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. 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. Transposition of the great arteries tga was first recognized and described more than 2 centuries ago. Transposition of the great arteries radcliffecardiology.
Facts about dextrotransposition of the great arteries dtga. In transposition, oxygenated blood returning to the left side of the heart is carried back to the lungs by a transposed pulmonary artery. The pulmonary artery in infants with tga in general banded more loosely than in infants with normally related great arteries. Transposition of the great arteries childrens memorial hermann. Transposition of the great arteries differential diagnoses. 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. 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. In a normal heart, there are two large arteries that carry blood out of the heart. Corrected tga is defined by the coexistence of atrioventricular and ventriculoarterial discordance.
Transposition of the great arteries is the most common cyanotic congenital heart defect. Transposition of the great arteries childrens hospital. 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. Transposition of the great arteries symptoms and causes. This operation is called an atrial or venous switch. Dextrotransposition of the great arteries dtga occurs when the position of the main pulmonary artery and aorta are switched. Levotransposition 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. The two major arteries that carry blood away from the heart the aorta and the pulmonary artery are switched transposed.
Transposition of the great arteries statpearls ncbi bookshelf. Tga is one of the most common and severe congenital heart diseases. Systemic venous return enters into the right atrium, which connects to a morphologic left ventricle, which then connects to a pulmonary artery. Overall annual incidence is 2030 per 100,000 live births, tga is isolated in 90% of patients rarely associated. 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. It accounts for up to 7% of all congenital cardiac anomalies 1 and can be assessed with echocardiography. 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. Understanding transposition of the great arteries the obg. The result is that blood containing oxygen from the lungs is pumped back into the lungs, while blood that lacks oxygen, which is necessary to nourish the. But this requires special diligence and therefore, at times, can be missed on a routine fetal ultrasound. Transposition of the great arteries is usually detected either prenatally or within the first hours to weeks of life. Transposition of the great arteries, dextrolooped 3. It is one of the most common cyanotic heart diseases, and most affected neonates are undiagnosed as fetuses.
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. The arterial switch operation aso has replaced atrial switch procedures for dtga, and 90% of patients now reach adulthood. 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. Hie multimedia transposition of the great arteries. Multimodality imaging guidelines of patients with transposition of. Transposition of the great arteries in the developing world jacc. In a structurally normal heart, the aorta arises from the left ventricle and the pulmonary artery pa arises from the. In 50% of cases, the va discordance is an isolated. Pdf transposition of the great arteries researchgate.
Transposition of the great arteries tga is a common congenital cardiac anomaly and accounts for 45% of all congenital heart disease. 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. Transposition of the great arteries tga is a complex congenital heart defect. Dtransposition of the great arteries is a form of congenital heart disease that must be corrected shortly after birth for survival.
Transposition of great arteries tga radiology case. Apr 11, 2017 transposition of the great arteries tga is the most common cyanotic congenital heart lesion that presents in neonates. D transposition of the great arteries is a form of congenital heart disease that must be corrected shortly after birth for survival. 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. Transposition of the great arteries radiology reference. Population trends in prenatal detection of transposition of great arteries. Transposition of great arteries dr amarja sachin nagre md,dm,fca. 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. It accounts for approximately 5% of congenital heart disease cases and is characterized by ventriculoarterial discordance. Transposition of the great arteries tga the embryo. Complete transposition of the great arteries request pdf.
Transposition of the great arteries choc childrens. Pediatric cardiothoracic surgery transposition of great. The pulmonary artery, which usually arises from the right side of the heart and carries deoxygenated blood to the lungs, will now connect to the left side of the heart and send oxygenated blood back to the lungs. Coronary artery anatomy and transposition of the great arteries. Transposition of the great arteries, pediatric flashcards. Clinical guidelines for the management of patients with. 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. When a doctor sees for the first time a patient or a heart where the morphological right atrium is connected with the left ventricle lv and this in turn with the pulmonary artery, while the left atrium is communi. Any insult to the migration of neural crest cells can cause tetralogy of fallot, truncus arteriosus, and tga. The aorta is connected to the right ventricle, the pulmonary artery to the left ventricle. Jan 20, 20 pulmonary artery banding transposition associated with large vsd without left ventricular outflow tract stenosis, if untreated, is complicated by two major problems.
Transposition of the great arteries tga is a heart defect that occurs from birth congenital. Transposition of the great arteries tga is the most common cyanotic congenital heart lesion that presents in neonates. Transposition of great arteries video khan academy. Corrected transposition of the great arteries is one of the most fascinating entities in congenital heart disease. The transposition of the great arteries is ventriculo arterial 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 a serious but rare heart defect present at birth congenital, in which the two main arteries leaving the heart are reversed transposed.
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 requires corrective surgery in the first weeks of life. Transposition of the great arteries description and epidemiology. Oxygenpoor blue blood returns to the right atrium from the body, passes through the right atrium and ventricle, then goes into the misconnected. Levotransposition 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. Complete transposition of the great arteries is also known as dtga. Sometimes, a fetal echocardiogram performed by specialists is required to make a diagnosis of transposition in a fetus. Transposition of the great arteries cs mott childrens. 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 opposite of a normal hearts anatomy. 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. Transposition of the great arteries can be diagnosed by a fetal ultrasound. Congenital heart disease, transposition of the great arteries, arterial switch.
The association with other cardiac malformations such as ventricular septal defect vsd and left ventricular outflow tract obstruction is frequent and dictates timing. The more common form of transposition of the great arteries, dextrolooped tga, consists of complete inversion of the great vessels, so that the aorta incorrectly arises from the right ventricle and the pulmonary artery incorrectly arises from the left ventricle. Transposition of great arteries linkedin slideshare. In transposition of the great arteries tga, the pulmonary artery and aorta have changed places i. 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. 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. Figure 81 schematic diagram of the anatomy of dextro transposition of the great arteries dtga. Transposition of the great arteries is a rare, critical birth defect in which the two main arteries of the heart the aorta and the pulmonary artery are reversed.
Transposition of the great arteries orphanet journal of. Leftuntreated,tgaisassociatedwith from the achildrens national heart institute, childrens national medical center, george washington university, washington. The surgery for transposition of great arteries tga symbolises the manner in which cardiac surgery has progressed. 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 is the most common cyanotic congenital heart disease chd in newborns, with an incidence of 0. 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. We conducted a retrospective cohort study that included all cases of tga with intact ventricular septum and tga with ventricular septal defect performed from 2010. Transposition of the great arteries tga pediatrics. In children with tga, these arteries are connected to the heart abnormally. Transposition of the great arteries or tga is a potentially fatal congenital heart malformation where the pulmonary artery and the aorta are switched. Tga may occur as an isolated defect or in association with other cardiac anomalies. Diagnosis of transposition of the great arteries in the fetus.
Dextro transposition pronounced deckstro transpohzishun 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. 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 tga is a cyanotic congenital heart defect in which the aorta arises from the right ventricle and the pulmonary artery. Transposition of great arteries in transposition of the great arteries, the two major arteries leaving the heart are connected to the wrong ventricles, the two lower chambers of the heart. Transposition of the great arteries tga is a group of congenital cardiac defects characterized by ventriculoarterial discordance. Anatomical correction has a favourable outcome and arterial switch operation aso has become the preferred option for treating tga. This changes the way blood circulates through the body, creating a shortage of oxygen in the blood that flows from the heart to the rest of the. 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. Introduction tga most common etiology for cyanotic congenital heart disease in the newborn. 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. In 50% of cases, the va discordance is an isolated finding.
The sequelae of the complex congenital heart diseases could be severe if undiagnosed before birth. Heinle introduction first described by the scottish pathologist dr. This redirects the oxygenrich blood to the right ventricle and aorta and the oxygenpoor blood to the left ventricle and the pulmonary artery. Dextro transposition of the great arteries or dtga is a birth. Corrective surgery soon after birth is the usual treatment for transposition of the great arteries. Having a baby with transposition of the great arteries can be alarming, but with proper treatment, the outlook is promising. Transposition of the arteries childrens hospital colorado. Transposition of the great arteries tga is a rare but serious congenital heart defect, meaning it is present at birth, in which the two main arteries that leave the heart are reversed, or transposed. The first, developed in the late 1950s, creates a tunnel a baffle between the atria. The incidence is estimated at 1 in 3,5005,000 live births, with a maletofemale ratio 1.
Transposition is a switching, oneforone switch, a for b, and b going where a is supposed to go. Associated defects, such as abnormalities of the tricuspid valve, ventricular septal defect, and pulmonary stenosis, occur in the majority of patients. 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 tga is a complex congenital heart defect usually defined within the group of conotruncal defects. The switch means that the aorta, which normally carries oxygenated blood, carries deoxygenated blood. 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. The aorta is attached to the rightsided pumping chamber ventricle, instead of the left. The switching of the arterial position changes the way blood flows through the heart, resulting in a dangerous shortage of oxygenated blood going to the babys body. Matthew baillie in 1797 1, transposition of the great arteries was for decades merely known as a fascinating disease that was universally fatal. The aorta is located anteriorly and to the right, and the great arteries run in parallel instead of in their normal perpendicular relation. Overall annual incidence is 2030 per 100,000 live births, tga is isolated in 90% of patients rarely associated with syndromes or extracardiac malformations. Aug 11, 2018 in transposition of the great arteries, the origins of the main arteries leading away from the heart the aorta and the pulmonary artery are switched transposed. Transposition of the great arteries in the developing world. The eventually successful solution in the form of the now popular arterial switch operation aso was elucidated after multiple contributions from several surgical stalwarts and geniuses over more than 25 years table 1.
Two major types of surgery can correct the transposition. Note that the systemic venous return blue and pulmonary venous return red circulate separately unless a shunt at the atrial, ventricular, andor ductal level permits mixing between the. Note that the systemic venous return blue and pulmonary venous return red circulate separately unless a shunt at the atrial, ventricular, andor ductal level permits mixing between the two circuits. Dloop transposition of the great arteries tga is the second most common form of cyanotic congenital disease 3. 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. Because a baby with this defect may need surgery or other procedures soon after birth, dtga is.
1420 869 198 925 1296 983 1138 1248 818 605 1477 608 808 986 1231 451 1581 1397 813 104 1035 569 1438 962 247 1284 1289 173 132 1270 252 1219 306 1186 485 1217 1306