In a normal heart, there are two large arteries that carry blood out of the heart. Dloop transposition of the great arteries tga is the second most common form of cyanotic congenital disease 3. Sudden cardiac death is the leading cause of late mortality in patients who have undergone atrial switch repair for d. Characteristics and outcomes of transposition of great arteries in. You wont likely have time to prepare for your first meeting with your babys doctor because it. The aorta and pulmonary artery are reversed, which causes oxygenpoor blood to be circulated to the body and oxygenrich blood to be circulated between the. In this defect, which accounts for 5% of the cases of congenital heart disease, the two main arteries aorta and pulmonary artery are connected to the wrong chambers of the heart. Multimodality imaging guidelines of patients with transposition of the great arteries. The 5th pair of vessels only form in some cases without any known contribution to the final structure of the great arteries. Surgical treatment of patients with transposition of the great arteries tga has evolved rapidly during the last decades. Embryogenesis of transposition of the great arteries circulation.
Transposition of the great vessels tgv is a group of congenital heart defects involving an abnormal spatial arrangement of any of the great vessels. Transposition of the great arteries tga, also referred to as complete transposition, is a congenital cardiac malformation characterised by atrioventricular concordance and ventriculoarterial va. The history of surgery for transposition of great arteries tga has paralleled the history of cardiac surgery. Background little has been published regarding surgery for transposition of the great arteries tga in the developing world. Transposition of the great arteries tga is a common congenital cardiac anomaly and accounts for 45% of all congenital heart disease. Pulmonary artery banding for retraining was performed in 23 cases. Congenitally corrected transposition of the great arteries. Transposition is a switching, oneforone switch, a for b, and b going where a is supposed to go. For babies who have tga tgv, these vessels are transposed switched. Oct, 2008 transposition of the great arteries tga, also referred to as complete transposition, is a congenital cardiac malformation characterised by atrioventricular concordance and ventriculoarterial va discordance.
They are transposed from their normal position so that the aorta arises from the right ventricle and the pulmonary artery from the left ventricle. Transposition of the great arteries childrens hospital. Transposition of the great arteries, dtype congenital. The aorta typically carries oxygenrich blood to the body, while the pulmonary. In children with tga, these arteries are connected to the heart abnormally. A heart in which the two main arteries carrying blood away from the heart are reversed.
Clinical guidelines for the management of patients with. Transposition of the great arteries is a condition in which the connections of the great arteries are reversed so that the aorta arises from the right ventricle and the pulmonary artery from the left ventricle. Transposition of the great arteries tga pediatrics. Having a baby with transposition of the great arteries can be alarming, but with proper treatment, the outlook is promising. Transposition of the great arteries requires corrective surgery in the first weeks of life. The aorta also tends to be on the right and anterior, and the great arteries are parallel rather than crossing as they do in the normal heart. It is not associated with any one common genetic abnormality. Transposition of the great arteries definition transposition of the great arteries tga is a birth defect causing a fatal condition in which there is a reversal, or switch, in the primary connections of the two main great blood vessels to the heart, the aorta and pulmonary artery. Transposition of the great arteries choc childrens. Levo transposition of the great arteries ltga is a rarer condition in which not only the aorta and pulmonary arteries are switched but also the lower chambers of the heart called the ventricles. Dextro transposition of the great arteries dtga occurs when the position of the main pulmonary artery and aorta are switched. Pulmonary hypertension rarely develops in patients with transposition of the great arteries tga who undergo surgical correction with no residual defect. Transposition of the great vessels is a type of congenital heart defect involving an abnormal arrangement of any of the great vessels such as the superior vena cava, pulmonary arteries and aorta. The aorta also tends to be on the right and anterior, and the great arteries are parallel rather than crossing as.
The modified system also can be used in cases with normally related great arteries, cases with single sinus coronary arteries, and cases with bicuspid aortic. A report from the american society of echocardiography developed in collaboration with the society for cardiovascular magnetic resonance and the society of cardiovascular computed tomography. Transposition of the great arteries tga is a cyanotic congenital heart defect in which the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle, creating two parallel circuits. Jan 23, 2016 great artery level arterial switch operation first done by jatene in 1975. Transposition of the great arteries cs mott childrens. Comparison of late results of arterial switch versus atrial switch mustard procedure operation for transposition of the great arteries all marcs picu cardiology foam can be found on picu doctor and can be downloaded as a handy app for free on iphone or android. Congenital heart diseases concerning the primary arteries are further divided into subgroup called transposition of great arteries. In cases of transposition of the great arteries, these vessels arise from the wrong ventricle. Objectives this study sought to identify patient characteristics, surgical interventions, institutional characteristics, risk factors for mortality, and outcomes among patients undergoing surgery for tga in this setting. Transposition of great arteries video khan academy.
The aorta is attached to the rightsided pumping chamber ventricle, instead of the left. In cctga, the morphological left ventricle connects to the right atrium and the pulmonary artery and the right ventricle to the left atrium and the. The incidence is estimated at 1 in 3,5005,000 live. Caudle, daniella transposition of the great arteries or tga is a potentially fatal congenital heart malformation where the pulmonary artery and the aorta are switched. Levo or llooped transposition of the great arteries ltga is a rare form of congenital heart disease characterized by atrioventricular and ventriculoarterial discordance.
Introduction tga most common etiology for cyanotic congenital heart disease in the newborn. Patients were followed up for survival status, morbidity, and reinterventions. To determine the relative position of the aorta and pulmonary artery in conventional chest roentgenograms, a correlative study with large roll film angiocardiograms was made in 70 cases of transposition of the great arteries, including 14 cases of double outlet right ventricle of the transposition. The other one is the aorta that leads to all the arteries in the body. Transposition of the great arteries tga transposition of the great arteries tga is associated with several different arrangements of heart anatomy, so is not one condition.
Case 717 corrected transposition of the great arteries ventricular inversion this 19yearold woman with congenitally corrected transposition of the great vessels was referred for systemic atrioventricular valve replacement for severe regurgitation with worsening systemic ventricular function and progressive heart failure symptoms. Patients must be followed by a cardiologist with expertise in congenital heart disease for the rest of their lives. Transposition of the great arteries tga, also called transposition of the great vessels, is a birth defect involving the two main arteries in the heart being switched. In congenitally corrected transposition of the great arteries cctga, atrioventricular and ventriculoarterial discordance maintains the appropriate direction of blood. Transposition of the great arteries symptoms and causes. Because of this reversal, the aorta carries lowoxygen blood from the right ventricle to the body. Transposition of the great arteries tga, also referred to as complete transposition, is a congenital cardiac malformation characterised by atrioventricular concordance and ventriculoarterial va discordance.
Forty days old infant with transposition of the great arteries and. May 29, 2015 the recurrence risk in firstdegree relatives of patients with transposition of the great arteries tga is low compared to other conotruncal defects. Transposition of great arteries in 1yearold child residencia. Transposition of the great arteries tga is a complex congenital heart defect.
Thus, we sought to determine the longterm outcomes for patients after aso performed at a single institution over a 25year period. Transposition of the great arteries disease reference guide. Early surgery is essential and involves the arterial switch operation, which is carried out in the first week or two of life and corrects the abnormality. To report the case of a pediatric female patient with late diagnosis of transposition of the great arteries associated with atrial septal. Transposition of the great arteries genetic and rare. Transposition of the great arteries is a serious birth defect in which 2 large blood vessels leaving the heart are switched.
Overall annual incidence is 2030 per 100,000 live births, tga is isolated in 90% of patients rarely associated with syndromes or extracardiac. Transposition of the great arteries in the developing world. Leftuntreated,tgaisassociatedwith from the achildrens national heart institute, childrens national medical center, george washington university, washington. Studies on longterm outcomes of the arterial switch operation aso for transposition of the great arteries tga are uncommon. In fact, it began before the birth of open heart surgery when the palliative blalockhanlon septectomy was first performed in 1948. Transposition of the great arteries childrens memorial hermann. Because a baby with this defect may need surgery or other procedures soon after birth, dtga is. Transposition of the great arteries childrens hospital of. Transposition of the great vessels illustration transposition of the great vessels is a congenital heart defect in which the position of the two major vessels that carry blood away from the heart, the aorta and the pulmonary artery, is switched transposed.
Apr 11, 2017 transposition of the great arteries tga is the most common cyanotic congenital heart lesion that presents in neonates. The coronary arteries are transplanted to the pa, and the proximal great arteries are connected to the distal end of the other great artery. Depending on the type of repair performed in childhood, an adult who was born with this condition needs ongoing followup to screen for abnormal blood diversion and irregular heart rhythms and to assess left and right heart function. Transposition of the great arteries tga the embryo. Case 22005 forty days old infant with transposition of the great arteries and ventricular septal defect. Transposition of the great arteries orphanet journal of. Currently, most treated patients live to adulthood, with a 20year survival rate of nearly 90%. Transposition of the great arteries childrens memorial. Transposition of the great arteries tga is the most common cyanotic congenital heart lesion that presents in neonates. Transposition of the great arteries tga transposition of the great arteries or tga is a potentially fatal congenital heart malformation where the pulmonary artery and the aorta are switched. Corrective surgery soon after birth is the usual treatment for transposition of the great arteries. The second type is called the arterial switch operation. In a structurally normal heart, the aorta arises from the left ventricle and the pulmonary artery pa arises from the.
The heart forms during the first 8 weeks of fetal development. The incidence is estimated at 1 in 3,5005,000 live births, with a. Normally, there are two separate blood vessels leaving the heart. Patients with tga and a small vsd undergo aso and vsd repair. Transposition of the great arteries or vessels tga tgv. The incidence is estimated at 1 in 3,5005,000 live births, with a maletofemale ratio 1. Blood coming from the lungs gets sent back to the lungs rather than out to the body. It is also commonly referred to as congenitally corrected tga, double discordance, or ventricular inversion. Longterm outcome in congenitally corrected transposition. Congenital heart disease, transposition of the great arteries, arterial switch operation, atrial switch. Transposition of the great arteries 0 university of chicago. This condition is more common among males, affecting 3 boys to every 1 girl.
The blue blood entering the heart gets pumped directly out to the body without going through the lungs. Transposition of the great arteries in this case, dextro transposition occurs when the aorta arises directly from the right ventricle and the pulmonary artery arises from the left ventricle, resulting in independent, parallel pulmonary and systemic circulations. Clinical guidelines for the management of patients with transposition of the great arteries with intact ventricular septum. Transposition of the great arteries is usually diagnosed in the hospital shortly after birth, leading to urgent consultation with pediatric cardiologists and cardiac surgeons.
The switch means that the aorta, which normally carries. Transposition of the great arteries, dextrolooped 3. Congenital heart diseases involving only the primary arteries pulmonary artery and aorta belong to a subgroup called transposition of the great arteries. When a dtransposition occurs, the blood pathway is impaired because the two arteries are connecting to the wrong chambers in the heart. The two major arteries that carry blood away from the heart the aorta and the pulmonary artery are switched transposed. The hallmark of transposition of the great arteries is ventriculoarterial discordance, in which the aorta arises from the morphologic right ventricle and the pulmonary artery arises from the morphologic left ventricle. Transposition of the great arteries is the second most common congenital heart defect that causes problems in early infancy. Hie multimedia transposition of the great arteries. Background patients with cctga have the anatomical right ventricle as their systemic pumping chamber, with ventricular dysfunction and chf. Aug 11, 2018 transposition of the great arteries is usually detected either prenatally or within the first hours to weeks of life. The number of subsequent residual heart defects was low. Dextrotransposition of the great arteries or dtga is a birth defect of the heart in which the two main arteries carrying blood out of the heart the main pulmonary artery and the aorta are switched in position, or transposed.
The task force on transposition of the great arteries of the european association for cardiothoracic surgery eacts and the association for european paediatric and congenital cardiology aepc. Transposition of the great arteries tga is a pediatric cardiac congenital defect arising from an embryological discordance between the aorta. Transposition of the great arteries is usually detected either prenatally or within the first hours to weeks of life. Cardiac and aortic arch anomalies in offspring of vitamin a deficient rats correlated with. Since the overall survival after atrial and arterial switch operation has improved dramatically, the focus has shifted from mortality to longterm morbidity and quality of. The coronary arteries are replanted at the neoaortic root. Anatomic repair for congenitally corrected transposition of the great.
Longterm morbidity and quality of life after surgical. Objectives the purpose of this study was to determine longterm outcome in adults with congenitally corrected transposition of the great arteries cctga, with particular emphasis on systemic ventricular dysfunction and congestive heart failure chf. Patients must be followed by a cardiologist with expertise in. Late development of pulmonary arterial hypertension after arterial. The pulmonary artery leaves the left ventricle, and the aorta leaves the right ventricle. Emergency procedures are often needed to improve your babys oxygen level. Pdf historical evolution of surgery for transposition of. Complete transposition of the great arteries is also known as dtga.
Scribd is the worlds largest social reading and publishing site. Jun 27, 2011 transposition of the great arteries tga is a type of congenital heart defect in which there is a reversal of the normal connections of the aorta and the pulmonary artery with the heart. Its also called the mustard procedure or the senning procedure. Transposition of the great arteries tga is a heart defect that occurs from birth congenital. Congenitally corrected transposition of the great arteries cctga is characterized by atrioventricular and ventriculoarterial discordance, with an incidence of 0. So the two great arteries that come out of the heart are the pulmonary artery, which leads to the lungs, and the aorta oops, its not artery, its arteries. Arterial switch operation for transposition of the great arteries with. The right 6th almost completely regresses with only the proximal part contributing to the base of the pulmonary arteries, while the left 6th forms the ductus arteriosus, which disappears after birth. The current approach to surgical correction is the arterial switch procedure, which results in anatomically normal circulation. Tga occurs in about 3% of all congenital heart defects. The morphological right ventricle rv supports the systemic circulation and the morphological tricuspid valve is the systemic atrioventricular valve savv 1. Transposition of the great arteries statpearls ncbi bookshelf.
Overall annual incidence is 2030 per 100,000 live births, tga is isolated in 90% of patients rarely associated with syndromes or extracardiac malformations. Objective this study assessed adult survival and morbidity patterns in patients who underwent atrial correction according to mustard or senning for transposition of the great arteries tga. The great arteries in this anomaly refer to the aorta and the pulmonary artery, the two major arteries carrying blood away from the heart. Transposition of the great arteries tga was first discussed by matthew baillie in 1797 in the morbid anatomy of some of the most important parts of the human body. Other heart defects may occur along with transposition of the great arteries. In babies with this birth defect, the pulmonary artery and aorta are reversed. The small coronary arteries, which feed the heart muscle with blood, need to be transferred as well as the two great arteries aorta and pulmonary artery. Methods in 76 adult patients with tga 59% male after atrial correction, longterm survival and morbidity were investigated in three periods. Complete transposition of the great arteries toga occurs when the aorta, which normally comes off the left ventricle and pumps red blood to the body, arises from the right ventricle1 and pumps blue blood returning from the body back to the body bypassing the lungs completely.
23 273 1390 808 369 325 300 1047 328 672 1414 264 27 239 1070 5 1221 470 508 749 1218 353 716 1488 435 634 1442 805