The role of multimodal imaging in the diagnostics of non-compaction cardiomyopathy (a clinical case)
DOI:
https://doi.org/10.14739/2310-1210.2019.2.161514Keywords:
non-compaction cardiomyopathy, echocardiography, magnetic resonance tomographyAbstract
Non-compaction cardiomyopathy is a rare heart disease of non-ischemic origin, which is characterized by increased myocardial trabecularity with deep intertrabecular recessions, which contributes to increased thrombogenicity, loss of myocardial contractility and heart failure manifestation. Despite of the increasing number of publications describing this pathology during last years, this form of cardiomyopathy remains underdiagnosed and little known for a wide range of physicians.
The purpose of the study was to present the role of speckle tracking echocardiography and cardiac magnetic resonance tomography in the differential diagnosis of non-compaction cardiomyopathy.
Results. It was described a clinical case of a 53-year old female with intact coronary arteries, who had been followed up for ten years with a diagnosis of dilated cardiomyopathy after the detection of a left bundle branch block. When performing standard echocardiography, a two-layer structure of the myocardium was found in the apical lateral segments of the left ventricle (LV) with the presence of hypertrabeculated internal layer and diffuse decrease in global LV contractility (three-dimensional ejection fraction 34 %). There was a decrease in LV global longitudinal strain up to 12 % measured by speckle-tracking echocardiography. Analysis of segmental myocardial deformation curves in conditions of left bundle branch block revealed the most prognostically unfavorable dyssynchrony pattern. Cardiac magnetic resonance imaging confirmed the presence of hypertrabecularity with compact to non-compact layer ratio of more than 1/2,5, which verified the diagnosis of non-compaction cardiomyopathy.
Conclusions. The clinical manifestations of non-compaction cardiomyopathy are non-specific, therefore instrumental criteria play an important role in the diagnosis. Cardiac magnetic resonance imaging, three-dimensional and speckle-tracking echocardiography are reliable methods for diagnostics and prediction of this disease outcomes.
References
Jenni, R., Oechslin, E., Schneider, J., Attenhofer Jost., C., & Kaufmann, P. A. (2001). Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart, 86(6), 666–71. doi: 10.1136/heart.86.6.666.
Stanton, C., Bruce, C., Connolly, H., Brady, P., Syed, I., Hodge, D., et al. (2009). Isolated left ventricular noncompaction syndrome. Am J Cardiol., 104(8), 1135–8. doi: 10.1016/j.amjcard.2009.05.062.
Schwartzenberg, S., Sherez, J., Wexler, D., Aviram, G., & Keren, G. (2009). Isolated ventricular non-compaction: an underdiagnosed cause of congestive heart failure. Isr Med Assoc J., 11(7), 426–9.
Chin, T. K., Perloff, J. K., Williams, R. G., Jue, K., & Mohrmann, R. (1990). Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation, 82(2), 507–13. doi: 10.1161/01.CIR.82.2.507
Novo, G., Vernuccio, F., Fazio, G., Grutta, G., Lo Voi, A., Giambanco, S., et al. (2015). Left ventricular non compaction: epidemiology, clinical findings, diagnosis and therapy. Minerva Cardioangiol., 63(3), 239–51.
Zuccarino, F., Vollmer, I., Sanchez, G., Navallas, M., Pugliese, F., & Gayete, A. (2015). Left ventricular noncompaction: imaging findings and diagnostic criteria. AJR Am J Roentgenol., 204(5), 519–30. doi: 10.2214/AJR.13.12326.
Oechslin, E. N., Attenhofer Jost., C. H., Rojas, J. R., Kaufmann, P. A., & Jenni, R. (2000). Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol., 36(2), 493–500. doi: 10.1016/S0735-1097(00)00755-5.
Petersen, S. E., Selvanayagam, J. B., Wiesmann, F., Robson, M. D., Francis, J. M., Anderson, R. H., et al. (2005). Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol., 46(1), 101–5. doi: 10.1016/j.jacc.2005.03.045.
Leenders, G. E., Lumens, J., Cramer, M. J., De Boeck, B. W., Doevendans, P. A., Delhaas, T., & Prinzen F. W. (2012). Septal deformation patterns delineate mechanical dyssynchrony and regional differences in contractility: analysis of patient data using a computer model. Circ Heart Fail, 5(1), 87–96. doi: 10.1161/CIRCHEARTFAILURE.111.962704.
Zemrak, F., Ahlman, M. A., Captur, G., Mohiddin, S. A., Kawel-Boehm, N., Prince, M. R., et al. (2014). The relationship of left ventricular trabeculation to ventricular function and structure over a 9.5-year follow-up: the MESA study. J Am Coll Cardiol., 64(19), 1971–80. doi: 10.1016/j.jacc.2014.08.035.
Anderson, R. H., Jensen, B., Mohun, T. J., Petersen, S. E., Aung, N., Zemrak, F., et al. (2017). Key questions relating to left ventricular noncompaction cardiomyopathy: is the emperor still wearing any clothes? Can J Cardiol., 33(6), 747–757. doi: 10.1016/j.cjca.2017.01.017.
Huttin, O., Venner, C., Frikha, Z., Voilliot, D., Marie, P. Y., Aliot, E., et al. (2014). Myocardial deformation pattern in left ventricular non-compaction: Comparison with dilated cardiomyopathy. Int J Cardiol Heart Vasc., 5, 9–14. doi: 10.1016/j.ijcha.2014.11.001.
Menet, A., Bernard, A., Tribouilloy, C., Leclercq, C., Gevaert, C., Guyomar, Y., et al. (2017). Clinical significance of septal deformation patterns in heart failure patients receiving cardiac resynchronization therapy. Eur Heart J Cardiovasc Imaging, 18(12), 1388–1397. doi: 10.1093/ehjci/jew289.
van Waning, J. I., Caliskan, K., Hoedemaekers, Y. M., van Spaendonck-Zwarts, K. Y., Baas, A. F., Boekholdt, S. M., et al. (2018). Genetics, clinical features, and long-term outcome of noncompaction cardiomyopathy. J Am Coll Cardiol., 71(7), 711–722. doi: 10.1016/j.jacc.2017.12.019.
Fazlinezhad, A., Vojdanparast, M., Sarafan, S., & Nezafati, P. (2016). Echocardiographic characteristics of isolated left ventricular noncompaction. ARYA Atheroscler., 12(5), 243–247
Downloads
How to Cite
Issue
Section
License
Authors who publish with this journal agree to the following terms:- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access)