Definition the baron rokitansky, in his atlas of 1875, was the first to describe the entity we now know as congenitally corrected transposition figure figure1. These operations comprised 18 patients with a repair with a normal left ventricular outflow. Surgery for congenitally corrected transposition of the great. Request pdf congenitally corrected transposition of the great arteries pathological anatomypathophysiologynatural historyunnatural historyreferences find, read and cite all the research you. View enhanced pdf access article on wiley online library. Congenitally corrected transposition of the great arteries is a rare defect, seen in approximately 0. Congenitally corrected transposition of great arteries. Parallel vessels are evident in corrected tga, but as this sign is also present.
Jul 01, 2000 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. A minority of these patients maintain normal functional status into the seventh decade and generally only when no other anomalies exist. There is a wide variability in the physiology of cctga in patients presenting for lv retraining. Background patients with cctga have the anatomical right ventricle as their systemic pumping chamber, with ventricular dysfunction and chf being. Congenitally corrected transposition of the great arteries cctga is a rare cardiac anomaly with an incidence of less than 1% of patients with congenital heart disease. Cctga is a rare heart defect in which the hearts lower half is reversed. Binsalamah, md, msc, frcsc,a and christopher caldarone, md, frcscb congenitally corrected transposition of the great arteries cctga is a lesion. Cctga is a defect whereby the right atrium is connected to the left ventricle, supplying blood to the pulmonary artery, whereas the left atrium is connected to the right ventricle, supplying blood to the aorta. Patients with cctga are usually diagnosed at early stages of life due to associated anomalies, but they may even remain asymptomatic till later decades of their life. Congenitally corrected transposition of the great arteries cctga is a rare congenital heart defect in which the heart twists abnormally during fetal development and the ventricles are reversed. We describe a case of cctga, dextrocardia, and situs. Jan 22, 2019 congenitally corrected transposition of the great arteries cctga is a rare congenital heart defect in which the heart twists abnormally during fetal development and the ventricles are reversed. Introduction congenitally corrected transposition of the great arteries cctga is a rare defect combining atrioventricular discordance with ventriculoarte rial discordance. This condition is defined as the combination of atrioventricular av discordance and transposition of the great arteries.
The atriums are connected to the incorrect ventricles and the great arteries also come from the wrong ventricles transposition. Between may 1994 and september 2007, 68 patients with congenitally corrected transposition of the great arteries underwent anatomic repair. Congenitally corrected transposition of the great arteries cctga is a rare defect representing approximately 0. Some related complications begin and can be treated early in childhood, but others develop later in life.
Congenitally corrected transposition of the great arteries cctga is an uncommon cardiac defect characterized by the atria connecting with the anatomically discordant ventricles and the ventricles connecting with discordant and transposed great arteries. Congenitally corrected transposition of the great arteries cctga is a rare anomaly comprising a minimal portion of congenital heart disease cases. To understand cctga, it helps to first understand how a normal heart works. Anatomic repair is being actively evaluated as the preferred option for congenitally corrected transposition of the great arteries. Coronary anatomy in congenitally corrected transposition of the great arteries. Cctga requires lifelong care by a cardiologist experienced in congenital heart disease. Congenitally corrected transposition of the great arteries. Cctga is a rare congenital heart defect accounting for arteries. This chapter describes the pertinent pathoanatomy of dtransposition of the great arteries, ltransposition of the great arteries congenitally corrected transposition, as well as the malpositions of the great arteries associated with doubleoutlet right ventricle. Transposition of the great arteries tga is a common congenital cardiac anomaly and accounts for 45% of all congenital heart disease. Anatomic, physiologic repair, and palliation zachary spigel, md,a ziyad m. Most cases of ctga are associated with cardiac anomalies.
Congenitally corrected transposition of the great arteries cctga and situs inversus as confirmed by imaging. In cctga, the receiving chambers atria are connected to their opposite pumping chambers ventricles because the ventricles are switched from their normal anatomic positions. The morphology of the heart is distinctly abnormal, but the circulatory system is. Brizard, md, ms,a,b,c alice lee, md, bbiomed,b diana zannino, msc,c.
Congenitally corrected transposition of the great arteries english c. The morphological right ventricle rv supports the systemic circulation and the morphological tricuspid valve is the systemic atrioventricular valve savv 1. Clinical recognition can be challenging, so echocardiography is often the means by which definitive diagnosis is made. As the ventricular outflow tract may appear to arise correctly from the right and left ventricles, cases of ctga with a mild associated anomaly are rarely. Congenitally corrected transposition of the great arteries cctga is a rare congenital cardiac defect with atrioventricular and ventriculoarterial. Congenitally corrected transposition of the great arteries cctga is a rare heart defect in which the hearts lower half is reversed.
In this complex malformation the ventricles are on the opposite side of the heart to the usual. Congenitally corrected transposition of the great arteries cctga is a rare cardiac defect characterized by the atria connecting with anatomically discordant ventricles and the ventricles. In cctga both ventricles pumping chambers of the heart are reversed. Congenitally corrected transposition of the great arteries cctga is a rare heart defect in which the positions of the left and right ventricles the two pumping chambers of the heart are reversed. Congenitally corrected transposition of the great arteries ctga is an uncommon cardiac malformation characterized by discordant atrioventricular and ventriculoarterial connections. Longterm outcome in congenitally corrected transposition. The left atrioventricular valve and the leftsided ventricle resembled the usual right atrioventricular valve and right ventricle. Pdf congenitally corrected transposition of the great arteries cctga is a rare congenital cardiac defect with atrioventricular and. Congenitally corrected transposition of the great arteries, corrected transposition of the great arteries, ltransposition of the great arteries, ventricular inversion. Longterm results of anatomic correction for congenitally corrected transposition of the great arteries. Long term follow up after surgery in congenitally corrected. Coronary anatomy in congenitally corrected transposition. The detailed anatomy of the heart is described in 32 autopsy cases of congenitally corrected transposition of the great arteries.
We report a patient with congenitally corrected transposition of the great arteries, dextrocardia in congestive heart failure, with severe tricuspid regurgitation. We report a case of a 42yearold man who presented at. Congenitally corrected transposition of the great arteries bmj heart. Anatomic repair for congenitally corrected transposition of. Anatomic repair for congenitally corrected transposition of the great arteries rajesh sharma,a,b,c sachin talwar,b ashutosh marwah,a,c sejal shah,a sunita maheshwari,a pujari suresh,a rajnish garg,a,b bijender singh bali,a,c rajnish juneja, banita saxena,b and shyam sunder kothari objective. Outcomes of definitive surgical repair for congenitally corrected transposition of the great arteries or double outlet right ventricle with discordant atrioventricular connections. Congenitally corrected transposition of the great arteries is a complex heart condition. It may be present with or without associated anomalies.
Coronary anatomy in congenitally corrected transposition of. Chapter 6 congenitally corrected transposition of the great arteries. This means there are about 5,000 to 10,000 people in the united states with this condition. Congenitally corrected transposition of the great arteries cctga is a rare structural heart disease constituting less than 1% of all congenital. Sep 01, 2014 congenitally corrected transposition of the great arteries cctga is characterized by atrioventricular and ventriculoarterial discordance, with an incidence of 0. Longterm outcome in congenitally corrected transposition of. Surgery for congenitally corrected transposition of the. Sep 28, 2010 to investigate the longterm outcome of surgical treatment for congenitally corrected transposition of the great arteries cctga, in patients with biventricular repair with the right ventricle as systemic ventricle. Congenitally corrected transposition and dtransposition of. As a result, the aorta and the pulmonary artery are connected to the wrong lower heart chambers. Ventricular fibrillation in congenitally corrected. The patient underwent a twoventricle repair with physiologic correction and had an excellent postoperative outcome. Congenitally corrected transposition of the great arteries cctga is characterized by atrioventricular and ventriculoarterial discordance, with an incidence of 0. Transthoracic echocardiography confirmed the combination of atrioventricular av discordance and ventriculoarterial discordance repre.
Contemporary management and outcomes in congenitally. Left ventricular retraining in corrected transposition. Pdf congenitally corrected transposition of great arteries. Background patients with cctga have the anatomical right ventricle as their systemic pumping chamber, with ventricular dysfunction and chf. Characteristically the right atrium is connected to the morphologically left ventricle, which connects to the pulmonary artery and the left atrium is connected to the morphologically right ventricle, which connects to the. Congenitally corrected transposition of the great arteries, also known as levo or lloop transposition ltga, is a rare cardiovascular anomaly with inversion of the ventricles and great arteries. Request pdf congenitally corrected transposition of the great arteries pathological anatomypathophysiologynatural historyunnatural historyreferences find, read. Aug 27, 2009 congenitally corrected transposition of the great arteries. Surgical options for the failing right ventricle andor severe tricuspid regurgitation. Progressive tricuspid valve disease in patients with congenitally corrected transposition of the great. Echocardiography showed congenitally corrected transposition of the great arteries cctga with systolic dysfunction of the systemic right ventricle, ejection fraction was 15 to 20% and no other associated abnormalities. It is different from and much less common than regular transposition of the great arteries tga or dtga. Anatomic repair is being actively evaluated as the preferred option for congenitally corrected trans. Cctga is a rare congenital heart defect accounting for of all congenital heart diseases.
Atrioventricular dis cordance and ventriculoarterial discordance. Congenitally corrected transposition of the great arteries cctga can occur in isolation, or in combination with other structural cardiac anomalies, most commonly ventricular septal defect, pulmonary stenosis and tricuspid valve disease. Various terms have been used to describe this anatomy. Outcomes of biventricular repair for congenitally corrected. Congenitally corrected transposition of the great vessels.
Congenitally corrected transposition of the great arteries cctga or lloop tga what the nurse caring for a patient with chd needs to know mary rummell, mn, rn, cpnp, cns, faha clinical nurse specialist, pediatric cardiology, cardiac services oregon. Anatomic repair for congenitally corrected transposition. Congenitally corrected transposition of the great arteries cctga is rare form of congenital heart diseases. Pdf congenitally corrected transposition of the great. Longterm outcome in congenitally corrected transposition of the great arteries. Pdf congenitally corrected transposition of the great arteries in. Congenitally corrected transposition of the great arteries cctga, also known as ltransposition of the great arteries or ventricular inversion, is a rare and intricate form of congenital heart diseases. Congenitally corrected transposition of the great vessels cctga.
This is manifested in the array of lv pressure ratios and lv masses seen before intervention. Corrected transposition of the great arteries is the less common type of transposition. Nov 27, 2003 congenitally corrected transposition of the great arteries cctga is an uncommon congenital cardiac anomaly, accounting for less than 1% of live births with congenital heart disease. Congenitally corrected transposition of great arteries cctga is a rare congenital heart defect, mostly associated with ventricular septal defect, pulmonary stenosis, ebsteinlike malformation of the systemic atrioventricular av valve and highdegree av block.
Congenitally corrected transposition of the great arteries in a. Cardiac conduction system in congenitally corrected transposition. Congenitally corrected transposition of the great arteries ncbi. View enhanced pdf access article on wiley online library html view download pdf for offline viewing. A total of 32 patients with cctga were operated between january 1972 and october 2008.
Chest xray a and computed tomography d revealed situs inversus with the cardiac apex on the right. 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. Becker, md, facct hugh cameron, md, frc patht leon m. Longterm results of anatomic correction for congenitally. Anderson, md, mrc path ii london, birmingham and liverpool, england amsterdam, the netherlands the detailed anatomy of. Patients andor their parentsguardians should receive pregnancy counseling, 1 education regarding infective endocarditis prophylaxis, as well as.
Congenitally corrected transposition of the great arteries cctga or lloop tga what the nurse caring for a patient with chd needs to know mary rummell, mn, rn, cpnp, cns, faha clinical nurse specialist, pediatric cardiology, cardiac services oregon health and science university retired embryology. Levotransposition of the great arteries is an acyanotic congenital heart defect in which the primary arteries the aorta and the pulmonary artery are transposed, with the aorta anterior and to the left of the pulmonary artery. This chapter describes the pertinent pathoanatomy of d transposition of the great arteries, l transposition of the great arteries congenitally corrected transposition, as well as the malpositions of the great arteries associated with doubleoutlet right ventricle. Cctga, congenitally corrected transposition of the great arteries. Congenitally corrected transposition and dtransposition. Congenitally corrected transposition of great arteries bmj. Congenitally corrected transposition of the great arteries is a rare heart defect that occurs when the ventricles and attached valves are switched. 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. In congenitally corrected transposition of the great arteries cctga, atrioventricular and ventriculoarterial discordance maintains the appropriate direction of blood. May 14, 2011 congenitally corrected transposition of the great arteries, corrected transposition of the great arteries, l transposition of the great arteries, ventricular inversion. A case of congenitally corrected transposition of great.
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