Non-compact cardiomyopathy, manifested by acute left ventricular failure in the early postpartum period

Authors: Bliznyuk S.A., Alekseev V.G., Lyakhova N.L., Libov I.A., Komarova A.G.

Company: City Clinical Hospital named after S.P. Botkin, Moscow, Russian Federation

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Type:  Clinical Cases


For citation: Bliznyuk S.A., Alekseev V.G., Lyakhova N.L., Libov I.A., Komarova A.G. Non-compact cardiomyopathy, manifested by acute left ventricular failure in the early postpartum period. Creative Cardiology. 2022; 16 (3): 404–12 (in Russ.). DOI: 10.24022/1997-3187-2022-16-3-404-412

Received / Accepted:  02.08.2022 / 30.08.2022

Keywords: non-compact myocardium of the left ventricle pregnancy acute left ventricular failure

Full text:  



Left ventricular non-compaction (LVNC) is a rare cardiomyopathy characterized by thickening of the endocardial layer with the formation of multiple trabeculae and intertrabecular recesses not communicate with the coronary circulation. Diagnosis of the disease is based on the results of echocardiography. In addition to echocardiography, cardiac magnetic resonance imaging is used, which is the method of choice to confirm or rule out LVNC because echocardiography cannot allow proper visualization of the apex in some cases.
The clinical picture of the disease is varied and is determined by thromboembolic syndrome, cardiac arrhythmias and heart failure. Treatment of LVNC is based on the treatment of heart failure, medical and surgical treatment of cardiac arrhythmias and the prevention of thromboembolic complications. Patients with end-stage heart failure need a cardiac transplantation.
We present a clinical case of a 38-year-old patient with acute left ventricular failure in early postpartum period due to non-compact cardiomyopathy. The prevalence of non-compact myocardium of the left ventricle in the population and among pregnant women was analyzed. Diagnostics, treatment and cardiovascular complications in this pathology were considered.


  1. Bhatia N.L., Tajik A.J., Wilansky S., Steidley D.E., Mookadam F. Isolated noncompaction of the left ventricular myocardium in adults: a systematic overview. J. Card. Fail. 2011; 17 (9): 771–8. DOI: 10.1016/j.cardfail.2011.05.002
  2. Oechslin E.N., Attenhofer Jost C.H., Rojas J.R., Kaufmann P.A., Jenni R. 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. DOI: 10.1016/s0735-1097(00)00755-5
  3. Bowles N.E., Bowles K.R., Towbin J.A. The "final common pathway" hypothesis and inherited cardiovascular disease. The role of cytoskeletal proteins in dilated cardiomyopathy. Herz. 2000; 25 (3): 168–75. DOI: 10.1007/s000590050003
  4. Maron B.J., Towbin J.A., Thiene G., Antzelevitch C., Corrado D., Arnett D. 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. Circulation. 2006; 113 (14): 1807–16. DOI: 10.1161/CIRCULATIONAHA.106.174287
  5. Richardson P., McKenna W., Bristow M., Maisch B., Mautner B., O’Connell J. et al. Report of the 1995 World Health Organization /International Society and Federation of cardiology Task Force on the Definition and Classification of Cardiomyopathies. Circulation. 1996; 93 (5): 841–2. 6. Sedmera D., Pexieder T., Vuillemin M. Developmental patterning of the myocardium. Anat. Rec. 2000; 258: 319–37.
  6. Arbustini E., Favalli V., Narula N., Serio A., Grasso M. Left ventricular noncompaction: a distinct genetic cardiomyopathy? J. Am. Coll. Cardiol. 2016; 68 (9): 949–66. DOI: 10.1016/j.jacc.2016. 05.096
  7. Hirono K., Hata Y., Nakazawa M., Momoi N., Tsuji T., Matsuoka T. et al. Clinical and echocardiographic impact of tafazzin variants on dilated cardiomyopathy phenotype in left ventricular noncompaction patients in early infancy. Circ. J. 2018; 82 (10): 2609–18. DOI: 10.1111/cge.13484
  8. Chin T.K., Perloff J.K., Williams R.G., Jue K., Mohrmann R. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation. 1990; 82 (2): 507–13. DOI: 10.1161/01.cir.82.2.507
  9. Kohli S.K., Pantazis A.A., Shah J.S., Adeyemi B., Jackson G., McKenna W.J. et al. Diagnosis of leftventricular non-compaction in patients with leftventricular systolic dysfunction: time for a reappraisal of diagnostic criteria? Eur. Heart J. 2008; 29 (1): 89–95. DOI: 10.1093/eurheartj/ehm481
  10. Gati S., Chandra N., Bennett R.L., Reed M., Kervio G., Panoulas V.F. et al. Increased left ventricular trabeculation in highly trained athletes: do we need more stringent criteria for the diagnosis of left ventricular non-compaction in athletes? Heart. 2013; 99 (6): 401–8. DOI: 10.1136/heartjnl-2012-303418
  11. Gati S., Papadakis M., Van Niekerk N., Reed M., Yeghen T., Sharma S. Increased left ventricular trabeculation in individuals with sickle cell anaemia: physiology or pathology? Int. J. Cardiol. 2013; 168 (2): 1658–60. DOI: 10.1016/j.ijcard.2013.03.039
  12. Nugent A.W., Daubeney P.E., Chondros P., Carlin J.B., Cheung M., Wilkinson L.C. et al. National Australian Childhood Cardiomyopathy Study. The epidemiology of childhood cardiomyopathy in Australia. N. Engl. J. Med. 2003; 348 (17): 1639–46. DOI: 10.1056/NEJMoa021737
  13. Jenni R., Oechslin E., Schneider J., Attenhofer Jost C., Kaufmann P.A. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart. 2001; 86 (6): 666–71. DOI: 10.1136/heart.86.6.666
  14. Stöllberger C., Finsterer J., Blazek G. Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am. J. Cardiol. 2002; 90 (8): 899–902. DOI: 10.1016/s0002-9149(02)02723-6
  15. Petersen S.E., Selvanayagam J.B., Wiesmann F., Robson M.D., Francis J.M., Anderson R.H. et al. Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. J. Am. Coll. Cardiol. 2005; 46 (1): 101–5. DOI: 10.1016/j.jacc.2005.03.045
  16. Jacquier A., Thuny F., Jop B., Giorgi R., Cohen F., Gaubert J.Y. et al. Measurement of trabeculated left ventricular mass using cardiac magnetic resonance imaging in the diagnosis of left ventricular non-compaction. Eur. Heart J. 2010; 31 (9): 1098–104. DOI: 10.1093/eurheartj/ehp595
  17. Oechslin E., Jenni R. Left ventricular non-compaction revisited: a distinct phenotype with genetic heterogeneity? Eur. Heart J. 2011; 32 (12): 1446–56. DOI: 10.1093/eurheartj/ehq508
  18. Bauersachs J., König T., Meer P., Petrie M.C., Hilfiker-Kleiner D., Mbakwem A. et al. Pathophysiology, diagnosis and management of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Study Group on peripartum cardiomyopathy. Eur. J. Heart Failure. 2019; 21 (7): 827–43. DOI: 10.1002/ejhf.1493
  19. Zeppenfeld K., Tfelt-Hansen J., de Riva M., Winkel B.G., Behr E.R., Blom N.A. et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur. Heart J. 2022; 43 (40): 3997–4126. DOI: 10.1093/eurheartj/ehac262
  20. Stöllberger C., Blazek G., Dobias C., Hanafin A., Wegner C., Finsterer J. Frequency of stroke and embolism in left ventricular hypertrabeculation/noncompaction. Am. J. Cardiol. 2011; 108 (7): 1021–3. DOI: 10.1016/j.amjcard.2011.05.039
  21. Blagova O.V., Pavlenko E.V., Varionchik N.V., Nedostup A.V., Sedov V.P., Kogan Е.A. et al. Myocarditis as a legitimate phenomenon in non-compaction myocardium: diagnostics, management and influence on outcomes. Russian Journal of Cardiology. 2018; 2: 44–52 (in Russ.). DOI: 10.15829/1560-4071-2018-2-44-52
  22. Captur G., Nihoyannopoulos P. Left ventricular non-compaction: genetic heterogeneity, diagnosis and clinical course. Int. J. Cardiol. 2010; 140 (2): 145–53. DOI: 10.1016/j.ijcard.2009.07.003
  23. Gati S., Papadakis M., Papamichael N.D., Zaidi A., Sheikh N., Reed M. et al. Reversible de novo left ventricular trabeculations in pregnant women: implications for the diagnosis of left ventricular noncompaction in low-risk populations. Circulation. 2014; 130 (6): 475–83. DOI: 10.1161/CIRCULATIONAHA.114.008554
  24. Aung N., Doimo S., Ricci F., Sanghvi M.M., Pedrosa C., Woodbridge S.P. et al. Prognostic significance of left ventricular noncompaction: systematic review and meta-analysis of observational studies. Circ. Cardiovasc. Imaging. 2020; 13 (1): e009712. DOI: 10.1161/CIRCIMAGING.119 009712
  25. Ross S.B., Jones K., Blanch B., Puranik R., McGeechan K., Barratt A. et al. A systematic review and meta-analysis of the prevalence of left ventricular non-compaction in adults. Eur. Heart J. 2020; 41 (14): 1428–36. DOI: 10.1093/eurheartj/ehz317
  26. Ichida F., Hamamichi Y., Miyawaki T., Ono Y., Kamiya T., Akagi T. et al. Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background. J. Am. Coll. Cardiol. 1999; 34 (1): 233–40. DOI: 10.1016/s0735-1097(99)00170-9

About Authors

  • Svetlana A. Bliznyuk, Cand. Med. Sci., Head of Department, Researcher; ORCID
  • Vladimir G. Alekseev, Dr. Med. Sci., Professor, Therapist; ORCID
  • Nataliya L. Lyakhova, Cand. Med. Sci., Head of Department, Researcher, Cardiologist; ORCID
  • Igor’ A. Libov, Cand. Med. Sci., Senior Researcher; ORCID
  • Anna G. Komarova, Cand. Med. Sci., Deputy Chief Physician, Leading Researcher, Cardiologist; ORCID

Chief Editor

Leo A. Bockeria, MD, PhD, DSc, Professor, Academician of Russian Academy of Sciences, President of Bakoulev National Medical Research Center for Cardiovascular Surgery