Tetralogy of fallot congenital heart defects treatment. Tetralogy tehtraloje of fallot fahlo is a congenital heart defect. Delayed presentation of tetralogy of fallot with isolated. Pregnancy is considered to be highrisk and not recommended for women with unoperated tetralogy of fallot who remain cyanotic blue. In symptomatic neonates or infants, i would go ahead with a primary repair provided. Tetralogy of fallot is most often diagnosed in the first few weeks of life due to either a loud murmur or cyanosis. Tof is diagnosed with an echocardiogram ultrasound of the heart. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of fallot. The mean interval between tetralogy repair and pulmonary valve replacement was 10. 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. The most common complications are symptomatic right heart failure and. 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. Numbers above the curves show the number of living patients 25 and 43 years after corrective operation.
In this video series, you will learn how our experts diagnose tof before birth, monitor babies during pregnancy, plan for delivery and care after. Type of surgical repair, use of prostaglandins and postnatal death were among the outcomes investigated. Repairing the heart defects will allow oxygenpoor blue blood to travel its normal route through the. 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. Comparison of pulmonary regurgitation and rv size after repair of tetralogy of fallot. 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. Treatment of fallot tetralogy with a transannular patch.
Tetralogy of fallot msd manual professional edition. 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. Age distribution of 366 patients undergoing transatrial, transpulmonay repair of 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. Transatrial, transpulmonary tetralogy repair ann thorac surg 1992. Six women 7% with repaired tof at the time of pregnancy reported cardiovascular complications during pregnancy. Twelve of 29 41% patients received prostaglandins at birth. An analysis was performed on the clinical variables, morbidity and mortality. Most women with repaired tetralogy of fallot tolerate pregnancy well. 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. The cove point foundation congenital heart resource center is the worlds largest resource for information on pediatric and adult congenital heart disease.
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. Most patients with tetralogy of fallot tof undergo elective surgical repair between 3 and 6 months of age 15. Tetralogy of fallot is composed of a malaligned ventricular septal defect vsd. Tetralogy of fallot tof is the most common cyanotic congenital heart defect and. Twentysix underwent valvesparing repair median age 5. 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 outcomes among 31 patients with tetralogy of fallot, a. Education in heart congenital heart disease repaired. Tetralogy of fallot is a common form of congenital heart disease amenable to full surgical repair. A 29yearold man born with tetralogy of fallot tof underwent repair when he was 9. Tetralogy of fallot, pediatric cardiac center delaware. Surgery for tetralogy of fallot at less than six months of age. Complete repair of tetralogy of fallot in the neonate.
Need of transannular patch in tetralogy of fallot surgery. Tetralogy of fallot is a birth defect of the heart consisting of. Results of 72 respondents, 43 mean age, 26 years had 112 pregnancies range, 1 to 5. Image a depicts the underlying anatomical substrate of tetralogy of fallot. Pregnancy outcome in women with congenital heart disease. The association of tetralogy of fallot tof with complete atrioventricular septal defect cavsd is rare1, 2. All pregnant women with or without surgical repair were evaluated by. 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. A total of 42 patients 16 female and 26 male were operated on between july 1, 1974, and january 1, 1998. Background pregnancy outcomes in patients with tof are incompletely defined. Tetralogy of fallot is a combination of four related heart defects. Outcomes of prenatally diagnosed tetralogy of fallot. Normally, the left ventricle pumps blood to the body and the right ventricle pumps it to the lungs. 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.
Surgery to repair tetralogy of fallot improves blood flow to the lungs. Symptoms include episodes of bluish color to the skin. Tetralogy of fallot results in low oxygenation of blood. Anesthesia for the patient with congenital heart disease. Pulmonary stenosis congenital heart disease cove point.
Transannular patching is used to relieve significant pulmonary annular stenosis during tetralogy of fallot repair. Effect of pregnancy on ventricular and aortic dimensions in. Tof repair, ventriculotomy, transannular patch 370. The study included patients who had complete repair of fallot tetralogy with transannular patch from january 2000 to december 2009.
Initial reparative surgery characteristics of 43 women with tetralogy of fallot who had pregnancies characteristics n 43 age at surgery, yrs 14. In many cases, the repair is made at around 6 months of age, or even a little earlier. Pulmonic stenosis narrowing of the pulmonary artery. Towards the end of pregnancy, visits may be as often as two to three times a week. Delivery planning 25 year old female term pregnancy. The cause of most congenital heart defects is unknown. Surgery also ensures that oxygenrich and oxygenpoor blood flow to the right places. The adult with congenital heart disease elizabeth e. 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. 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. 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. Tetralogy of fallot with pulmonary stenosis differential. Pregnancy, fertility, and recurrence risk in corrected tetralogy of fallot. Many reports have dis cussed the desirability of avoiding transannular patches and the resultant pulmonary regurgita.
Longterm outcomes after repair of congenital heart. One patient required a transannular patch repair after the. Transannular patching is a valid alternative for tetralogy. Most women with repaired tetralogy of fallot can have successful pregnancies.
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. A team of cardiac surgeons performs the surgery, usually before an infant is 1 year old. This condition, present in utero, is caused by the narrowing of the pulmonary artery and a hole between the ventricles. Upon completion, the surgeon placed a large transannular patch to repair the. Discontinuous left pulmonary artery interrupted inferior vena cava aortopulmonary window. After the first complete repair, residual problems may require you to have more openheart surgeries or. Fortysix pregnancies were singletons, and one was a twin pregnancy in. There is a small risk of heart related complications, such as abnormal fast heart rates medical term.
Other symptoms may include a heart murmur, finger clubbing, and easy tiring upon. Tetralogy of fallot with pulmonary atresia treatment. Some patients may require earlier correction in the setting of severe cyanosis. Pregnant women with cyanotic congenital heart disease have a mortality.
Tetralogy of fallot tof is a severe type of congenital heart disease chd and it. Transventricular repair with a transannular patch a is via a ventricular incision. Tetralogy of fallot is a heart defect made up of four different heart problems. Surgical results of monocusp implantation with transannular patch angioplasty in tetralogy of fallot repair. Tetralogy of fallot is a common syndrome of congenital heart defects. 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. Tetralogy of fallot video series childrens hospital of. 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. Outcomes of pregnancy in women with tetralogy of fallot. A severe case of tof may be diagnosed as early as 12 weeks gestation. There were seven deaths before surgery and one posttransannular patch repair.
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. Tetralogy of fallot is a rare, complex heart defect that occurs in about 5 out of every 10,000 babies. Total repair of tetralogy of fallot radiology reference. Tetralogy of fallot tof is a type of heart defect present at birth. 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 tof is a congenital heart defect. Tetralogy of fallot causes, symptoms, surgery, 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. Echocardiographically guided repair of tetralogy of fallot. Objectives we sought to determine pregnancy outcomes in patients with tetralogy of fallot tof. 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. One patient required a transannular patch repair after the initial valvesparing repair. B survival after repair of tetralogy of fallot with or without a transannular patch tap or no tap in different decades.
Pulmonary valve replacement for regurgitation after repair. Patient has history of mbt shunt followed by transannular patch for tetralogy of fallot. Tetralogy of fallot tof is a congenital heart defect chd. Although recent literature has focused on the deleterious effects of pulmonary regurgitation, inadequate relief of stenosis may increase postoperative mortality and the reintervention rate. How is surgical treatment of tetralogy of fallot tof. Pregnancy in repaired tetralogy of fallot thoracic key. N in legend is the number of patients operated during the era in each group. 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 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 nord national organization for rare. People with tetralogy of fallot are more likely to also have other congenital defects. Total repair of tetralogy of fallot requires cardiopulmonary bypass and aortic crossclamping. Tetralogy of fallot is rare, but it is the most common form of cyanotic congenital heart disease. Pregnancy outcome in women with congenital heart disease and. The risk of maternal cardiac complications depends on the severity of residual lesions at the time of conception. Tetralogy of fallot definition of tetralogy of fallot by. Current strategy of repair of tetralogy of fallot in children and adults.
If complications occur, they can usually be treated with medications. Tetralogy of fallot university of maryland medical system. Pregnancy in repaired tetralogy of fallot springerlink. Women who have favourable anatomy and underwent uncomplicated repair of tetralogy of fallot in childhood can expect low. Transannular patch use is indicated for each age stratum. 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. In general, targeted anatomy ultrasound at 1823 weeks is the best time to identify tof. We would like to refer to the recently published article by luijten et al. This type of heart defect changes the normal flow of blood through the heart. Management of pulmonary regurgitation after tetralogy of. 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. Pregnancy in repaired tetralogy of fallot may be associated with arrhythmia, heart failure and low birthweight babies. Tetralogy of fallot is treated by surgical repair of the defects.
A congenital heart defect is a problem with the hearts structure thats present at birth. For patients who receive a transannular rvot patch as part of the tof repair, the. 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. Echocardiography, fetal, prenatal, tetralogy of fallot, valve sparing.
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