In transposition of the great arteries, the aorta is connected to the right ventricle, and the pulmonary artery is connected to the left ventricle the exact opposite of a normal hearts anatomy. Apr 11, 2017 transposition of the great arteries tga is the most common cyanotic congenital heart lesion that presents in neonates. Transposition of the great arteries radiology reference. Levo or llooped transposition of the great arteries ltga is a rare form of congenital heart disease characterized by atrioventricular and ventriculoarterial discordance. For the success of anatomical correction of transposition of the great arteries tga it is essential to transfer the coronary ostia to the posterior vessel without undue tension, torsion, or kinking of the proximal coronary arteries or their early branches. Transposition of the great arteries is the second most common congenital heart defect that causes problems in early infancy. The adult congenital and pediatric cardiology council of the american college of cardiology. Transposition of the great arteries tga constitutes 5% of all cyanotic congenital heart defects chds and 10% of all neonatal cyanotic chds 1 and is the most common cyanotic chd in the neonate. In transposition of the great arteries tga, the hearts two major arteries are reversed. Transposition of the great arteries 0 university of chicago. 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 the great arteries symptoms and causes. Tga may occur as an isolated defect or in association with other cardiac anomalies.
Cardiac surgery, kirklin jw, barrattboyes bg eds, churchill livingston, new york 1993. 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 tga the embryo. 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. Transposition of the great arteries choc childrens.
Transposition of the great arteries childrens memorial. The switch means that the aorta, which normally carries oxygenated blood, carries deoxygenated blood. Transposition of the great arteries childrens memorial hermann. 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. Transposition of the great arteries is usually diagnosed in the hospital shortly after birth, leading to urgent consultation with pediatric cardiologists and cardiac surgeons. Complete transposition of the great arteries request pdf. 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. First medically described by matthew baillie during the year 1797, transposition of the great arteries tga is a congenital heart defect, and it affects two vital vessels which carry the blood away from the heart. Read on for information about the characteristics of the defect at birth and the treatment and followup care that is necessary into adulthood. Transposition of the great arteries tga also known as transposition of the great vessels tgv is the most common cyanotic congenital cardiac anomal y presenting during the newborn period, with cyanosis in the first 24 hours of life.
In rare cases, even the chambers of the heart will be swapped. Transposition of the great arteries requires corrective surgery in the first weeks of life. A heart in which the two main arteries carrying blood away from the heart are reversed. Transposition of the great arteries orphanet journal of. 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. Due to the wedged position of the subpulmonary outflow tract in the morphologically left ventricle, there is gross malalignment between the atrial septum and the inlet. Until aroud 1980 this delicate surgery could not be performed safely and an alternative procedure was used, redirecting blood within the atriums. Transposition of the great arteries cs mott childrens. Emergency procedures are often needed to improve your babys oxygen level. Learn for free about math, art, computer programming, economics, physics, chemistry, biology, medicine, finance, history, and more. An image on an egg on a string is superimposed on this chest radiograph to clairfy the appearance of the egg on a string sign of transposition of the great arteries, with enlargement of the cardiac silhouette and atrophy of the thymus leading to narrowing of the upper mediastinum. Transposition of great arteries tga radiology case.
The aorta is located anteriorly and to the right, and the great arteries run in parallel instead of in their normal perpendicular relation. Congenitally corrected transposition of the great arteries. Khan academy is a nonprofit with the mission of providing a free, worldclass education for anyone, anywhere. Transposition of the great arteries tga is a complex congenital heart defect. Tga occurs in 5 to 7 percent of all congenital heart defects.
Facts about dextrotransposition of the great arteries dtga. The aorta is attached to the rightsided pumping chamber ventricle, instead of the left. Jan 23, 2016 transposition of great arteries dr amarja sachin nagre md,dm,fca. When a d transposition occurs, the blood pathway is impaired because the two arteries are connecting to the wrong chambers in the heart. 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. The incidence is estimated at 1 in 3,5005,000 live births, with a maletofemale ratio 1.
Leftuntreated,tgaisassociatedwith from the achildrens national heart institute, childrens national medical center, george washington university, washington. It is also commonly referred to as congenitally corrected tga, double discordance, or ventricular inversion. Tga is a congenital heart disease in which the hearts largest arteries, the aorta and the pulmonary arteries, are switched. Transposition of the great arteries tga, also referred to as complete transposition, is a congenital cardiac malformation characterised by atrioventricular concordance and ventriculoarterial va. Between, may of 1973 and may of 1978, patients underwent correction of complete transposition of the great arteries by a modification of the mustard operation specifically designed to avoid. Transposition of the great arteries tga pediatrics. The arterial switch operation aso has replaced atrial switch procedures for dtga, and 90% of patients now reach adulthood.
Transposition of great arteries dr amarja sachin nagre md,dm,fca. It accounts for up to 7% of all congenital cardiac anomalies 1 and can be assessed with echocardiography. Transposition of the great arteries or tga is a potentially fatal congenital heart malformation where the pulmonary artery and the aorta are switched. The aorta is connected to the right ventricle, the pulmonary artery to the left ventricle. 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. Introduction tga most common etiology for cyanotic congenital heart disease in the newborn. Dextro transposition of the great arteries dtga occurs when the position of the main pulmonary artery and aorta are switched. Oxygenpoor blue blood returns to the right atrium from the body, passes through the right atrium and ventricle, then goes into the misconnected. The aorta also tends to be on the right and anterior, and the great arteries are parallel rather than crossing as they do in. Blood coming from the lungs gets sent back to the lungs rather than out to the body. In 50% of cases, the va discordance is an isolated finding. Transposition of the great arteries diagnosis and treatment. All patients with transposition of the great arteries require lifelong surveillance by a congenital heart expert.
A ventricular septal defect is detected in at least 50% of patients. Transposition of the great arteries tga is a group of congenital defects in which the position of major blood vessels of the heart are switched. Dloop transposition of the great arteries tga is the second most common form of cyanotic congenital disease 3. Dtransposition of the great arteries henceforth referred to simply as transposition or tga represents 57 % of congenital heart disease and is the second most common cyanotic defect after tetralogy of fallot 1.
If they have had a mustard or senning procedure, the issues have to do with slow heart rates, abnormally fast heart rates, pumping chamber weakness, leaky valves, and technical issues related to the surgical repair. Transposition of the great arteries tga was first recognized and described more than 2 centuries ago. Corrected transposition of the great arteries 359 anatomical characteristics and associated lesions notably, in most cases, the interventicular septum has a sagittal orientation with the ventricles at both sides in hearts that tend to mesocardia within the thorax fig. Transposition of the great arteries in this case, dextrotransposition 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. Transposition of the great arteries tga heart defect. Complete transposition of the great arteries is also known as dtga. Situs solitus or inversus, circ cardiovasc imaging 75. 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. Subxiphoid twodimensional echocardiographic identification of tricuspid valve abnormalities in transposition of the great arteries with ventricular septal defect. The heart forms during the first 8 weeks of fetal development. D transposition of the great arteries is a form of congenital heart disease that must be corrected shortly after birth for survival. This complex always has discordant ventriculoarterial alignment, such that the aorta arises entirely or. Levo transposition of the great arteries is an acyanotic heart defect in which the primary arteries are transposed, with the aorta anterior and to the left of the pulmonary artery, and the morphological left and right ventricles with their corresponding atrioventricular valves are also transposed.
In children with tga, these arteries are connected to the heart abnormally. This paper aims to update clinicians on hot topics in the management of patients with dloop transposition of the great arteries dtga in the current surgical era. Transposition of great arteries linkedin slideshare. 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. In the majority of patients with tga, the aorta is rightward and anterior to the pulmonary artery, but there can be wide variability in the spatial relationships between the great arteries. Because a baby with this defect may need surgery or other procedures soon after birth, dtga is. 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. Anatomy of the coronary arteries in transposition of the. 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 great arteries video khan academy. 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. Transposition of the great arteries description and epidemiology 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. In a normal heart, there are two large arteries that carry blood out of the heart.
Explaining transposition of the great arteries 1 of 5. Otto md, in intraoperative and interventional echocardiography second edition, 2018. Corrective surgery soon after birth is the usual treatment for transposition of the great arteries. 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. 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. In cases of transposition of the great arteries aorta and pulmonary artery, these vessels are transposed from their normal position so that the aorta arises from the right ventricle and the. Dtransposition of the great arteries is a form of congenital heart disease that must be corrected shortly after birth for survival. Complete transposition of the great arteries is the most common form of neonatal cyanotic heart disease. Transposition of the great arteries disease reference guide. Transposition of the great vessels tgv is a group of congenital heart defects involving an abnormal spatial arrangement of any of the great vessels. The blue blood entering the heart gets pumped directly out to the body without going through the lungs. Aug 05, 2014 this paper aims to update clinicians on hot topics in the management of patients with dloop transposition of the great arteries dtga in the current surgical era. The transposition of the great arteries was first described by mathew baillie in 1797, in the second edition of the book the morbid anatomy of some of the most important parts of the human body. Having a baby with transposition of the great arteries can be alarming, but with proper treatment, the outlook is promising.
Although reference was made to malposition of the aorta and pulmonary artery by steno in 1672 and morgagni in 1761, the anatomic description of tga is credited to baillie in 1797. Tga causes the diversion of normal blood circulation, robbing the body of oxygen and nutrients. Tga occurs in about 3% of all congenital heart defects. However, the term transposition was only applied in 1814, by farre, meaning that aorta and pulmonary trunk were placed positio across trans the ventricular. Aug 11, 2018 transposition of the great arteries is usually detected either prenatally or within the first hours to weeks of life. Transposition of the great arteries tga is the most common cyanotic congenital heart disease chd in newborns, with an incidence of 0. Transposition of the great arteries after mustardsenning.
Transposition of the great arteries 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. Congenital heart diseases involving only the primary arteries pulmonary artery and aorta belong to a subgroup called transposition of the great arteries. It accounts for up to 7% of all congenital cardiac anomalies 1 and can be assessed with echocardiography, gated cardiac ct, or cardiac mri. Transposition of the great arteries is usually detected either prenatally or within the first hours to weeks of life. A variety of descriptions have been used to characterize tga, but the most precise portrayal is a chd in which the morphologic right ventricle rv. Transposition of the great arteries childrens hospital of. Overall annual incidence is 2030 per 100,000 live births, tga is isolated in 90% of patients rarely associated with syndromes or extracardiac malformations. In this disorder, the two heart vessels, the pulmonary artery.
1257 246 1541 896 697 1071 789 1158 1098 63 9 1246 946 932 875 1498 623 195 508 267 740 744 682 546 475 1029 726 1127 1233 812 712 302 41 258 619 1103 985 865 501 1114