Right ventricular thrombi — suspicion of arrhythmogenic right ventricular dysplasia: a case report

    Authors

    Keywords

    dyspnea, syncope, cardiac arrhythmia, thrombus, arrythmogenic right ventricular cardiomyopathy

    DOI

    https://doi.org/10.15836/ccar.2015.81

    Full Text

    ## Case report 61-year-old woman with long history of hypertension presented with progressive dyspnea and chest pain that she has been experiencing at minor physical exertion for several months. Current medical records confirmed T-wave inversion in right precordial leads, attacks of supraventricular and ventricular arrhythmia, including attacks of non-sustained ventricular tachycardia and reccurent syncopal episodes from the age of 23. Dilated right heart chamber are detected by transthoracic echocardiogram year 2006, presence of the shunt was excluded by scintigraphy. ## Imaging studies 2-dimensional transthoracic echocardiogram revealed one large (Figure 1) and two smaller hyperechogenic masses (Figure 2) inside extremely dilated (Figure 3) and globally hypokinetic right ventricle with moderate tricuspid regurgitation. 3-dimensional transesophageal echocardiogram confirmed mobile pedunculated mass by free wall of right ventricle (Figure 4). Magnetic resonance imaging describe dilated right heart chambers, hypertrophic septomarginal trabeculae of the mid-apical part of right ventricular and mass within the same chamber appears to be a tumour. CT pulmonary angiogram, PET CT and coronarography ruled out specific pathomorphological substrate. Figure 1. 2-dimensional transthoracic echocardiogram - PLAX: large hyperechogenic mass (4.2 x 2 cm) inside right ventricle. Figure 2. 2-dimensional transthoracic echocardiogram - PLAX: one large and two smaller hyperechogenic masses inside right ventricle. Figure 3. 2-dimensional transthoracic echoardiogram - PLAX: diameter of right ventricle (47.6 mm). Figure 4. 3-dimensional transesophageal echocardiogram: mobile pedunculated mass by free wall of right ventricle. ## Treatment Three large individual masses were surgically removed from the chamber of right ventricle (Figure 5). Annuloplasty of the tricuspid valve was performed. Figure 5. Three large individual masses that were surgically removed from the chamber of right ventricle. Pathohistological diagnosis: focal-organizing thrombi. ## Conclusion Although final diagnosis has not been made, it is possible that the patient suffers from arrhythmogenic right ventricular dysplasia. This conclusion is based on the presence of three big McKenna’s criteria (1) (T-wave inversion in right precordial leads, ECHO-PLAX RVOT >32 mm and non-sustained ventricular tachycardia detected during Holter monitoring) and the fact that literary sources describe several cases of thrombotic masses in right heart chambers in patients with the same diagnosis. (2, 3)

    Literature

    1. Marcus FI, McKenna WJ, Sherrill D, Basso C, Bauce B, Bluemke DA, et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria. Eur Heart J. 2010;31(7):806–14. https://doi.org/10.1093/eurheartj/ehq025
    2. Wlodarska EK, Wozniak O, Konka M, Rydlewska-Sadowska W, Biederman A, Hoffman P. Thromboembolic complications in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Europace. 2006;8(8):596–600. https://doi.org/10.1093/europace/eul053
    3. Pinamonti B, Dragos AM, Pyxaras SA, Merlo M, Pivetta A, Barbati G, et al. Prognostic predictors in arrhythmogenic right ventricular cardiomyopathy: results from a 10-year registry. Eur Heart J. 2011;32(9):1105–13. https://doi.org/10.1093/eurheartj/ehr040
    Cardiologia Croatica
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    Right ventricular thrombi — suspicion of arrhythmogenic right ventricular dysplasia: a case report

    Abstract
    Issue3-4
    Published
    Pages81-82
    PDF via DOIhttps://doi.org/10.15836/ccar.2015.81
    dyspnea
    syncope
    cardiac arrhythmia
    thrombus
    arrythmogenic right ventricular cardiomyopathy

    Authors

    Livija Susic*ORCIDHealth Centre Osijek, Osijek, Croatia
    Vedrana BarabanORCIDJosip Juraj Strossmayer University of Osijek School of Medicine, University Hospital Centre Osijek, Osijek, Croatia
    Josip VinceljORCIDDubrava University Hospital, Zagreb, Croatia
    Jasmina CaticORCIDDubrava University Hospital, Zagreb, Croatia
    Robert BlazekovicORCIDDubrava University Hospital, Zagreb, Croatia

    *Correspondence email: livija.susic@gmail.com

    Full Text

    Case report

    61-year-old woman with long history of hypertension presented with progressive dyspnea and chest pain that she has been experiencing at minor physical exertion for several months. Current medical records confirmed T-wave inversion in right precordial leads, attacks of supraventricular and ventricular arrhythmia, including attacks of non-sustained ventricular tachycardia and reccurent syncopal episodes from the age of 23. Dilated right heart chamber are detected by transthoracic echocardiogram year 2006, presence of the shunt was excluded by scintigraphy.

    Imaging studies

    2-dimensional transthoracic echocardiogram revealed one large (Figure 1) and two smaller hyperechogenic masses (Figure 2) inside extremely dilated (Figure 3) and globally hypokinetic right ventricle with moderate tricuspid regurgitation. 3-dimensional transesophageal echocardiogram confirmed mobile pedunculated mass by free wall of right ventricle (Figure 4). Magnetic resonance imaging describe dilated right heart chambers, hypertrophic septomarginal trabeculae of the mid-apical part of right ventricular and mass within the same chamber appears to be a tumour. CT pulmonary angiogram, PET CT and coronarography ruled out specific pathomorphological substrate.

    Figure 1. 2-dimensional transthoracic echocardiogram - PLAX: large hyperechogenic mass (4.2 x 2 cm) inside right ventricle.

    Figure 2. 2-dimensional transthoracic echocardiogram - PLAX: one large and two smaller hyperechogenic masses inside right ventricle.

    Figure 3. 2-dimensional transthoracic echoardiogram - PLAX: diameter of right ventricle (47.6 mm).

    Figure 4. 3-dimensional transesophageal echocardiogram: mobile pedunculated mass by free wall of right ventricle.

    Treatment

    Three large individual masses were surgically removed from the chamber of right ventricle (Figure 5). Annuloplasty of the tricuspid valve was performed.

    Figure 5. Three large individual masses that were surgically removed from the chamber of right ventricle.

    Pathohistological diagnosis: focal-organizing thrombi.

    Conclusion

    Although final diagnosis has not been made, it is possible that the patient suffers from arrhythmogenic right ventricular dysplasia. This conclusion is based on the presence of three big McKenna’s criteria (1) (T-wave inversion in right precordial leads, ECHO-PLAX RVOT >32 mm and non-sustained ventricular tachycardia detected during Holter monitoring) and the fact that literary sources describe several cases of thrombotic masses in right heart chambers in patients with the same diagnosis. (2, 3)

    Literature

    1. 1.
      Marcus FI, McKenna WJ, Sherrill D, Basso C, Bauce B, Bluemke DA, et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria. Eur Heart J. 2010;31(7):806–14.DOI
    2. 2.
      Wlodarska EK, Wozniak O, Konka M, Rydlewska-Sadowska W, Biederman A, Hoffman P. Thromboembolic complications in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Europace. 2006;8(8):596–600.DOI
    3. 3.
      Pinamonti B, Dragos AM, Pyxaras SA, Merlo M, Pivetta A, Barbati G, et al. Prognostic predictors in arrhythmogenic right ventricular cardiomyopathy: results from a 10-year registry. Eur Heart J. 2011;32(9):1105–13.DOI