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(Radiology 2003;226:701-705.)
© RSNA, 2003

Diagnosis Please

Case 56: Cor Triatriatum Dexter1

Bonnie N. Joe, MD, PhD, Mehdi Poustchi-Amin, MD and Pamela K. Woodard, MD

1 From the Mallinckrodt Institute of Radiology, Washington University Medical Center, 510 South Kingshighway Blvd, St Louis, MO 63110. Received November 27, 2000; revision requested January 10, 2001; revision received June 28; accepted July 5. Address correspondence to B.N.J. (e-mail: joeb@mir.wustl.edu).

Index terms: Atrial septal defect, 514.141, 521.141 • Diagnosis Please • Heart, abnormalities, 514.141, 521.141 • Heart, atria, 514.141, 521.141


   HISTORY
TOP
 HISTORY
IMAGING FINDINGS
 DISCUSSION
 REFERENCES
 
A 70-year-old Afghan refugee who recently immigrated to the United States had a history of dyspnea on exertion for several months. Medical history included systemic hypertension, recent onset of congestive heart failure, and recent peripheral pitting edema of the lower extremities. A soft systolic heart murmur with a split S2 was present at physical examination. An echocardiogram showed findings that prompted the request for a cardiac magnetic resonance (MR) imaging examination for further evaluation (Figs 1 –3).



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  		Figure 1a. (a-d) Selected transverse half-Fourier rapid acquisition with relaxation enhancement images (repetition time msec/echo time msec, 1,180/48; flip angle, 160°) acquired through the heart during diastole. Arrows point to membrane dividing right atrium.

 


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  		Figure 1b. (a-d) Selected transverse half-Fourier rapid acquisition with relaxation enhancement images (repetition time msec/echo time msec, 1,180/48; flip angle, 160°) acquired through the heart during diastole. Arrows point to membrane dividing right atrium.

 


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  		Figure 1c. (a-d) Selected transverse half-Fourier rapid acquisition with relaxation enhancement images (repetition time msec/echo time msec, 1,180/48; flip angle, 160°) acquired through the heart during diastole. Arrows point to membrane dividing right atrium.

 


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  		Figure 1d. (a-d) Selected transverse half-Fourier rapid acquisition with relaxation enhancement images (repetition time msec/echo time msec, 1,180/48; flip angle, 160°) acquired through the heart during diastole. Arrows point to membrane dividing right atrium.

 


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  		Figure 2a. (a-d) Transverse bright blood cine gradient-recalled-echo image (56/6.1; flip angle, 20°) acquired through the heart during diastole. Arrows point to membrane dividing right atrium. A small amount of turbulent flow is seen across the dividing membrane (arrowhead).

 


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  		Figure 2b. (a-d) Transverse bright blood cine gradient-recalled-echo image (56/6.1; flip angle, 20°) acquired through the heart during diastole. Arrows point to membrane dividing right atrium. A small amount of turbulent flow is seen across the dividing membrane (arrowhead).

 


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  		Figure 2c. (a-d) Transverse bright blood cine gradient-recalled-echo image (56/6.1; flip angle, 20°) acquired through the heart during diastole. Arrows point to membrane dividing right atrium. A small amount of turbulent flow is seen across the dividing membrane (arrowhead).

 


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  		Figure 2d. (a-d) Transverse bright blood cine gradient-recalled-echo image (56/6.1; flip angle, 20°) acquired through the heart during diastole. Arrows point to membrane dividing right atrium. A small amount of turbulent flow is seen across the dividing membrane (arrowhead).

 


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  		Figure 3a. (a-d) Oblique transverse bright blood cine gradient-recalled-echo images (56/6.1; flip angle, 20°) obtained during systole through the heart at the level of the aortic valve. Arrow points to a jet through a small ASD.

 


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  		Figure 3b. (a-d) Oblique transverse bright blood cine gradient-recalled-echo images (56/6.1; flip angle, 20°) obtained during systole through the heart at the level of the aortic valve. Arrow points to a jet through a small ASD.

 


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  		Figure 3c. (a-d) Oblique transverse bright blood cine gradient-recalled-echo images (56/6.1; flip angle, 20°) obtained during systole through the heart at the level of the aortic valve. Arrow points to a jet through a small ASD.

 


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  		Figure 3d. (a-d) Oblique transverse bright blood cine gradient-recalled-echo images (56/6.1; flip angle, 20°) obtained during systole through the heart at the level of the aortic valve. Arrow points to a jet through a small ASD.

 

   IMAGING FINDINGS
TOP
 HISTORY
 IMAGING FINDINGS
DISCUSSION
 REFERENCES
 
MR images demonstrated moderate right atrial enlargement, with a membrane separating the right atrium into an anterior and a posterior chamber (Figs 1, 2). Turbulent flow was seen across this membrane (Fig 2; Movie, radiology.rsnajnls.org/cgi/content/full/2263001845/DC1) and indicated that it was extensively fenestrated and not completely obstructive. Gradient-echo cine images demonstrated a small secundum atrial septal defect (ASD) that was approximately 4 mm in diameter (Fig 3). A small left-to-right jet through the ASD was also seen on the cine images. The right ventricle was mildly enlarged. There was mild enlargement of bilateral pulmonary arteries. Mild left ventricular hypertrophy was present, which was likely related to the patient’s known systemic hypertension. Findings of the examination of the remainder of the heart were within normal limits.


   DISCUSSION
TOP
 HISTORY
 IMAGING FINDINGS
 DISCUSSION
REFERENCES
 
While on the basis of the clinical findings the differential diagnosis considerations included entities such as constrictive pericarditis, tricuspid stenosis, or right atrial tumor, the imaging findings helped to narrow the possibilities to one diagnosis: cor triatriatum dexter. The term cor triatriatum, also known as "triatrial heart," usually refers to cor triatriatum sinister or "divided left atrium," which is a rare condition with estimated incidence of 0.1% of all congenital cardiac malformations (1). In cor triatriatum sinister, the left atrium is divided by a membrane into a posterior-superior chamber that receives the four pulmonary veins and an anterior-inferior chamber that connects to the left ventricle by means of the mitral valve. Although several theories have been proposed in the literature regarding the development of cor triatriatum sinister, the most widely accepted theory is that this anomaly results from a failure of incorporation of the common pulmonary vein into the left atrium (2,3).

The case presented in this article is an example of cor triatriatum dexter in which a membrane divides the right atrium into two chambers. It is generally believed that cor triatriatum dexter results from persistence of the right valve of the sinus venosus (4–6). During embryogenesis, the right horn of the sinus venosus gradually incorporates into the right atrium to form the smooth posterior portion of the right atrium, whereas the original embryologic right atrium forms the trabeculated anterior portion. The connection between the right horn of the sinus venosus and the embryologic right atrium is the sinoatrial orifice, which is flanked on either side by two valvular folds, the right and left venous valves (7). At some point during this incorporation, the right valve of the right horn of the sinus venosus divides the right atrium in two. This right valve forms a sheet that serves to direct the oxygenated venous return from the inferior vena cava across the foramen ovale to the left side of the heart during fetal life (8).

Normally, the valve regresses by approximately 12 weeks gestation (9) and leaves behind the crista terminalis superiorly and the eustachian valve of the inferior vena cava and the thebesian valve of the coronary sinus inferiorly (5,7,10). Trento et al (11) examined 14 hearts (specimens in the heart museum of the Children's Hospital of Pittsburgh) from pediatric patients that demonstrated abnormal persistence of the right sinus venosus valve and classified them into two main categories: those with filigreed or weblike networks as remnants of the right valve (also known as Chiari net) and those with a partition between the venous (smooth) and trabeculated portions of the right atrium, that is, cor triatriatum dexter. Unlike cor triatriatum sinister, which carries a high mortality rate if not repaired (3), cor triatriatum dexter has varying clinical manifestations, depending on the degree of partitioning or septation of the right atrium.

With only a minor degree of septation of the right atrium, cor triatriatum dexter often is asymptomatic and is detected only incidentally, for example, during surgery to correct other cardiac abnormalities or during echocardiography. In one case, recurrent supraventricular tachycardias prompted evaluation that led to diagnosis of cor triatriatum dexter (9). Persistence of a large membrane dividing the right atrium can cause right-sided heart failure and elevated central venous pressures secondary to obstruction of the tricuspid valve, the right ventricular outflow tract, or the inferior vena cava (5). Associated cardiac defects, such as ASDs, have been described (12,13).

In the past, the mainstay of treatment for symptomatic patients has been surgical resection of the dividing membrane (9,12,13). However, percutaneous catheter disruption of the membrane has been reported (14) and has been suggested as a preferred alternative to open heart surgery (5). Asymptomatic patients are generally not treated unless they are undergoing cardiac surgery for other reasons.

Our patient had a history of shortness of breath and peripheral edema at presentation. Findings at echocardiography suggested an ASD and indicated the presence of a membrane in the right atrium (Fig 4). Cardiac MR imaging was then performed to assess the extent of these anomalies and to assess the pulmonary arteries and right side of the heart.



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  		Figure 4. Transesophageal echocardiogram (long axis view) demonstrates membrane (*) in right atrium. ASD = atrial septal defect, LA = left atrium, RA = right atrium, RV = right ventricle.

 
Since many patients are asymptomatic, the diagnosis of cor triatriatum dexter often is determined at postmortem examination. Antemortem diagnosis can be determined by using angiography, echocardiography, or MR imaging. There have been numerous reports about the use of echocardiography to determine the diagnosis noninvasively that mainly date from the mid-1980s to early 1990s (15–19).

Within the last decade, case reports (20–24) in which MR imaging was used for diagnosis and preoperative planning for cor triatriatum sinister have been published. In a study in which MR imaging was compared with echocardiography and cardiac angiography in the evaluation of pulmonary venous anomalies, which included cases of cor triatriatum (sinister), MR imaging had a higher detection rate (95%) than the other modalities (69% for angiography and 38% for echocardiography) (25).

In general, MR imaging provides better spatial resolution and superior tissue contrast compared with echocardiography. Another advantage of cardiac MR imaging is that it can easily be performed in multiple planes, including long axis, double oblique, and coronal planes for valve assessment and in the short-axis plane for functional studies of the left and right ventricles. In cor triatriatum dexter, the dividing membrane is best visualized on transverse images. For our patient, some transverse images were obtained along a slightly oblique plane to best profile the secundum ASD.

Although echocardiography has better temporal resolution than does MR imaging, fast gradient-recalled-echo cine imaging of the cardiac cycle, because of its better spatial resolution and increased number of possible imaging planes, has been shown to be of equal or better benefit in the assessment of cardiac function (26). Cine images were useful in this particular case to demonstrate the turbulent flow across the dividing membrane in the right atrium and the jet across the ASD. If necessary, the ratio of right and left ventricular stroke volume can be determined to estimate the shunt ratio, but accuracy can be affected by the presence of valvular or other cardiac disease (27).


   FOOTNOTES
 
Part 1 of this case appeared 4 months previously and may contain larger images.


   REFERENCES
TOP
 HISTORY
 IMAGING FINDINGS
 DISCUSSION
 REFERENCES
 

  1. Arrants JE, Riopel DA, Catalano PW. Cor triatriatum: preoperative diagnos is and successful surgical correction in a ten-week-old infant. Chest 1973; 63:1027-1028.[Abstract/Free Full Text]
  2. Schujman E, Loewenthal M. Cor triatriatum. Med J Aust 1976; 1:304-307.[Medline]
  3. Herlong JR, Jaggers JJ, Ungerleider RM. Congenital Heart Surgery Nomenclature and Database Project: pulmonary venous anomalies. Ann Thorac Surg 2000; 69(4 suppl):S56-S69.[Abstract/Free Full Text]
  4. Arciniegas E. Cor triatriatum dextrum. In: Arciniegas E, eds. Pediatric cardiac surgery. Chicago, Ill: Year Book Medical Publishers, 1985; 373-374.
  5. Embrey RP. Cor triatriatum, pulmonary vein stenosis, atresia of the common pulmonary vein. In: Mavroudis C, Backer CL, eds. Pediatric cardiac surgery. 2nd ed. St Louis, Mo: Mosby–Year Book, 1994; 503-504.
  6. Schutte DA, Rowland DG, Allen HD, Bharati S. Prominent venous valves in hypoplastic right hearts. Am Heart J 1997; 134:527-531.[CrossRef][Medline]
  7. Sadler TW. Langman’s medical embryology 6th ed. Baltimore, Md: Williams & Wilkins, 1990.
  8. Anderson RH. Understanding the nature of congenital division of the atrial chambers. Br Heart J 1992; 68:1-3.
  9. Ott DA, Cooley DA, Angelini P, Leachman RD. Successful surgical correction of symptomatic cor triatriatum dexter. J Thorac Cardiovasc Surg 1979; 78:573-575.[Abstract]
  10. Yater WM. Variations and anomalies of the venous valves of the right atrium of the human heart. Arch Pathol 1929; 7:418-441.
  11. Trento A, Zuberbuhler JR, Anderson RH, Park SC, Siewers RD. Divided right atrium (prominence of the eustachian and thebesian valves). J Thorac Cardiovasc Surg 1988; 96:457-463.[Abstract]
  12. Mazzucco A, Bortolotti U, Gallucci V, Del Torso S, Pellegrino P. Successful repair of symptomatic cor triatriatum dexter in infancy. J Thorac Cardiovasc Surg 1983; 85:140-143.[Abstract]
  13. Hansing CE, Young WP, Rowe GG. Cor triatriatum dexter: persistent right sinus venosus valve. Am J Cardiol 1972; 30:559-564.[CrossRef][Medline]
  14. Savas V, Samyn J, Schreiber TL, Hauser A, O’Neill WW. Cor triatriatum dexter: recognition and percutaneous transluminal correction. Cathet Cardiovasc Diagn 1991; 23:183-186.[Medline]
  15. Alboliras ET, Edwards WD, Driscoll DJ, Seward JB. Cor triatriatum dexter: two-dimensional echocardiographic diagnosis. J Am Coll Cardiol 1987; 9:334-337.[Abstract]
  16. Burton DA, Chin A, Weinber PM, Pigott JD. Identification of cor triatriatum dexter by two-dimensional echocardiography. Am J Cardiol 1987; 60:409-410.[CrossRef][Medline]
  17. Dobbertin A, Warnes CA, Seward JB. Cor triatriatum dexter in an adult diagnosed by transesophageal echocardiography: a case report. J Am Soc Echocardiogr 1995; 8:952-957.[CrossRef][Medline]
  18. Fiorilli R, Argento G, Tomasco B, Serino W. Cor triatriatum dexter diagnosed by transesophageal echocardiography. J Clin Ultrasound 1995; 23:502-504.[Medline]
  19. Trakhtenbroit A, Majaid P, Rokey R. Cor triatriatum dexter: antemortem diagnosis in an adult by cross sectional echocardiography. Br Heart J 1990; 63:314-316.[Abstract/Free Full Text]
  20. Sakamoto I, Matsynaga N, Hayashi K, Ogawa Y, Fukui J. Cine-magnetic resonance imaging of cor triatriatum. Chest 1994; 106:1586-1589.[Abstract/Free Full Text]
  21. Aydogan U, Onursal E, Cantez T, Barlas C, Tanman B, Gurgan L. Giant congenital coronary artery fistula to left superior vena cava and right atrium with compression of left pulmonary vein simulating cor triatriatum-diagnostic value of magnetic resonance imaging. Eur J Cardiothorac Surg 1994; 8:97-99.[Abstract]
  22. Ono Y, Fukui K, Munakata M, et al. Usefulness of the preoperative MRI for diagnosis and operative method in a case of cor triatriatum. Kyobu Geka 1996; 49:921-923. [Japanese].[Medline]
  23. Horike K, Matsumura C, Egawa Y, et al. A case report of cor triatriatum benefit of MRI for preoperative diagnosis and surgical method. Kyobu Geka 1993; 46:1063-1065. [Japanese].[Medline]
  24. Gutierrez FR, Canter CE, Mirowitz SA. MR appearance of congenital heart defects. In: Gutierrez FR, Brown JJ, Mirowitz SA, eds. Cardiovascular magnetic resonance imaging. St Louis, Mo: Mosby–Year Book, 1992; 77-78.
  25. Masui T, Seelos KC, Kersting-Sommerhoff BA, Higgins CB. Abnormalities of the pulmonary veins: evaluation with MR imaging and comparison with cardiac angiography and echocardiography. Radiology 1991; 181:645-649.[Abstract/Free Full Text]
  26. Reichek N, Theobald TM, Malkowski MJ, Rogers WJ, Kramer CM. MRI and echo LV ejection fractions after recent acute myocardial infarction (abstr). Circulation 1997; 96(s):I-189.
  27. Sechtem U, Pflugfelder P, Cassidy MC, Holt W, Wolfe C, Higgins CB. Ventricular septal defect: visualization of shunt flow and determination of shunt size by cine MR imaging. AJR Am J Roentgenol 1987; 149:689-692.[Abstract/Free Full Text]

Congratulations to the 22 individuals who submitted the most likely diagnosis (Cor Triatriatum Dexter) for Diagnosis Please, Case 56. The names and locations of the individuals, as submitted, are as follows:
Philip A. Araoz, MD, Rochester, Minn
Andrew Berkow, MD, Davenport, Iowa
Eric Bressler, MD, Minnetonka, Minn
Peter Buetow, Bellingham, Wash
Marc G. de Baets, MD, Lugano, Switzerland
María Jesús Díaz Candamio, MD, PhD, La Coruña, Spain
Mark Goldshein, MD, Andover, Mass
Bhaskar Golla, Kingston, Pa
Roger Lao, MD, Westbury, NY
John T. Lim, Irvine, Calif
Mike O’Loughlin, MD, West Hartford, Conn
Jeffrey Robinson, MD, Mercer Island, Wash
Dr Pierre Schmit, L’Hay-les-Roses, France
Steven M. Schultz, MD, Fort Worth, Tex
Anthony J. Scuderi, MD, Johnstown, Pa
James D. Sprinkle, Jr, MD, Spotsylvania, Va
Douglas L. Teich, MD, Brookline, Mass
Eugene Tong, MD, Austin, Tex
Zhen Jane Wang, MD, San Francisco, Calif
Tatsuya Yamamoto, Obama, Japan
Benjamin M. Yeh, MD, San Francisco, Calif
Jeffrey H. Zapolsky, Oshkosh, Wis





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