Congenitally Corrected Transposition

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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. Patients and/or their parents/guardians should receive pregnancy counseling, ^[1]  education regarding infective endocarditis prophylaxis, as well as counseling about moderate and not heavy exercise routines. ^[2]

See the image below.

Symptoms usually reflect associated cardiac anomalies. The most common presenting features include the following:

bradycardia related to high-degree AV heart block

a single loud second heart sound, which is often palpable to the left of the sternum, arising from the anteriorly positioned aortic valve

heart murmur due to associated ventricular septal defect, pulmonic stenosis, or tricuspid regurgitation

cyanosis (only if there is an associated cardiac defect, such as pulmonary atresia or ventricular septal defect)

heart failure

tachyarrhythmia

See Clinical Presentation for more detail.

This condition is usually diagnosed later in childhood or in early adult life when patients present with complete heart block or heart failure due to right ventricular decompensation or systemic tricuspid valve regurgitation.

Diagnosis may require some or all of the following tests:

Echocardiogram

Chest radiography

Transesophageal echocardiography

Cardiac MRI

Electrocardiography

Echocardiogram and cardiac MRI are most commonly used in the diagnosis of CCTGA.

See Workup for more detail.

Medical care

There is little evidence that established medical treatment options for left ventricle dysfunction (ACE inhibitors, beta-blockers, nitrates) produce similar outcomes for systemic right ventricles. Caution should be used with administration of beta-blockers, as complete heart block may be precipitated in these patients with known conduction system abnormalities. Ultimately, patients with failing systemic ventricular function may best be served by cardiac transplantation.

Surgical care

For patients who need surgery, the type of operation will vary according to the associated defects. There are several options available including the following:

Ventricular septal defect closure (VSD) and insertion of a tube (conduit) between the heart and the lungs

Tricuspid valve replacement

Switch procedures, including double switch and the Senning-Rastelli procedure

Early pacemaker placement is recommended in the setting of complete heart block either during or after surgical intervention, or if any significant associated defect, such as cardiomegaly, decreased right ventricular function, symptomatic bradycardia, or heart failure, is present.

See Treatment and Medication for more detail.

Congenitally corrected transposition of the great vessels is a rare congenital heart defect associated with multiple cardiac morphologic abnormalities and conduction defects.

During embryologic development, left-handed looping of the heart tube results in atrioventricular (AV) discordance, and the aortopulmonary septum fails to rotate 180°, resulting in ventriculoarterial discordance. Blood flows in an effective sequence, hence the name corrected; however, the right ventricle supports the systemic circulation in this disorder.

Venous blood returns from the body into the right atrium before passing through the mitral valve into a morphological left ventricle. Blood then enters the lungs via the pulmonic valve into the main pulmonary artery. Pulmonary venous blood returns to the left atrium and then passes through the tricuspid valve to the morphological right ventricle, exiting to the aorta via the aortic valve. The aorta is positioned anterior and to the left of the pulmonary artery. In effect, the ventricles are transposed.

Causes and exposures associated with congenitally corrected transposition of the great arteries have not been identified clearly.

A substantial number of patients with congenital heart disease have a deletion of chromosome band 22q11. These deletions have been associated with abnormalities of the pulmonary arteries and aortic arch or its major branches regardless of the intracardiac anatomy. ^[3]  Rarely, these deletions are found in patients with transposition of the great vessels. In one series, none of 45 patients with transposition had the deletion.

Data from the Baltimore-Washington Infant Study supported the fact that congenitally corrected transposition is a rare disorder. ^[4]  As many as 40 infants per 100,000 live births are affected by congenitally corrected transposition of the great vessels; this is fewer than 1% of all congenital heart defects.

This disorder is reported in 0.5% of patients with congenital heart disease, and the literature reports fewer than 1000 cases. Most pediatric cardiologists have seen multiple cases of congenitally corrected transposition of the great vessels; however, the true prevalence of the malformation is not known.

Patient prognosis depends on AV conduction, arrhythmias, structural abnormalities, and degree of hemodynamic disturbance. ^[5]

Sudden death may be related to the onset of complete heart block or atrial or ventricular arrhythmias.

Right ventricular failure can develop over time. This may be related to coronary perfusion mismatch as the right ventricle is supplied by a single coronary artery. In addition, differences in right and left ventricular fiber orientation, geometry, and microscopic structural features may play a role in early failure of the right ventricle when functioning as the systemic ventricle. Poor prognostic indicators include cyanosis, polycythemia, pulmonary vascular obstructive disease, tricuspid regurgitation, younger age at surgery, larger preoperative shunt size, and lower right ventricular ejection fraction. A multicenter series of 182 patients with congenitally corrected transposition of the great arteries demonstrated that 25% of patients without associated cardiac lesions and 67% of patients with other cardiac abnormalities developed congestive heart failure by age 45. ^[6]

Note the following:

Ten-year survival rate ranges from 64-83% from the time of diagnosis and is dependent on associated anomalies.

Freedom ^[7]  reported an operative mortality rate of 6% and a 15-year actuarial survival rate of 48% in a cohort of patients with congenitally corrected transposition of the great vessels at the Hospital for Sick Children in Toronto.

A rare patient without associated cardiac anomalies may have a much more benign course, and literature documents many examples of these patients being diagnosed in the sixth and seventh decades of life. ^{[8, 9, 10]}

A median age at death of 40 years has been reported in both patients who have undergone operation and those who have not.

Major postoperative residual complications include contractile dysfunction of the systemic right ventricle, progressive tricuspid (systemic AV) regurgitation, complete heart block, atrial or ventricular arrhythmias, and infective endocarditis. Patients may develop conduit or homograph dysfunction postoperatively.

Systemic AV valve regurgitation is well described after surgery even when the valve has not been directly manipulated.

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van der Bom T, Winter MM, Groenink M, et al. Right ventricular end-diastolic volume combined with peak systolic blood pressure during exercise identifies patients at risk for complications in adults with a systemic right ventricle. J Am Coll Cardiol. 2013 Sep 3. 62(10):926-36. [Medline].

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Chaurasia A, Nawale J, Yemul M, Patil S, Mukhedkar S. Percutaneous transvenous tricuspid commissurotomy in a patient with congenitally corrected transposition of great arteries: a case report. Catheter Cardiovasc Interv. 2013 Dec 1. 82(7):E932-8. [Medline].

Arnold S Baas, MD, FACC, FACP Associate Professor of Medicine, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine; Attending Physician, Ronald Reagan UCLA Medical Center

Arnold S Baas, MD, FACC, FACP is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Federation for Medical Research, American Society of Echocardiography

Disclosure: Nothing to disclose.

Weiyi Tan, MD Fellow in Adult Congenital Cardiology, University of California, Los Angeles, David Geffen School of Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Yasmine S Ali, MD, FACC, FACP, MSCI President, Nashville Preventive Cardiology, PLLC; Assistant Clinical Professor of Medicine, Vanderbilt University School of Medicine

Yasmine S Ali, MD, FACC, FACP, MSCI is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, National Lipid Association, Tennessee Medical Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: MCG Health, LLC.

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Stacy D Fisher, MD; Martin Englehardt, MD, PhD; Wojciech Zareba, MD, PhD, FACC; and Roger Vermilion, MD to the development and writing of this article.

Congenitally Corrected Transposition

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