Cor triatriatum dexter is a rare cardiac abnormality in which the right atrium is subdivided into two distinct chambers. This anomaly is generally attributed to the persistence of the right sinus venosus valve and it is frequently associated with severe malformations of other right heart structures.
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. 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. 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. The valve usually regresses by the 12th week of gestation leaving behind the crista terminalis superiorly and the eustachian valve of the inferior vena cava and the thebesian valve of the coronary sinus inferiorly. There can be remnants or patritions. There are those with filigreed weblike networks as remnants of the right valve (also known as Chiari network) and those with a persistent partition between the venous (smooth) and trabeculated portions of the right atrium - cor triatriatum dexter.
Once the diagnosis is established, the condition is amenable to a relatively simple surgical correction.
References:
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.
Schutte DA, Rowland DG, Allen HD, Bharati S. Prominent venous valves in hypoplastic right hearts. Am Heart J 1997; 134:527-531.
Anderson RH. Understanding the nature of congenital division of the atrial chambers. Br Heart J 1992; 68:1-3.
Ott DA, Cooley DA, Angelini P, Leachman RD. Successful surgical correction of symptomatic cor triatriatum dexter. J Thorac Cardiovasc Surg 1979; 78:573-575.
Yater WM. Variations and anomalies of the venous valves of the right atrium of the human heart. Arch Pathol 1929; 7:418-441.
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.
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.
Hansing CE, Young WP, Rowe GG. Cor triatriatum dexter: persistent right sinus venosus valve. Am J Cardiol 1972; 30:559-564.
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.
Alboliras ET, Edwards WD, Driscoll DJ, Seward JB. Cor triatriatum dexter: two-dimensional echocardiographic diagnosis. J Am Coll Cardiol 1987; 9:334-337.
Burton DA, Chin A, Weinber PM, Pigott JD. Identification of cor triatriatum dexter by two-dimensional echocardiography. Am J Cardiol 1987; 60:409-410.
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.
Fiorilli R, Argento G, Tomasco B, Serino W. Cor triatriatum dexter diagnosed by transesophageal echocardiography. J Clin Ultrasound 1995; 23:502-504.
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.
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