© 2014, Wiley Periodicals, Inc. DOI: 10.1111/echo.12516
Echocardiography
Cor Triatriatum Dexter, Atrial Septal Defects, and Pulmonary Stenosis—A Rare Association Ziya Simsek, M.D., Yavuzer Koza, M.D., and Hakan Tas, M.D. Faculty of Medicine, Department of Cardiology, Ataturk University, Erzurum, Turkey
Cor triatriatum dexter (CTD) is an extremely rare congenital anomaly in which the right atrium is divided into 2 chambers by a membrane. The estimated incidence of cor triatriatum has been reported as 0.1% of congenital cardiac malformations. The septation of the right atrium in the setting of CTD is the result of failed resorption of the right valve of the sinus venosus. This results in anterolateral and posteromedial portions of the divided right atrium. CTD can be diagnosed at any age, especially if it is incidentally discovered. (Echocardiography 2014;31:E124–E127) Key words: congenital heart disease, cor triatriatum dexter, atrial septal defect, pulmonary stenosis, echocardiography
Case: A 22-year-old woman, with no history of cardiac disease, presented with complaints of dyspnea, palpitation, and near syncope for about 2 years. At admission, physical examination revealed a harsh systolic murmur in the pulmonary area and a grade 2/6 holosystolic murmur at the left sternal border. Her blood pressure was 100/60 mmHg and the pulse was irregular at 110 beats/min. The jugular venous pressure was increased with prominent v waves of tricuspid regurgitation. Chest x-ray revealed increased cardiothoracic ratio. A 12-lead electrocardiogram (Nihon Koden, Tokyo, Japan) showed rSR pattern, atrial fibrillation with about 140 beat/min, and occasional premature ventricular complexes. Transthoracic echocardiogram (TTE) (Vingmed System 7, General Electric, Horten, Norway) revealed the following findings: primum and secundum atrial septal defects (ASDs), a membrane that divides the right atrium into 2 separate chambers, valvular pulmonary stenosis, prominent tricuspid, and mitral regurgitation (Fig. 1, movie clips S1, S2, S3, S4). Dilatation of the right heart chambers and eccentric hypertrophy of the right ventricle were noted. Continuous-wave Doppler revealed a maximum 48 mmHg pressure gradient at the level of pulmonary valve. There was Address for correspondence and reprint requests: Yavuzer Koza, M.D., Ataturk University Faculty of Medicine, Department of Cardiology, Yakutiye, Erzurum 25100, Turkey. Fax: +9004422361301; E-mail: [email protected]
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no obstruction of the tricuspid valve or the inferior vena cava. The dimensions and systolic function of the left ventricle were normal. To confirm the diagnosis and to exclude other associated congenital abnormalities, cardiac computed tomographic imaging (CT) was performed. Cardiac CT (Siemens, Somatom Definition Flash, Siemens, Erlangen, Germany) imaging showed a membrane separating the right atrium into an anterior and a posterior chamber with pulmonary stenosis and poststenotic dilatation (Fig. 2). Discussion: In cor triatriatum dexter (CTD), the right atrium is divided into 2 separate chambers by a membrane which represents the right-sided valve of the embryonic sinus venosus. During embryogenesis, this valve tends to regress into the crista terminalis, the eustachian valve of the inferior vena cava and the thebesian valve of the coronary sinus. Complete persistence of the right sinus valve results in a separation of the right atrium into an inlet portion and an outlet portion. The proximal (inlet) chamber receives superior and inferior vena caval flow, and the distal (outlet) chamber incorporates the right atrial appendage.1,2 Thus, venous flow is directed to the proximal chamber. Echocardiographically, the membrane is usually seen running from the inferior vena cava to the superior vena cava, separating the right atrial appendage and tricuspid valve from the great veins.3 In such cases, it is important to know the echocardiographic appearance of the eustachian valve and its
Triatrial Heart
Figure 1. Transthoracic echocardiography. A. Secundum ASD (arrow) and the membrane (asterisk) that divides right atrium into 2 separate chambers. B. Color flow Doppler image shows the left to right shunt with prominent tricuspid and mitral regurgitation. C. Primum ASD (arrow). D. Subcostal view shows the membrane (arrow). LA = left atrium.
anatomical variants. The eustachian valve originates from the margin of the inferior vena cava, and may show considerable rapid movement within the right atrial cavity. Thus, this structure can be readily identified on cross-sectional echocardiographic examination.4,5 The clinical presentation of CTD is highly variable and dependent on the degree of septation. When the septation is incomplete, the condition often is asymptomatic, usually detected incidentally in adults during surgery or echocardiography for other cardiac conditions. If septation is more
severe and causes obstruction of the tricuspid valve and right ventricle inflow, it can lead to the underdevelopment of the right heart structures such as hypoplastic right ventricle, and pulmonary stenosis or atresia.6 As many patients are asymptomatic this anomaly is often determined at postmortem examination.3 In our case, the symptoms were dyspnea, palpitation, and near syncope. CTD may be associated with tricuspid valve abnormality, interatrial defects, and Ebstein’s anomaly or it can occur as an isolated anomaly.6–8 In our case, there were a primum E125
Simsek, Koza and Tas
Figure 2. Cardiac computed tomographic (CT) imaging. A. Axial maximum intensity projection image shows the right atrium that divided 2 separate chambers (arrow). B. Pulmonary artery stenosis (arrow) and poststenotic dilatation (asterisk). C. Triatrial heart image showing the IVC and SVC drain. RA = right atrium; RV = right ventricle; LA = left atrium; LV = left ventricle; Ao = aorta; SVC = superior venae cava; IVC = inferior venae cava; A1 + A2 = right atrium; A3 = left atrium.
ASD and a secundum ASD with visible left–right interatrial shunt. The pulmonary artery was dilated and pulmonary to systemic flow ratio was estimated to be >2.0. There was also a valvular pulmonary stenosis with a maximum 48 mmHg pressure gradient over the pulmonary valve. CTD can be diagnosed noninvasively by echocardiography, cardiac CT, and magnetic resonance imaging. Our case is particularly interesting because the diagnosis was exclusively echocardiographic and until today CTD associated with E126
primum ASD has not been reported. The management of CTD depends on clinical status. Asymptomatic patients do not require treatment unless they are undergoing cardiac surgery for other reasons. Patients with obstructive symptoms need surgical or percutaneous resection of the membrane.9,10 We recommended surgical treatment primarily for ASD and other cardiac lesions but she and her family denied operation. To the best of our knowledge, this is the first case report or a rare variant of CTD diagnosed by TTE.
Triatrial Heart
References 1. Sarikouch S, Blanz U, Sandica E, et al: Adult congenital heart disease: Cor triatriatum dextrum. J Thorac Cardiovasc Surg 2006;132:164–165. 2. Hansing CE, Young WP, Rowe GG: Cor triatriatum dexter. Persistent right sinus venosus valve. Am J Cardiol 1972;30:559–564. 3. Munoz Castellanos L, Kari Nivon M, Garcia Arenal F, et al: The pathological remnants of the right sinus venosus valve: A presentation of 6 hearts of the cor triatiratum dexter type (inSpanish). Arch Inst Cardiol Mex 1991;61:293–301. 4. Weyman AE: Right ventricular inflow tract. In: Weyman AE (ed.): Principles and Practice of Echocardiography, 2nd ed. Philadelphia, PA: Lea & Febiger, 1994, pp. 824–900. 5. Martınez-Quintana E, Rodrıguez-Gonzalez F, MarreroSantiago H, et al: Cor Triatriatum Dexter versus Prominent Eustachian Valve in an Adult Congenital Heart Disease Patient. Congenit Heart Dis 2012;8:589–591. 6. Lasa JJ, Westover T, Khandelwal M, et al: Cor triatriatum dexter and right ventricular hypoplasia in a fetus. J Ultrasound Med 2011;30:1744–1747. 7. Eroglu ST, Yildirir A, Simsek V, et al: Cor triatriatum dexter, atrial septal defect, and Ebstein’s anomaly in an adult
given a diagnosis by transthoracic and transesophageal echocardiography: A case report. J Am Soc Echocardiogr 2004;17:780–782. 8. Low TT, Uy CC, Wong RC: Unique sail-like structure of cor triatriatum dexter in three-dimensional echocardiogram. Echocardiography 2013; doi: 10.1111/echo.12315. 9. Mazzucco A, Bortolotti U, Gallucci V, et al: Successful repair of symptomatic cor triatriatum dexter in infancy. J Thorac Cardiovasc Surg 1983;85:140–143. 10. Savas V, Samyn J, Schreiber TL, et al: Cor triatriatum dexter: Recognition and percutaneous transluminal correction. Cathet Cardiovasc Diagn 1991;23:183–186.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. for Figure 1A. Movie clip S2. for Figure 1B. Movie clip S3. for Figure 1C. Movie clip S4. for Figure 1D.
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Echocardiography
Cor Triatriatum Dexter, Atrial Septal Defects, and Pulmonary Stenosis—A Rare Association Ziya Simsek, M.D., Yavuzer Koza, M.D., and Hakan Tas, M.D. Faculty of Medicine, Department of Cardiology, Ataturk University, Erzurum, Turkey
Cor triatriatum dexter (CTD) is an extremely rare congenital anomaly in which the right atrium is divided into 2 chambers by a membrane. The estimated incidence of cor triatriatum has been reported as 0.1% of congenital cardiac malformations. The septation of the right atrium in the setting of CTD is the result of failed resorption of the right valve of the sinus venosus. This results in anterolateral and posteromedial portions of the divided right atrium. CTD can be diagnosed at any age, especially if it is incidentally discovered. (Echocardiography 2014;31:E124–E127) Key words: congenital heart disease, cor triatriatum dexter, atrial septal defect, pulmonary stenosis, echocardiography
Case: A 22-year-old woman, with no history of cardiac disease, presented with complaints of dyspnea, palpitation, and near syncope for about 2 years. At admission, physical examination revealed a harsh systolic murmur in the pulmonary area and a grade 2/6 holosystolic murmur at the left sternal border. Her blood pressure was 100/60 mmHg and the pulse was irregular at 110 beats/min. The jugular venous pressure was increased with prominent v waves of tricuspid regurgitation. Chest x-ray revealed increased cardiothoracic ratio. A 12-lead electrocardiogram (Nihon Koden, Tokyo, Japan) showed rSR pattern, atrial fibrillation with about 140 beat/min, and occasional premature ventricular complexes. Transthoracic echocardiogram (TTE) (Vingmed System 7, General Electric, Horten, Norway) revealed the following findings: primum and secundum atrial septal defects (ASDs), a membrane that divides the right atrium into 2 separate chambers, valvular pulmonary stenosis, prominent tricuspid, and mitral regurgitation (Fig. 1, movie clips S1, S2, S3, S4). Dilatation of the right heart chambers and eccentric hypertrophy of the right ventricle were noted. Continuous-wave Doppler revealed a maximum 48 mmHg pressure gradient at the level of pulmonary valve. There was Address for correspondence and reprint requests: Yavuzer Koza, M.D., Ataturk University Faculty of Medicine, Department of Cardiology, Yakutiye, Erzurum 25100, Turkey. Fax: +9004422361301; E-mail: [email protected]
E124
no obstruction of the tricuspid valve or the inferior vena cava. The dimensions and systolic function of the left ventricle were normal. To confirm the diagnosis and to exclude other associated congenital abnormalities, cardiac computed tomographic imaging (CT) was performed. Cardiac CT (Siemens, Somatom Definition Flash, Siemens, Erlangen, Germany) imaging showed a membrane separating the right atrium into an anterior and a posterior chamber with pulmonary stenosis and poststenotic dilatation (Fig. 2). Discussion: In cor triatriatum dexter (CTD), the right atrium is divided into 2 separate chambers by a membrane which represents the right-sided valve of the embryonic sinus venosus. During embryogenesis, this valve tends to regress into the crista terminalis, the eustachian valve of the inferior vena cava and the thebesian valve of the coronary sinus. Complete persistence of the right sinus valve results in a separation of the right atrium into an inlet portion and an outlet portion. The proximal (inlet) chamber receives superior and inferior vena caval flow, and the distal (outlet) chamber incorporates the right atrial appendage.1,2 Thus, venous flow is directed to the proximal chamber. Echocardiographically, the membrane is usually seen running from the inferior vena cava to the superior vena cava, separating the right atrial appendage and tricuspid valve from the great veins.3 In such cases, it is important to know the echocardiographic appearance of the eustachian valve and its
Triatrial Heart
Figure 1. Transthoracic echocardiography. A. Secundum ASD (arrow) and the membrane (asterisk) that divides right atrium into 2 separate chambers. B. Color flow Doppler image shows the left to right shunt with prominent tricuspid and mitral regurgitation. C. Primum ASD (arrow). D. Subcostal view shows the membrane (arrow). LA = left atrium.
anatomical variants. The eustachian valve originates from the margin of the inferior vena cava, and may show considerable rapid movement within the right atrial cavity. Thus, this structure can be readily identified on cross-sectional echocardiographic examination.4,5 The clinical presentation of CTD is highly variable and dependent on the degree of septation. When the septation is incomplete, the condition often is asymptomatic, usually detected incidentally in adults during surgery or echocardiography for other cardiac conditions. If septation is more
severe and causes obstruction of the tricuspid valve and right ventricle inflow, it can lead to the underdevelopment of the right heart structures such as hypoplastic right ventricle, and pulmonary stenosis or atresia.6 As many patients are asymptomatic this anomaly is often determined at postmortem examination.3 In our case, the symptoms were dyspnea, palpitation, and near syncope. CTD may be associated with tricuspid valve abnormality, interatrial defects, and Ebstein’s anomaly or it can occur as an isolated anomaly.6–8 In our case, there were a primum E125
Simsek, Koza and Tas
Figure 2. Cardiac computed tomographic (CT) imaging. A. Axial maximum intensity projection image shows the right atrium that divided 2 separate chambers (arrow). B. Pulmonary artery stenosis (arrow) and poststenotic dilatation (asterisk). C. Triatrial heart image showing the IVC and SVC drain. RA = right atrium; RV = right ventricle; LA = left atrium; LV = left ventricle; Ao = aorta; SVC = superior venae cava; IVC = inferior venae cava; A1 + A2 = right atrium; A3 = left atrium.
ASD and a secundum ASD with visible left–right interatrial shunt. The pulmonary artery was dilated and pulmonary to systemic flow ratio was estimated to be >2.0. There was also a valvular pulmonary stenosis with a maximum 48 mmHg pressure gradient over the pulmonary valve. CTD can be diagnosed noninvasively by echocardiography, cardiac CT, and magnetic resonance imaging. Our case is particularly interesting because the diagnosis was exclusively echocardiographic and until today CTD associated with E126
primum ASD has not been reported. The management of CTD depends on clinical status. Asymptomatic patients do not require treatment unless they are undergoing cardiac surgery for other reasons. Patients with obstructive symptoms need surgical or percutaneous resection of the membrane.9,10 We recommended surgical treatment primarily for ASD and other cardiac lesions but she and her family denied operation. To the best of our knowledge, this is the first case report or a rare variant of CTD diagnosed by TTE.
Triatrial Heart
References 1. Sarikouch S, Blanz U, Sandica E, et al: Adult congenital heart disease: Cor triatriatum dextrum. J Thorac Cardiovasc Surg 2006;132:164–165. 2. Hansing CE, Young WP, Rowe GG: Cor triatriatum dexter. Persistent right sinus venosus valve. Am J Cardiol 1972;30:559–564. 3. Munoz Castellanos L, Kari Nivon M, Garcia Arenal F, et al: The pathological remnants of the right sinus venosus valve: A presentation of 6 hearts of the cor triatiratum dexter type (inSpanish). Arch Inst Cardiol Mex 1991;61:293–301. 4. Weyman AE: Right ventricular inflow tract. In: Weyman AE (ed.): Principles and Practice of Echocardiography, 2nd ed. Philadelphia, PA: Lea & Febiger, 1994, pp. 824–900. 5. Martınez-Quintana E, Rodrıguez-Gonzalez F, MarreroSantiago H, et al: Cor Triatriatum Dexter versus Prominent Eustachian Valve in an Adult Congenital Heart Disease Patient. Congenit Heart Dis 2012;8:589–591. 6. Lasa JJ, Westover T, Khandelwal M, et al: Cor triatriatum dexter and right ventricular hypoplasia in a fetus. J Ultrasound Med 2011;30:1744–1747. 7. Eroglu ST, Yildirir A, Simsek V, et al: Cor triatriatum dexter, atrial septal defect, and Ebstein’s anomaly in an adult
given a diagnosis by transthoracic and transesophageal echocardiography: A case report. J Am Soc Echocardiogr 2004;17:780–782. 8. Low TT, Uy CC, Wong RC: Unique sail-like structure of cor triatriatum dexter in three-dimensional echocardiogram. Echocardiography 2013; doi: 10.1111/echo.12315. 9. Mazzucco A, Bortolotti U, Gallucci V, et al: Successful repair of symptomatic cor triatriatum dexter in infancy. J Thorac Cardiovasc Surg 1983;85:140–143. 10. Savas V, Samyn J, Schreiber TL, et al: Cor triatriatum dexter: Recognition and percutaneous transluminal correction. Cathet Cardiovasc Diagn 1991;23:183–186.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. for Figure 1A. Movie clip S2. for Figure 1B. Movie clip S3. for Figure 1C. Movie clip S4. for Figure 1D.
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