Congenitally corrected transposition of the great arteries: An update

Opinion statement  Congenitally corrected transposition of the great arteries is a rare condition in which systemic venous blood returns to normally positioned atria. However, the atria are connected to the opposite ventricle, right atrium to left ventricle, left atrium to right ventricle; so-called atrioventricular disordance. In addition, the ventricles are inverted (right to left change in position) and are connected to the opposite great artery, left ventricle to pulmonary artery, right ventricle to aorta; thus, forming ventricular-arterial discordance. The aorta is anterior and to the left of the pulmonary artery, L-transposed. Atrioventricular discordance plus ventricular-arterial discordance results in normal blood flow (ie, congenitally corrected). The right ventricle with the tricuspid valve is the systemic ventricle. Common associated conditions are ventricular septal defects (VSDs), pulmonary stenosis, and congenital heart block. Major issues related to management revolve around the status of the systemic right ventricle, which can develop dysfunction with increasing age and tricuspid regurgitation, which can increase in severity with age and contribute to ventricular dysfunction. One emerging treatment is the double switch operation. In patients with no pulmonary obstruction, it is possible to switch the systemic and pulmonary venous return using an atrial baffle procedure followed by an arterial switch procedure. This results in the anatomical left ventricle now functioning as the systemic ventricle. In those patients with associated pulmonary obstruction and a VSD, another type of double switch can be performed in which the left ventricle is tunneled through the VSD to the aorta, the right ventricle is connected to the pulmonary artery with a homograft or other conduit, and the atrial baffle procedure is performed. The most difficult challenge is choosing the patient who is a candidate for the double-switch operation and the timing of that operation, or the timing of a more classical operation for associated defects.     

Congenitally corrected transposition of the great arteries: Current treatment options

Opinion statement Congenitally corrected transposition of the great arteries is a relatively rare cardiac malformation characterized by atrioventricular and ventriculoarterial discordance. This double discordance results in a physiologically corrected circulation with the morphologic right ventricle (RV) serving as the systemic pump. Associated anomalies are present in approximately 98% of cases and include most commonly ventricular septal defect, pulmonary stenosis, and anomalies of the systemic atrioventricular valve (SAVV). Conduction abnormalities are common as well, predisposing these patients to the development of complete heart block. The management of these patients is primarily determined by the presence and severity of the associated anomalies. Conventional repair, which leaves the morphologic RV as the systemic ventricle, has resulted in high incidence of tricuspid regurgitation (TR) and progressive dysfunction of the RV. In the majority of patients, congestive heart failure secondary to RV dysfunction occurs by the fifth or sixth decade. The cause and effect relationship between TR and RV dysfunction remains to be determined. The advent of the Double Switch operation, which restores the morphologic left ventricle (LV) as the systemic ventricle, has yielded favorable outcomes at early follow-up. This procedure should be applied in young patients to achieve optimal results. Retraining the LV by pulmonary artery banding is associated with high morbidity and mortality when attempted after infancy or early childhood. Long-term follow-up is needed to evaluate the potential superiority of this procedure and the incidence of rhythm, baffle, and conduit complications for this management option.     

Congenitally corrected transposition of the great arteries in a 65-year-old woman.

A 65-year-old Japanese woman was admitted to hospital because of exertional dyspnea. Transthoracic echocardiography showed diffuse hypokinesis of the left-sided ventricular wall, but was not clear enough to provide useful information because of the rotation of the cardiac apex and the presence of lung tissue. Systemic ventriculography showed that the left-sided ventricle with heavy trabeculations was morphologically similar to a normal right ventricle. Magnetic resonance imaging (MRI) clearly revealed corrected transposition of the great arteries. Because this patient had no severe associated cardiac anomalies, systemic ventricular dysfunction is thought to be the major cause of exertional dyspnea. MRI is a useful non-invasive method for the rapid evaluation of cardiac morphology.     

Congenitally corrected transposition of the great arteries and participation in competitive sport

Congenitally corrected transposition of the great arteries is a rare anomaly, where the systemic circulation is supported by the morphological right ventricle. We present a 43 year-old asymptomatic male, a former competitive short-distance runner, with recently diagnosed congenitally corrected transposition of the great arteries. To our knowledge this is the first report of such a case.     

Congenitally corrected transposition of the great arteries treated by partial systemic ventriculectomy.

Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart disease characterized by atrioventricular (AV) and ventriculo-arterial discordance;(1) that is, the left ventricle supports the pulmonary circulation and the right ventricle supports the systemic circulation. The most common cardiac anomalies in CCTGA include ventricular septal defect, pulmonary outflow tract obstruction and abnormalities of the systemic AV valve. (1) The dysfunction of the systemic ventricle occurs with increasing frequency in older patients with CCTGA, independent of their commonly associated structural defects.(2-4) We report a patient with CCTGA undergone the partial ventriculectomy for his systemic ventricular dysfunction.     

Congenitally corrected transposition of the great arteries: Morphologic study of 32 cases

The detailed anatomy of the heart is described in 32 autopsy cases of congenitally corrected transposition of the great arteries. This condition is defined as the combination of atrioventricular (A-V) discordance and transposition of the great arteries. Examples of primitive (single) ventricle with “inverted” (that is, left-sided in situs solitus) outlet chamber are excluded. Six hearts with A-V discordance and pulmonary atresia are described in an appendix. In 29 cases of corrected transposition the heart was in situs solitus; in 3 it was in situs inversus totalis. Only 5 of these 32 hearts had no potential for intracardiac shunting. Anomalies of the tricuspid valve (91 percent of cases), ventricular septal defect (78 percent) and pulmonary outflow tract obstruction (44 percent) occurred with sufficient frequency to be considered part of the basic malformation and are described in detail. The precise anatomy and disposition of the A-V valve tension apparatus, the coronary arteries and the conducting tissues are described with special reference to possible surgical approaches for repair of the anomalies. In two hearts with situs solitus the aortic valve was right-sided with respect to the pulmonary valve. This finding is important for both diagnosis and nomenclature.     

Congenitally corrected transposition of the great arteries in an 80 year old woman

Congenitally corrected transposition of the great arteries (CCTGA) is a rare form of congenital heart disease characterised by atrioventricular as well as ventriculoarterial discordance. It is usually associated with a variety of severe intracardiac defects. Few patients with this abnormality survive past 50 years. An 80 year old woman was admitted to the hospital because of mild congestive heart failure. Cardiac examination revealed a 4/6 holosystolic and a 2/6 decrescendo diastolic murmur at the left sternal border. Radiography, echocardiography, and computed tomography confirmed newly diagnosed CCTGA without associated intracardiac defects.     

Congenitally corrected transposition and degenerative severe aortic stenosis

Congenitally corrected transposition is a rare cardiac anomaly characterized by the combination of discordant atrioventricular and ventriculoarterial connections. Young patients with this lesion can present with congestive cardiac failure, usually secondary to a large ventricular septal defect or pulmonary stenosis. We report here our experience with a lady aged 79, admitted to our unit because of deterioration of her congestive cardiac failure as a consequence of uncorrected congenitally corrected transposition associated with degenerative severe aortic stenosis.     

Congenitally corrected transposition of the great arteries in a 72 year old man--a case report

Congenitally corrected transposition of the great arteries (CCTGA) is a rare form of congenital heart disease characterised by atrioventricular as well as ventriculoarterial discordance. The life expectancy of individuals with CCTGA is limited by the onset of the systemic ventricular failure. There have been only a few patients with CCTGA and age >50 years reported in literature. We describe a 72-year-old man with CCTGA who was admitted to the hospital because of severe congestive heart failure. Our patient s survival is one of the longest ever described.     

Congenitally corrected transposition of the great arteries plus dextrocardia operated with an unusual operative technique

We describe a case of congenitally corrected transposition of the great arteries plus dextrocardia and normal anatomical abdominal viscera. Systemic (tricuspid) valve replacement was performed due to moderately severe valve regurgitation. An unusual operative technique was used because of the patient's rare morphology. On cardiopulmonary bypass, the systemic valve was approached via a left atriotomy anterior to the left pulmonary veins, whereby the surgeon was positioned on the patient's left side. As the surgeon had excellent exposure from the opposite side of the table, he was able to perform a tricuspid valve replacement through the left atrium. Our aim is to share our limited experience of such cardiac morphology, which may oblige the surgeon to be positioned on the left side of the table to perform systemic valve surgery.     

Congenitally corrected transposition of the great arteries with severely stenotic bicuspid aortic valve in an adult: a case report

Congenitally corrected transposition of the great arteries (CCTGA) is a rare disease accounting for 0.5–1.4% of patients with congenital heart disease. In this congenital cardiopathy, the left atrium is connected to the right ventricle, from which the aorta stems. We present a case of an adult with CCTGA associated with aortic stenosis and bicuspid aortic valve—an association not yet described to our knowledge. (Echocardiography 2012;29:E13-E15)