Tetralogy of fallot definition of tetralogy of fallot by. Education in heart congenital heart disease repaired. Most women with repaired tetralogy of fallot can have successful pregnancies. Tetralogy of fallot tof is a severe type of congenital heart disease chd and it. Effect of pregnancy on ventricular and aortic dimensions in. When the baby is born and begins to breathe on its own, the baby turns cyanotic, or blue, due to the deoxygenated blood that bypasses the lungs because the narrowed pathway and the hole between the. Generally, repair includes a patch closure of the vsd and modification of the rv outflow tract to improve pulmonary flow, which can include opening of the pulmonary valve, resection of muscle bundles, andor placement of a transannular patch if.
We would like to refer to the recently published article by luijten et al. An analysis was performed on the clinical variables, morbidity and mortality. The adult with congenital heart disease elizabeth e. Tof repair, ventriculotomy, transannular patch 370. Tetralogy of fallot with pulmonary atresia treatment. 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. 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.
Tetralogy of fallot is estimated to occur in as many as 1 in 3600 live births, making it the most common cyanotic congenital heart disease. When affected babies cry or have a bowel movement, they may develop a tet spell where they turn very blue, have difficulty breathing, become limp, and occasionally lose consciousness. 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. Pulmonic stenosis narrowing of the pulmonary artery. A team of cardiac surgeons performs the surgery, usually before an infant is 1 year old. B survival after repair of tetralogy of fallot with or without a transannular patch tap or no tap in different decades. 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. Complete repair of tetralogy of fallot in the neonate. Repair of this lesion can be approached via either a right atriotomy or a right ventriculotomy, depending on the degree and length of right ventricular outflow tract obstruction.
The mean ratio between the two groups was statistically significant p patch placement in 91 patients undergoing repair of tetralogy of fallot transannular ventriculotomy ii. Pregnancy is considered to be highrisk and not recommended for women with unoperated tetralogy of fallot who remain cyanotic blue. Normally, the left ventricle pumps blood to the body and the right ventricle pumps it to the lungs. Discontinuous left pulmonary artery interrupted inferior vena cava aortopulmonary window. Transventricular repair with a transannular patch a is via a ventricular incision. In a study of pregnancy in women with repaired tetralogy of fallot which included 123 completed pregnancies in two centres, the use of cardiac medications prior to pregnancy was the most important predictor of both maternal cardiac events and of smallforgestationalage babies. Tetralogy of fallot is a common form of congenital heart disease amenable to full surgical repair. Towards the end of pregnancy, visits may be as often as two to three times a week. Tetralogy of fallot is a common syndrome of congenital heart defects. Components of tetralogy of fallot include ventricular septal defect vsd, an aorta that overrides both the left and right ventricles and receives blood from both, pulmonic stenosis valvar, subvalvar, or both, and compensatory right ventricular hypertrophy. Pregnancy outcome in women with congenital heart disease and. In many cases, the repair is made at around 6 months of age, or even a little earlier. Tetralogy of fallot is rare, but it is the most common form of cyanotic congenital heart disease. Many reports have dis cussed the desirability of avoiding transannular patches and the resultant pulmonary regurgita.
The most common complications are symptomatic right heart failure and. Numbers above the curves show the number of living patients 25 and 43 years after corrective operation. Image a depicts the underlying anatomical substrate of tetralogy of fallot. Delivery planning 25 year old female term pregnancy. 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. There is a small risk of heart related complications, such as abnormal fast heart rates medical term. Background pregnancy outcomes in patients with tof are incompletely defined. Upon completion, the surgeon placed a large transannular patch to repair the. This condition, present in utero, is caused by the narrowing of the pulmonary artery and a hole between the ventricles.
Tetralogy of fallot, pediatric cardiac center delaware. The study included patients who had complete repair of fallot tetralogy with transannular patch from january 2000 to december 2009. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of fallot. Tetralogy of fallot results in low oxygenation of blood. Pregnancy in repaired tetralogy of fallot thoracic key.
Other symptoms may include a heart murmur, finger clubbing, and easy tiring upon. Pregnancy, fertility, and recurrence risk in corrected tetralogy of fallot. The optimal timing for tof repair has evolved to balance the benefits of early repair with the risks related to the technical and physiological challenges of neonatal surgery. At the same time, they also reported favourable 25 year survival after tetralogy of fallot tof repair with a transatrial approach, which was great work. Six women 7% with repaired tof at the time of pregnancy reported cardiovascular complications during pregnancy. Women who have favourable anatomy and underwent uncomplicated repair of tetralogy of fallot in childhood can expect low. The plan is to take the infant back to the operating room to perform a complete repair with a transannular patch at 46 months of life. Although recent literature has focused on the deleterious effects of pulmonary regurgitation, inadequate relief of stenosis may increase postoperative mortality and the reintervention rate. Tetralogy of fallot is a heart defect made up of four different heart problems. Surgery for tetralogy of fallot at less than six months of age. In general, targeted anatomy ultrasound at 1823 weeks is the best time to identify tof.
Tetralogy of fallot msd manual professional edition. Tetralogy of fallot tof is a type of heart defect present at birth. Tetralogy of fallot tof is the most common cyanotic congenital heart defect and. Results of 72 respondents, 43 mean age, 26 years had 112 pregnancies range, 1 to 5. Tetralogy of fallot is most often diagnosed in the first few weeks of life due to either a loud murmur or cyanosis. The cove point foundation congenital heart resource center is the worlds largest resource for information on pediatric and adult congenital heart disease. Tetralogy of fallot with pulmonary stenosis differential. For patients who receive a transannular rvot patch as part of the tof repair, the. 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.
Tetralogy of fallot is a combination of four related heart defects. Surgical results of monocusp implantation with transannular patch angioplasty in tetralogy of fallot repair. Anesthesia for the patient with congenital heart disease. Transannular patching is a valid alternative for tetralogy. After the first complete repair, residual problems may require you to have more openheart surgeries or. Tetralogy of fallot congenital heart defects treatment. Objectives we sought to determine pregnancy outcomes in patients with tetralogy of fallot tof. Patient has history of mbt shunt followed by transannular patch for tetralogy of fallot. Total repair of tetralogy of fallot radiology reference. A 29yearold man born with tetralogy of fallot tof underwent repair when he was 9. Tetralogy of fallot causes, symptoms, surgery, treatment.
In symptomatic neonates or infants, i would go ahead with a primary repair provided. Total repair of tetralogy of fallot requires cardiopulmonary bypass and aortic crossclamping. There were seven deaths before surgery and one posttransannular patch repair. Some patients may require earlier correction in the setting of severe cyanosis. Outcomes of prenatally diagnosed tetralogy of fallot. In this video series, you will learn how our experts diagnose tof before birth, monitor babies during pregnancy, plan for delivery and care after. Outcomes of pregnancy in women with tetralogy of fallot. Tetralogy of fallot nord national organization for rare. 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.
Transannular patching is used to relieve significant pulmonary annular stenosis during tetralogy of fallot repair. Tetralogy of fallot university of maryland medical system. Pregnant women with cyanotic congenital heart disease have a mortality. Tetralogy of fallot is treated by surgical repair of the defects. Most women with repaired tetralogy of fallot tolerate pregnancy well. 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. One patient required a transannular patch repair after the initial valvesparing repair.
Comparison of pulmonary regurgitation and rv size after repair of tetralogy of fallot. Tetralogy of fallot is a birth defect of the heart consisting of. Pregnancy in repaired tetralogy of fallot may be associated with arrhythmia, heart failure and low birthweight babies. Pulmonary valve replacement for regurgitation after repair. A total of 42 patients 16 female and 26 male were operated on between july 1, 1974, and january 1, 1998. Need of transannular patch in tetralogy of fallot surgery. This type of heart defect changes the normal flow of blood through the heart. Tetralogy of fallot transannular patch repair, subsequent rvpa homograft conduit iii severe stenosis of left pulmonary artery 4 valvar pulmonary stenosis pulmonary valvotomy iii restrictive rv physiology, rvh twin pregnancy 5 tetralogy of fallot transannular patch repair, subsequent rvpa homograft conduit iii. Tetralogy of fallot tof is a congenital heart defect chd. 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.
Transatrial, transpulmonary tetralogy repair ann thorac surg 1992. One patient required a transannular patch repair after the. Symptoms include episodes of bluish color to the skin. How is surgical treatment of tetralogy of fallot tof. 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. Delayed presentation of tetralogy of fallot with isolated.
A congenital heart defect is a problem with the hearts structure thats present at birth. The risk of maternal cardiac complications depends on the severity of residual lesions at the time of conception. Tetralogy of fallot is composed of a malaligned ventricular septal defect vsd. Echocardiography, fetal, prenatal, tetralogy of fallot, valve sparing. Most patients with tetralogy of fallot tof undergo elective surgical repair between 3 and 6 months of age 15. Type of surgical repair, use of prostaglandins and postnatal death were among the outcomes investigated. The cause of most congenital heart defects is unknown. Tetralogy tehtraloje of fallot fahlo is a congenital heart defect. People with tetralogy of fallot are more likely to also have other congenital defects. Pregnancy outcomes among 31 patients with tetralogy of fallot, a. Transannular patch use is indicated for each age stratum. Pulmonary stenosis congenital heart disease cove point. 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.
The vsd causes cyanosis bluish discoloration of the skin due to lack of oxygen by allowing blood to flow from the right side of the heart to the left side without passing through the lungs. Surgery also ensures that oxygenrich and oxygenpoor blood flow to the right places. Age distribution of 366 patients undergoing transatrial, transpulmonay repair of tetralogy of fallot. Twelve of 29 41% patients received prostaglandins at birth. Management of pulmonary regurgitation after tetralogy of.
Pregnancy in repaired tetralogy of fallot springerlink. Tetralogy of fallot video series childrens hospital of. Twentysix underwent valvesparing repair median age 5. Treatment of fallot tetralogy with a transannular patch. The association of tetralogy of fallot tof with complete atrioventricular septal defect cavsd is rare1, 2. Tetralogy of fallot is a rare, complex heart defect that occurs in about 5 out of every 10,000 babies. A severe case of tof may be diagnosed as early as 12 weeks gestation. 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. Pregnancy outcome in women with congenital heart disease. All pregnant women with or without surgical repair were evaluated by. In tetralogy of fallot, blood may flow across the ventricular septal defect hole, from the right ventricle to the left ventricle, where it is pumped to the rest of the body.
If complications occur, they can usually be treated with medications. Tof is diagnosed with an echocardiogram ultrasound of the heart. Repairing the heart defects will allow oxygenpoor blue blood to travel its normal route through the. Current strategy of repair of tetralogy of fallot in children and adults. The mean interval between tetralogy repair and pulmonary valve replacement was 10. Tetralogy of fallot tof is a congenital heart defect. Echocardiographically guided repair of tetralogy of fallot.
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