is unknown. Most adult patients with tetralogy of fallot have been operated on, usually with an rv outflow patch and vsd closure. Tetralogy of fallot tof is an important lesion for all pediatric and congenital heart surgeons. Pulmonary valve replacement in adult congenital cardiac surgery.
A congenital heart defect is a problem with the hearts structure thats present at birth. The association of tetralogy of fallot tof with complete atrioventricular septal defect cavsd is rare1, 2. Pulmonary valve replacement pvr was not included as an adverse event but, rather, as an inevitable consequence of early transannular patchtype repair of tetralogy. Backgroundin tetralogy of fallot, transannular patching is suspected to be responsible for late right ventricular dilatation. 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. Tof is described as 4 abnormalities that occur together. Patients with genetic syndromes had significantly lower overall survival after repair. Chd longterm results after early primary repair of tetralogy of fallot. Four patients had previously undergone palliative treatment with a ptfe. Tetralogy tehtraloje of fallot fahlo is a congenital heart defect. The study included patients who had complete repair of fallot tetralogy with transannular patch from january 2000 to december 2009.
Treatment of fallot tetralogy with a transannular patch. 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. Twelve of 29 41% patients received prostaglandins at birth. Results in the tetralogy of fallot group, 24 out of 57 patients underwent primary repair with a transannular patch. An analysis was performed on the clinical variables, morbidity and mortality. Pulmonary effects on exercise testing in tetralogy of. Complete correction of tetralogy of fallot has good longterm results. 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. You may need to limit your activity if there is leftover obstruction or a pulmonary valve leak, which is common after repair. As a result, i got hold of the most interesting ecg i have recorded to date.
However, if there is also a need to widen the outflow tract of the right ventricle, a transannular patch may be required. 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. Total repair of tetralogy of fallot radiology reference. Tetralogy of fallot, survival, transannular patch, late results. Tetralogy of fallot is the most common congenital heart defect. If surgeries are performed in babies, then more than 90% survive to adulthood and lead healthy lives. 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. In a case of tetralogy of fallot, blood flow to the lungs and the body is affected.
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. The longterm prognosis of surgically corrected tof patients is good and has improved with each decade since the beginning of tof surgery in finland. A pericardial or synthetic polytetrafluoroethylene goretex. The timing of surgery and the initial surgical procedure performed in the patient with symptomatic tetralogy of fallot remain controversial. Ylitalo p1, nieminen h1, pitkanen om1, jokinen e1, sairanen h2. Pulmonary effects on exercise testing in tetralogy of fallot. This type of heart defect changes the normal flow of blood through the heart. Complete surgical repair of this lesion is riskier than repair of tetralogy of fallot or avsd alone. Results of total correction of tetralogy of fallot with complete atrioventricular canal. Cardiac surgeons perform an open heart surgery during a childs first year to treat tetralogy of fallot. The mortality rate is high among cases with chromosomal andor extracardiac anomalies.
We sought to analyze risk factors for longterm survival up to 36 years after surgical repair of tetralogy of fallot tof. Tetralogy of fallot patients repaired with a transannular patch without pulmonary valve replacement were compared with age, gender, and sizematched normal controls. Tetralogy of fallot is a rare, complex heart defect that occurs in about 5 out of every 10,000 babies. Adult patients with tetralogy of fallot who undergo surgery again are usually symptom free for 1015 years, but by the time they reach their fifth decade, even these patients begin to have symptoms. How to diminish reoperation rates after initial repair of. 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. Gore, newark, del patch is then sutured to both margins of the incision, increasing the. Methods and resultsin our institution, 191 patients survived a tetralogy of fallot repair between 1964 and 1984. Transannular patching for tetralogy of fallot with an. Involves closure of the vsd and resection of the infundibular stenosis, with placement of a pericardial patch to enlarge the rvot. Ppvi after repair of tet fallot with transannular patch. The procedure completely relieved recurrent stenosis. Tetralogy of fallot in the current era seminars in thoracic and. Tetralogy of fallot is most often diagnosed in the first few weeks of life due to either a loud murmur or cyanosis.