Correspondence

Corrected Transposition of the Great Arteries

N Engl J Med 1996; 334:866-867March 28, 1996

Article

To the Editor:

Corrected transposition of the great arteries without associated intracardiac anomalies is a very rare condition.1 This lesion is also known as ventricular inversion with l-transposition. As with complete or d-transposition, the aorta arises from the right ventricle and the pulmonary artery from the left ventricle (Figure 1Figure 1Diagram of the Anatomy of Corrected Transposition of the Great Arteries.), but in corrected transposition of the great arteries there is also ventricular inversion, so that the “right ventricle” is on the left side of the heart and is connected to the left atrium. Thus, pulmonary venous return to the left atrium flows to the morphologic right ventricle and is then ejected into the aorta. Systemic venous return to the right atrium flows to the morphologic left ventricle and is ejected into the pulmonary artery. Patients with corrected transposition of the great arteries are pink and asymptomatic and may survive with their conditions undiagnosed until adulthood. The majority of patients (90 percent) have a ventricular septal defect or pulmonary stenosis (80 percent) (or both),2 which often produces congestive heart failure due to incompetence of the left atrioventricular valve or gradual deterioration of the anatomic right ventricle. Complete heart block is also a common cause of death.3

We report on an 80-year-old woman who was admitted to the hospital because of heart failure. She had been asymptomatic until two months earlier, when progressive exertional dyspnea developed. Physical examination revealed that her blood pressure was 125/65 mm Hg and her heart rate was 102 beats per minute. The apical impulse was felt at the left midclavicular line; the second heart sound was single and loud, and a soft grade 2/6 holosystolic murmur was heard at the apex. There were basal pulmonary rales. A chest film showed mild cardiomegaly with pulmonary congestion; the ascending aortic shadow and the main pulmonary-artery segment were absent.

Two-dimensional echocardiography revealed atrioventricular and ventriculoarterial discordance without ventricular septal defect or pulmonary stenosis. These findings were also confirmed by transesophageal echocardiography. In a frontal projection of the electrocardiogram-gated magnetic resonance image, the aorta arose anteriorly, and in the horizontal section the pulmonary artery arose posteromedially from a smooth left ventricle. These anatomical findings are typical of corrected transposition of the great arteries.

The patient was treated with digoxin, furosemide, and captopril; she was discharged 10 days later. She recently celebrated her 81st birthday in good health. We believe that this patient with uncomplicated corrected transposition of the great arteries is the oldest described in the literature.4

A. Melero-Pita, M.D.
F. Alonso-Pardo, M.D.
J.L. Bardají-Mayor, M.D.
J. Higueras, M.D.
Hospital Virgen de la Luz, 16002 Cuenca, Spain

4 References

1. 1

   Lundstrom U, Bull C, Wyse RKH, Somerville J. The natural and “unnatural“ history of congenitally corrected transposition. Am J Cardiol 1990;65:1222-1229
   CrossRef | Web of Science | Medline

2. 2

   Allwork SP, Bentall HH, Becker AE, et al. Congenitally corrected transposition of the great arteries: morphologic study of 32 cases. Am J Cardiol 1976;38:910-923
   CrossRef | Web of Science | Medline

3. 3

   Ikeda U, Furuse M, Suzuki O, Kimura K, Sekiguchi H, Shimada K. Long-term survival in aged patients with corrected transposition of the great arteries. Chest 1992;101:1382-1385
   CrossRef | Web of Science | Medline

4. 4

   Pezard P, Banus Y, Laporte J, Geslin P, Garnier H, Tadei A. Transposition corrigée des gros vaisseaux de l'adulte âgé. Arch Mal Coeur Vaiss 1986;79:1637-1642
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Citing Articles (4)

Citing Articles

1. 1

   Kousik Krishnan, Naomi A. Avramovitch, Michael H. Kim, Richard G. Trohman. (2005) Cardiac Resynchronization Therapy: A Potential Option for Congenitally Corrected Transposition of the Great Vessels. The Journal of Heart and Lung Transplantation 24:12, 2293-2296
   CrossRef

2. 2

   JoAnn Lindenfeld, Karin Keller, David N Campbell, Robert R Wolfe, Robert A Quaife. (2003) Improved systemic ventricular function after carvedilol administration in a patient with congenitally corrected transposition of the great arteries. The Journal of Heart and Lung Transplantation 22:2, 198-201
   CrossRef

3. 3

   PAULO S. DUARTE, ALFONSO Y. MATSUMOTO, VALDIR A. MOISÉS, ANTÔNIO S. TEBEXRENI, LUIZ E. MASTROCOLLA. (2002) Tc-99m MIBI SPECT in a Patient with Congenitally Corrected Transposition of the Great Arteries and Situs Solitus. Clinical Nuclear Medicine 27:7, 530-531
   CrossRef

4. 4

   Luc M Beauchesne, Carole A Warnes, Heidi M Connolly, Naser M Ammash, A.Jamil Tajik, Gordon K Danielson. (2002) Outcome of the unoperated adult who presents with congenitallycorrected transposition of the great arteries. Journal of the American College of Cardiology 40:2, 285-290
   CrossRef