Rare clinical cases: noncompaction cardiomyopathy concurrent with Ebstein’s anomaly

DOI: https://doi.org/10.29296/25877305-2019-12-11
Download full text PDF
Issue: 
12
Year: 
2019

Professor O. Komissarova, MD(1); N. Rineiskaya(1); T. Sevruk(1); I. Gaidel(1); N. Chakova, Candidate of Biological Sciences(2); S. Niyazova(2) 1-Republican Research and Practical Center «Cardiology», Minsk, Republic of Belarus 2-Institute of Genetics and Cytology, National Academy of Sciences of Belarus, Minsk, Republic of Belarus

The paper describes rare clinical cases of Ebstein’s anomaly concurrent with noncompaction cardiomyopathy and Wolff-Parkinson-White syndrome, which are associated with MYH7 gene mutations. It discusses diagnosis, clinical manifestations, and management tactics in patients with this pathology.

Keywords: 
cardiology
noncompaction cardiomyopathy
Ebstein’s anomaly
Wolf-Parkinson- White syndrome
sarcomere genes
MYH7 gene mutations



It appears your Web browser is not configured to display PDF files. Download adobe Acrobat или click here to download the PDF file.

References: 
  1. Van Engelen K., Postma A., van de Meerakker J. et al. Ebstein’s anomaly may be caused by mutations in the sarcomere protein gene MYH7 // J. Neth. Heart. – 2013; 21 (3): 113–7. DOI: 10.1007/s12471-011-0141-1.
  2. Patra S., Singla V., Kharge J. et al. A patient of Ebstein’s anomaly associated with biventricular noncompaction presented with Wolf Parkinson White syndrome – A rare presentation // J. Cardiovasc. Dis. Res. – 2012; 3 (4): 323–5. DOI: 10.4103/0975-3583.102721.
  3. Attenhofer Jost C., Connolly H., Warnes C. et al. Noncompacted myocardium in Ebstein’s anomaly: Initial description in three patients // J. Am. Soc. Echocardiogr. – 2004; 17 (6): 677–80. DOI: 10.1016/j.echo.2004.02.013.
  4. Sinkovec M., Kozelj M., Podnar T. Familial biventricular myocardial noncompaction associated with Ebstein’s malformation // Int. J. Cardiol. – 2005; 102 (2): 297–302. DOI: 10.1016/j.ijcard.2004.05.033.
  5. Arslan S., Gurlertop H., Gundogdu F. et al. Left ventricular noncompaction and mid-caviter narrowing associated with Ebstein’s anomaly: three-dimensional transthoracic echocardiographic image // Int. J. Cardiol. – 2007; 115 (1): e52–5. DOI: 10.1016/j.ijcard.2006.07.117.
  6. Saltik L., Eroğlu E., Bayrak F. et al. Left ventricular non-compaction associated with Ebstein’s anomaly // J. Turk. Kardiyol. Dern. Ars. – 2009; 37: 269–72.
  7. Oechslin E., Attenhofer Jost C., Rojas J. et al. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis // J. Am. Coll. Cardiol. – 2000; 36 (2): 493–500. PMID: 10933363.
  8. Elliott P., Andersson B., Arbustini E. et al. Classification of the cardiomyopathies: a position statement from the european society of cardiology working group on myocardial and pericardial diseases // J. Eur. Heart. – 2008; 29 (2): 270–6. DOI: 10.1093/eurheartj/ehm342.
  9. Maron B., Towbin J., Thiene G. et al. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention // J. Circulation. – 2006; 113 (14): 1807–16. DOI: 10.1161/CIRCULATIONAHA.106.174287.
  10. Arbustini E., Narula N., Dec G. et al. The MOGE(S) classification of cardiomyopathy for clinicians. // J. Am. Coll. Cardiol. – 2014; 64 (3): 304–18. DOI: 10.1016/j.jacc.2014.05.027.
  11. Arbustini E., Weidemann F., Hall J. Left ventricular noncompaction: a distinct cardiomyopathy or a trait shared by different cardiac diseases? // J. Am. Coll. Cardiol. – 2014; 64 (17): 1840–50. DOI: 10.1016/j.jacc.2014.08.030.
  12. Chin T., Perloff J., Williams R. et al. Isolated noncompaction of left ventricular myocardium. A study of eight cases // J. Circulation. – 1990; 82 (2): 507–13. PMID: 2372897.
  13. Hussein A., Karimianpour A., Collier P. et al. Isolated Noncompaction of the Left Ventricle in Adults // J. Am. Coll. Cardiol. – 2015; 66 (5): 578–85. DOI: 10.1016/j.jacc.2015.06.017.
  14. Arbustini E. et al. Left Ventricular Noncompaction: A Distinct Genetic Cardiomyopathy? // J. Am. Coll. Cardiol. – 2016; 68 (9): 949–66. DOI: 10.1016/j.jacc.2016.05.096.
  15. Roberts J., Gollob M., Young C. et al. Bundle Branch Re-Entrant Ventricular Tachycardia: Novel Genetic Mechanisms in a Life-Threatening Arrhythmia // JACC Clin. Electrophysiol. – 2017; 3 (3): 276–88. DOI: 10.1016/j.jacep.2016.09.019.
  16. Delacretaz E., Ganz L., Soejima K. et al. Multi atrial maco-re-entry circuits in adults with repaired congenital heart disease: entrainment mapping combined with three-dimensional electroanatomic mapping // J. Am. Coll. Cardiol. – 2001; 37 (6): 1665–76. PMID: 11345382.
  17. Towbin J. Left ventricular noncompaction: a new form of heart failure // Heart Fail. Clin. – 2010; 6 (4): 453–69. DOI: 10.1016/j.hfc.2010.06.005.
  18. Caliskan K., Ujvari B., Bauernfeind T. et al. The prevalence of early repolarization in patients with noncompaction cardiomyopathy presenting with malignant ventricular arrhythmias // J. Cardiovasc. Electrophysiol. – 2012; 23 (9): 938–44. DOI: 10.1111/j.1540-8167.2012.02325.x.
  19. Postma A., van Engelen K., van de Meerakker J. et al. Mutations in the sarcomere gene MYH7 in Ebstein anomaly // J. Circ. Cardiovasc. Genet. – 2011; 4 (1): 43–50. DOI: 10.1161/CIRCGENETICS.110.957985.
  20. Budde B., Binner P., Waldmuller S. et al. Noncompaction of the ventricular myocardium is associated with a de novo mutation in the beta-myosin heavy chain gene // J. PLoS One. – 2007; 2 (12): e1362. DOI: 10.1371/journal.pone.0001362.