© 2014, Wiley Periodicals, Inc. DOI: 10.1111/echo.12660
Echocardiography
Multimodality Imaging of Cor Triatriatum Sinister in an Octagenerian it C € z, M.D.,* Fatih Yılmaz, M.D.,* Cem Dog an, M.D.,* Tahir Bezgin, M.D.,* Yig ß anga, M.D.,† Ali Karago Ali Elveran, M.D.,* and Cihangir Kaymaz, M.D.* *Cardiology Clinic, Kartal Kosßuyolu Heart & Research Hospital, Istanbul, Turkey; and †Cardiology Clinic, Kartal Yavuz Selim State Hospital, Istanbul, Turkey
Cor triatriatum sinister (CTS) is a rare congenital cardiac anomaly in which a fibromuscular membrane divides the atrium into two chambers. As CTS can occur as an isolated anomaly, it is frequently associated with other cardiac anomalies. Although symptoms are usually encountered in infancy, CTS may rarely present in adulthood when the membrane contains large fenestration or other escape drainage exists. We herein, present an 82-year-old patient with a typical late diagnosis of asymptomatic CTS. (Echocardiography 2014;31:E254–E256) Key words: Cor triatriatum, elderly, left atrium
Case Description: An 82-year-old-woman presented with dyspnoea on exertion and palpitation. She had history of hypertension. Blood pressure was 130/80 mmHg and the heart rate was regular at 85 beats/min. Cardiac auscultation revealed normal S1, S2, and grade 3/6 systolic murmur at the apex. Transthoracic echocardiographic (TTE) (Philips Medical Systems, Andover, MA, USA) examination revealed a membrane dividing the left atrium (LA) into two chambers (Fig. 1). Dilated left atrium (LA diameter 48 mm, volume 84 mL), increased left ventricle (LV) wall thickness, preserved LV systolic function, normal LV dimensions, and kinesis with a type-I diastolic dysfunction were noted. Color Doppler revealed moderate mitral and mild tricuspid regurgitation, with an estimated systolic pressure of 45 mmHg (Fig. 2). The transesophageal echocardiography (TEE), which included real time three-dimensional (RT3D) (Philips Medical Systems) views, demonstrated the anatomy of the membrane more detailed with the location, shape, and size of its fenestration. The membrane attached near the left atrial appendage at the junction of the left atrial appendage and left upper pulmonary vein (called the “warfarin ridge”) (Figs. 3–5, movie clips S1–S3). The “warfarin Address for correspondence and reprint requests: Tahir Bezgin, M.D., Kartal Kosßuyolu Heart Research Hospital, Denizer Cad. 34846 Cevizli Kartal, _Istanbul, Turkey. Fax: +90 216 459 63 21; E-mail: [email protected]
E254
ridge” seemed to continue on across the LA as a continuous ridge. One fenestration of 18 mm connected the two chambers and an echo color Doppler demonstrated a flow through this fenestration. In essence, the membrane divided the LA into inferior and superior chambers. Doppler interrogation of the LA confirmed that neither presence of a significant gradient across the membrane nor across the mitral valve. To explain the etiology of dyspnea (to disclose if any lung parenchymal disease), we ordered thorax CT (Aquilion, Toshiba Medical Systems, Tokyo, Japan) which also has cardiac
Figure 1. Transthoracic echocardiogram in the four-chamber view, revealing the membrane bisecting the left atrium (arrow).
Cor Triatriatum Sinister
Figure 2. Mild tricuspid regurgitation with estimated pulmonary artery systolic pressure 45 mmHg in the transthoracic apical four-chamber view.
Figure 3. Still image of 2DTEE showing the membrane (arrow) consistent with cor triatriatum and its relationship with the left atrial appendage, warfarin ridge, and the left upper pulmonary vein. TEE = transesophageal echocardiography.
Figure 5. Still image of the 3DTEE depicting the membrane (arrow). TEE = transesophageal echocardiography.
Figure 6. Cardiac CT finding of cor triatriatum sinister. A membranous structure is dividing the left atrium by two chambers (arrow).
In conclusion, it was felt that the patient had nonobstructive cor triatriatum sinister (CTS) and decided to continue medical therapy which she has been taking for 15 years (ramipril 2.5 mg + hydrochlorothiazide 12.5 mg [o.d.], metoprolol succinate 50 mg [o.d.], and ASA 100 mg [o.d.]).
Figure 4. Color Doppler, 2DTEE showing moderate degree mitral insufficiency. TEE = transesophageal echocardiography.
CT capability confirmed echocardiography revealing a membrane dividing the LA into two chambers (Fig. 6).
Discussion: Cor triatriatum sinister is an infrequent (0.1%) congenital heart disease characterized by an abnormal fibromuscular diaphragm in the LA because of the failure of resorption of the common pulmonary vein.1 As CTS can occur as an isolated anomaly, it is frequently associated with other cardiac anomalies. Although symptoms are usually encountered in infancy, it may rarely present in adulthood when the membrane contains E255
Bezgin, et al.
large fenestration or other escape drainage exists. The location of the atrial membrane differentiates cor triatriatum from a supravalvular mitral ring. CTS is differentiated from supravalvular mitral ring by the position of the LAA. In CTS, the LAA is part of the distal (mitral valve) atrial chamber, whereas the LAA is part of the proximal (pulmonary vein) atrial chamber in patient with a supravalvular ring. Although TTE alone can diagnose most cases of CTS, TEE has the advantage of better visualizing the LA, the LA appendage, the membrane, and the pulmonary veins, hence providing higher sensitivity.2 The real time 3D echocardiography provides improved spatial orientation, thus permitting optimal evaluation of the anatomical features of the separating membrane such as number and size of its orifices. In fact, 3D echocardiography is the only diagnostic tool which allows direct visualization and planimetric measurement of the fenestrations. Finally, it provides additional information to cardiologists and surgeons by showing how the malformation is related to the residual parts of the heart.3–5 Cardiac CT and MRI provide better spatial resolution and superior tissue contrast compared with echocardiography.6–8 The physiologic consequences of CTS are directly related to the size of the orifice between the accessory and the true chamber. When the foramen is small, the obstruction is sufficient to create a pressure gradient within the atrium, thus mimicking mitral stenosis. The most common presenting symptoms in adults are dyspnea, hemoptysis, and orthopnea. Relevant symptoms could also be related to accompanying cardiac abnormalities.8 Patients with obstructive symptoms at any age should undergo surgical correction or percutaneous balloon dilatation of the fenestrations.9 Most of the younger patients need surgical intervention because they are more likely to have significant degrees of transmembrane flow obstruction.10 The medical treatment in CTS depends on symptoms. An asymptomatic patient with CTS having no pressure gradient across the membrane does not require medical management. When exertional dyspnea and signs of pulmonary congestion occur, diuretics and preload reduction are the mainstay of medical therapy. Echocardiographic follow-up of asymptomatic patients with nonobstructive CTS is essential because of progressive calcification, fibrosis of membrane, and possible obstruction with time.
E256
We have used 3DTEE and thorax CT to visualize structures within the heart in more detail than that afforded by 2DTTE and TEE. In summary, CTS is a rare adult congenital heart defect that is typically an incidental finding identified by various imaging modalities, typically echocardiography. The case of our 82-year-old patient is typical of late diagnosis of asymptomatic CTS. References 1. van Son JA, Danielson GK, Schaff HV, et al: Cor triatriatum: Diagnosis, operative approach, and late results. Mayo Clin Proc 1993;68:854–859. 2. Low TT, Uy CC, Wong RC: Unique sail-like structure of Cor Triatriatum Dexter in three-dimensional echocardiogram. Echocardiography 2013;0:E1–E.3. 3. Einav E, Perk G, Kronzon I: Three-dimensional transthoracic echocardiographic evaluation of cor triatriatum. Eur J Echocardiogr 2008;9:110–112. 4. Hanna R, Chen MA, Gill EA: Cor triatriatum evaluated by real time 3D TEE. Echocardiography 2011;28:E125–E128. 5. D’Aloia A., Vizzardi E., Caretta G., et al: Diagnosis of Cor Triatriatum Sinister in patient with pulmonary edema and severe pulmonary arterial hypertension: Assessment by three-dimensional transesophageal echocardiography. Echocardiography 2011;28:E198–E201. 6. Su CS, Tsai IC, Lin WW, et al: Usefulness of multidetector-row computed tomography in evaluating adult cor triatriatum. Tex Heart Inst J 2008;35:349–351. 7. Thakrar A, Shapiro MD, Jassal DS, et al: Cor triatriatum: The utility of cardiovascular imaging. Can J Cardiol 2007;23:143–145. 8. Nassar PN, Hamdan RH: Cor Triatriatum Sinistrum: Classification and imaging modalities. Eur J Cardiovasc Med 2011;1:84–87. 9. Alphonso N, Norgaard MA, Newcomb A, et al: Cor triatriatum: Presentation, diagnosis and long-term surgical results. Ann Thorac Surg 2005;80:1666–1671. 10. Slight RD, Nzewi OC, Buell R, et al: Cor-triatriatum sinister presenting in the adult as mitral stenosis: An analysis of factors which may be relevant in late presentation. Heart Lung Circ 2005;14:8–12.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. Transthoracic apical fourchamber view shows the membrane dividing the left atrium into two chambers. Movie clip S2. Transesophageal 2D study showing the membrane dividing the left atrium into two chambers and a fenestration on the membrane. Movie clip S3. 3DTEE image with left atrial perspective demonstrated the membrane. 3DTEE = three-dimensional transesophageal echocardiography.
Echocardiography
Multimodality Imaging of Cor Triatriatum Sinister in an Octagenerian it C € z, M.D.,* Fatih Yılmaz, M.D.,* Cem Dog an, M.D.,* Tahir Bezgin, M.D.,* Yig ß anga, M.D.,† Ali Karago Ali Elveran, M.D.,* and Cihangir Kaymaz, M.D.* *Cardiology Clinic, Kartal Kosßuyolu Heart & Research Hospital, Istanbul, Turkey; and †Cardiology Clinic, Kartal Yavuz Selim State Hospital, Istanbul, Turkey
Cor triatriatum sinister (CTS) is a rare congenital cardiac anomaly in which a fibromuscular membrane divides the atrium into two chambers. As CTS can occur as an isolated anomaly, it is frequently associated with other cardiac anomalies. Although symptoms are usually encountered in infancy, CTS may rarely present in adulthood when the membrane contains large fenestration or other escape drainage exists. We herein, present an 82-year-old patient with a typical late diagnosis of asymptomatic CTS. (Echocardiography 2014;31:E254–E256) Key words: Cor triatriatum, elderly, left atrium
Case Description: An 82-year-old-woman presented with dyspnoea on exertion and palpitation. She had history of hypertension. Blood pressure was 130/80 mmHg and the heart rate was regular at 85 beats/min. Cardiac auscultation revealed normal S1, S2, and grade 3/6 systolic murmur at the apex. Transthoracic echocardiographic (TTE) (Philips Medical Systems, Andover, MA, USA) examination revealed a membrane dividing the left atrium (LA) into two chambers (Fig. 1). Dilated left atrium (LA diameter 48 mm, volume 84 mL), increased left ventricle (LV) wall thickness, preserved LV systolic function, normal LV dimensions, and kinesis with a type-I diastolic dysfunction were noted. Color Doppler revealed moderate mitral and mild tricuspid regurgitation, with an estimated systolic pressure of 45 mmHg (Fig. 2). The transesophageal echocardiography (TEE), which included real time three-dimensional (RT3D) (Philips Medical Systems) views, demonstrated the anatomy of the membrane more detailed with the location, shape, and size of its fenestration. The membrane attached near the left atrial appendage at the junction of the left atrial appendage and left upper pulmonary vein (called the “warfarin ridge”) (Figs. 3–5, movie clips S1–S3). The “warfarin Address for correspondence and reprint requests: Tahir Bezgin, M.D., Kartal Kosßuyolu Heart Research Hospital, Denizer Cad. 34846 Cevizli Kartal, _Istanbul, Turkey. Fax: +90 216 459 63 21; E-mail: [email protected]
E254
ridge” seemed to continue on across the LA as a continuous ridge. One fenestration of 18 mm connected the two chambers and an echo color Doppler demonstrated a flow through this fenestration. In essence, the membrane divided the LA into inferior and superior chambers. Doppler interrogation of the LA confirmed that neither presence of a significant gradient across the membrane nor across the mitral valve. To explain the etiology of dyspnea (to disclose if any lung parenchymal disease), we ordered thorax CT (Aquilion, Toshiba Medical Systems, Tokyo, Japan) which also has cardiac
Figure 1. Transthoracic echocardiogram in the four-chamber view, revealing the membrane bisecting the left atrium (arrow).
Cor Triatriatum Sinister
Figure 2. Mild tricuspid regurgitation with estimated pulmonary artery systolic pressure 45 mmHg in the transthoracic apical four-chamber view.
Figure 3. Still image of 2DTEE showing the membrane (arrow) consistent with cor triatriatum and its relationship with the left atrial appendage, warfarin ridge, and the left upper pulmonary vein. TEE = transesophageal echocardiography.
Figure 5. Still image of the 3DTEE depicting the membrane (arrow). TEE = transesophageal echocardiography.
Figure 6. Cardiac CT finding of cor triatriatum sinister. A membranous structure is dividing the left atrium by two chambers (arrow).
In conclusion, it was felt that the patient had nonobstructive cor triatriatum sinister (CTS) and decided to continue medical therapy which she has been taking for 15 years (ramipril 2.5 mg + hydrochlorothiazide 12.5 mg [o.d.], metoprolol succinate 50 mg [o.d.], and ASA 100 mg [o.d.]).
Figure 4. Color Doppler, 2DTEE showing moderate degree mitral insufficiency. TEE = transesophageal echocardiography.
CT capability confirmed echocardiography revealing a membrane dividing the LA into two chambers (Fig. 6).
Discussion: Cor triatriatum sinister is an infrequent (0.1%) congenital heart disease characterized by an abnormal fibromuscular diaphragm in the LA because of the failure of resorption of the common pulmonary vein.1 As CTS can occur as an isolated anomaly, it is frequently associated with other cardiac anomalies. Although symptoms are usually encountered in infancy, it may rarely present in adulthood when the membrane contains E255
Bezgin, et al.
large fenestration or other escape drainage exists. The location of the atrial membrane differentiates cor triatriatum from a supravalvular mitral ring. CTS is differentiated from supravalvular mitral ring by the position of the LAA. In CTS, the LAA is part of the distal (mitral valve) atrial chamber, whereas the LAA is part of the proximal (pulmonary vein) atrial chamber in patient with a supravalvular ring. Although TTE alone can diagnose most cases of CTS, TEE has the advantage of better visualizing the LA, the LA appendage, the membrane, and the pulmonary veins, hence providing higher sensitivity.2 The real time 3D echocardiography provides improved spatial orientation, thus permitting optimal evaluation of the anatomical features of the separating membrane such as number and size of its orifices. In fact, 3D echocardiography is the only diagnostic tool which allows direct visualization and planimetric measurement of the fenestrations. Finally, it provides additional information to cardiologists and surgeons by showing how the malformation is related to the residual parts of the heart.3–5 Cardiac CT and MRI provide better spatial resolution and superior tissue contrast compared with echocardiography.6–8 The physiologic consequences of CTS are directly related to the size of the orifice between the accessory and the true chamber. When the foramen is small, the obstruction is sufficient to create a pressure gradient within the atrium, thus mimicking mitral stenosis. The most common presenting symptoms in adults are dyspnea, hemoptysis, and orthopnea. Relevant symptoms could also be related to accompanying cardiac abnormalities.8 Patients with obstructive symptoms at any age should undergo surgical correction or percutaneous balloon dilatation of the fenestrations.9 Most of the younger patients need surgical intervention because they are more likely to have significant degrees of transmembrane flow obstruction.10 The medical treatment in CTS depends on symptoms. An asymptomatic patient with CTS having no pressure gradient across the membrane does not require medical management. When exertional dyspnea and signs of pulmonary congestion occur, diuretics and preload reduction are the mainstay of medical therapy. Echocardiographic follow-up of asymptomatic patients with nonobstructive CTS is essential because of progressive calcification, fibrosis of membrane, and possible obstruction with time.
E256
We have used 3DTEE and thorax CT to visualize structures within the heart in more detail than that afforded by 2DTTE and TEE. In summary, CTS is a rare adult congenital heart defect that is typically an incidental finding identified by various imaging modalities, typically echocardiography. The case of our 82-year-old patient is typical of late diagnosis of asymptomatic CTS. References 1. van Son JA, Danielson GK, Schaff HV, et al: Cor triatriatum: Diagnosis, operative approach, and late results. Mayo Clin Proc 1993;68:854–859. 2. Low TT, Uy CC, Wong RC: Unique sail-like structure of Cor Triatriatum Dexter in three-dimensional echocardiogram. Echocardiography 2013;0:E1–E.3. 3. Einav E, Perk G, Kronzon I: Three-dimensional transthoracic echocardiographic evaluation of cor triatriatum. Eur J Echocardiogr 2008;9:110–112. 4. Hanna R, Chen MA, Gill EA: Cor triatriatum evaluated by real time 3D TEE. Echocardiography 2011;28:E125–E128. 5. D’Aloia A., Vizzardi E., Caretta G., et al: Diagnosis of Cor Triatriatum Sinister in patient with pulmonary edema and severe pulmonary arterial hypertension: Assessment by three-dimensional transesophageal echocardiography. Echocardiography 2011;28:E198–E201. 6. Su CS, Tsai IC, Lin WW, et al: Usefulness of multidetector-row computed tomography in evaluating adult cor triatriatum. Tex Heart Inst J 2008;35:349–351. 7. Thakrar A, Shapiro MD, Jassal DS, et al: Cor triatriatum: The utility of cardiovascular imaging. Can J Cardiol 2007;23:143–145. 8. Nassar PN, Hamdan RH: Cor Triatriatum Sinistrum: Classification and imaging modalities. Eur J Cardiovasc Med 2011;1:84–87. 9. Alphonso N, Norgaard MA, Newcomb A, et al: Cor triatriatum: Presentation, diagnosis and long-term surgical results. Ann Thorac Surg 2005;80:1666–1671. 10. Slight RD, Nzewi OC, Buell R, et al: Cor-triatriatum sinister presenting in the adult as mitral stenosis: An analysis of factors which may be relevant in late presentation. Heart Lung Circ 2005;14:8–12.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. Transthoracic apical fourchamber view shows the membrane dividing the left atrium into two chambers. Movie clip S2. Transesophageal 2D study showing the membrane dividing the left atrium into two chambers and a fenestration on the membrane. Movie clip S3. 3DTEE image with left atrial perspective demonstrated the membrane. 3DTEE = three-dimensional transesophageal echocardiography.
