Multimodality imaging of cor triatriatum sinister

    Authors

    Abstract

    **Introduction:** Cor triatriatum sinister (CTS) is a very rare congenital cardiac malformation in which the left atrium (LA) is divided into two chambers by a fold of tissue, a membrane, or a fibromuscular band. The anomaly is usually diagnosed in childhood, but in adult age is less common. Clinical symptoms can mimic mitral stenosis. (1-5) **Case report:** We report a case of 54-year-old woman referred to our hospital for transesophageal echocardiography (TEE). She had in history of dyspnea, headache, dizziness and effort intolerance for five years. Physical examination and laboratory values were unremarkable. Two-dimensional and three-dimensional transesophageal echocardiography revealed fibromembranous structure in the dilated LA (**Figure 1** and **Figure 2**). The membrane attached laterally to the junction of the left upper pulmonic vein and left atrial appendage, and medially to the interatrial septum. The membrane divided LA into two chambers (proximal chamber and distal chamber). Proximal chamber was receiving the pulmonary veins, and distal chamber contained left atrial appendage and mitral valve orifice. We found few fenestrations connecting the two chambers (**Figure 3**). Multislice computed tomography (MSCT) confirmed diagnosis of CTS (**Figure 4**). Coronary angiography revealed normal coronary arteries. The patient was referred to surgery following a TEE and MSCT diagnosis of CTS. The atrial membrane was excised around its periphery. Recovery from the surgery was uneventful and she was asymptomatic on further hospital stay and follow-up. FIGURE 1. Two-dimensional transthoracic echocardiogram parasternal long-axis view showing a fibromuscular membrane dividing the dilated left atrium into two chambers. FIGURE 2. Transesophageal echocardiogram demonstrating a fibromuscular membrane in the left atrium. FIGURE 3. Three-dimensional transesophageal echocardiogram showing membrane in the left atrium. FIGURE 4. Multislice computed tomography showing a membrane dividing the left atrium into two chambers. **Conclusion:** The diagnosis of cor triatriatum sinister is paramount because of possibility of surgical repair with excellent long-term prognosis. 3D TEE is noninvasive method for comprehensive imaging and correct diagnosis of this rare congenital cardiac malformation. Surgical repair is an easy and definitive treatment choice of CTS should be considered in patients with left heart chamber obstruction symptoms.

    Keywords

    cor triatriatum sinister, congenital heart disease, transesophageal echocardiography, computed tomography

    DOI

    https://doi.org/10.15836/ccar2019.105

    Literature

    1. Briasoulis A, Sharma S, Afonso L. A Three-dimensional echocardiographic approach to cor tritariatum. Int J Cardiol. 2015 Feb 1;180:262–3. https://doi.org/10.1016/j.ijcard.2014.11.172
    2. Thakrar A, Shapiro MD, Jassal DS, Neilan TG, King ME, Abbara S. Cor triatriatum: the utility of cardiovascular imaging. Can J Cardiol. 2007 Feb;23(2):143–5. https://doi.org/10.1016/S0828-282X(07)70735-3
    3. Fox K, Achenbach S, Bax J, Cosyns B, Delgado V, Dweck MR, et al. Multimodality imaging in cardiology: a statement on behalf of the Task Force on Multimodality Imaging of the European Association of Cardiovascular Imaging. Eur Heart J. 2019 Feb 7;40(6):553–8. https://doi.org/10.1093/eurheartj/ehy669
    4. Moustafa S, Ejaz N, Momenah T, Alkhaldi A, Zuhairy H, Almoukirish A, et al. Unusual case of cor triatriatum sinister. J Cardiovasc Ultrasound. 2013 Jun;21(2):100–1. https://doi.org/10.4250/jcu.2013.21.2.100
    5. Udovičić M, Biočić S, Vincelj J, Crnogorac M, Sakić I, Starčević B. Tetralogy of Fallot with cor triatriatum dexter in an adult patient: a case report. Congenit Heart Dis. 2013 May-Jun;8(3):E77–80. https://doi.org/10.1111/j.1747-0803.2012.00671.x
    Cardiologia Croatica
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    Multimodality imaging of cor triatriatum sinister

    Extended Abstract
    Issue3-4
    Published
    Pages105-106
    PDF via DOIhttps://doi.org/10.15836/ccar2019.105
    cor triatriatum sinister
    congenital heart disease
    transesophageal echocardiography
    computed tomography

    Authors

    Josip Vincelj*ORCIDInstitute for Cardiovascular Prevention and Rehabilitation, Zagreb, Croatia
    Sandra Jakšić JurinjakORCIDUniversity Hospital Dubrava, Zagreb, Croatia
    Ida VuglecORCIDGeneral Hospital Zabok and Croatian Veterans Hospital, Zabok, Croatia

    *Correspondence email: jvincelj@kbd.hr

    Abstract

    **Introduction:** Cor triatriatum sinister (CTS) is a very rare congenital cardiac malformation in which the left atrium (LA) is divided into two chambers by a fold of tissue, a membrane, or a fibromuscular band. The anomaly is usually diagnosed in childhood, but in adult age is less common. Clinical symptoms can mimic mitral stenosis. (1-5) **Case report:** We report a case of 54-year-old woman referred to our hospital for transesophageal echocardiography (TEE). She had in history of dyspnea, headache, dizziness and effort intolerance for five years. Physical examination and laboratory values were unremarkable. Two-dimensional and three-dimensional transesophageal echocardiography revealed fibromembranous structure in the dilated LA (**Figure 1** and **Figure 2**). The membrane attached laterally to the junction of the left upper pulmonic vein and left atrial appendage, and medially to the interatrial septum. The membrane divided LA into two chambers (proximal chamber and distal chamber). Proximal chamber was receiving the pulmonary veins, and distal chamber contained left atrial appendage and mitral valve orifice. We found few fenestrations connecting the two chambers (**Figure 3**). Multislice computed tomography (MSCT) confirmed diagnosis of CTS (**Figure 4**). Coronary angiography revealed normal coronary arteries. The patient was referred to surgery following a TEE and MSCT diagnosis of CTS. The atrial membrane was excised around its periphery. Recovery from the surgery was uneventful and she was asymptomatic on further hospital stay and follow-up. FIGURE 1. Two-dimensional transthoracic echocardiogram parasternal long-axis view showing a fibromuscular membrane dividing the dilated left atrium into two chambers. FIGURE 2. Transesophageal echocardiogram demonstrating a fibromuscular membrane in the left atrium. FIGURE 3. Three-dimensional transesophageal echocardiogram showing membrane in the left atrium. FIGURE 4. Multislice computed tomography showing a membrane dividing the left atrium into two chambers. **Conclusion:** The diagnosis of cor triatriatum sinister is paramount because of possibility of surgical repair with excellent long-term prognosis. 3D TEE is noninvasive method for comprehensive imaging and correct diagnosis of this rare congenital cardiac malformation. Surgical repair is an easy and definitive treatment choice of CTS should be considered in patients with left heart chamber obstruction symptoms.

    Literature

    1. 1.
      Briasoulis A, Sharma S, Afonso L. A Three-dimensional echocardiographic approach to cor tritariatum. Int J Cardiol. 2015 Feb 1;180:262–3.DOI
    2. 2.
      Thakrar A, Shapiro MD, Jassal DS, Neilan TG, King ME, Abbara S. Cor triatriatum: the utility of cardiovascular imaging. Can J Cardiol. 2007 Feb;23(2):143–5.DOI
    3. 3.
      Fox K, Achenbach S, Bax J, Cosyns B, Delgado V, Dweck MR, et al. Multimodality imaging in cardiology: a statement on behalf of the Task Force on Multimodality Imaging of the European Association of Cardiovascular Imaging. Eur Heart J. 2019 Feb 7;40(6):553–8.DOI
    4. 4.
      Moustafa S, Ejaz N, Momenah T, Alkhaldi A, Zuhairy H, Almoukirish A, et al. Unusual case of cor triatriatum sinister. J Cardiovasc Ultrasound. 2013 Jun;21(2):100–1.DOI
    5. 5.
      Udovičić M, Biočić S, Vincelj J, Crnogorac M, Sakić I, Starčević B. Tetralogy of Fallot with cor triatriatum dexter in an adult patient: a case report. Congenit Heart Dis. 2013 May-Jun;8(3):E77–80.DOI