A clinical case of isolated non-compact left ventricular myocardium in a 29-year-old patient

Abstract

BACKGROUND: Non-compact left ventricular myocardium is a rare heterogeneous pathology, which in the two-layer structure of the myocardium. There is no generally accepted definition of this form of pathology for both echocardiography and cardiac magnetic resonance imaging. Non-compact left ventricular myocardium can occur at any age and is often asymptomatic. Treatment of non-compact left ventricular myocardium is nonspecific and symptomatic.

DESCRIPTION: This study describes a clinical case of a young patient who was diagnosed with non-compact left ventricular myocardium. The disease debuted in the form of cardiac arrhythmia. Holter monitoring showed frequent ventricular extrasystole and atrioventricular block of the first and second degrees. Moreover, echocardiography revealed a decrease in global contractile function of the left ventricle. The diagnosis was made after magnetic resonance imaging of the heart, which revealed the presence of non-compact myocardium of the anterior, lateral, and inferior walls of the left ventricle in the apical and middle segments. Sustained rhythm disturbances were not induced in electrophysiological research by rapid and programmed stimulation. The patient was prescribed antiarrhythmic therapy, followed by echocardiography Holter monitoring. In the future, the patient needs regular monitoring by a cardiologist.

CONCLUSION: Non-compact left ventricular myocardium should be diagnosed at an early stage, so that life expectancy can be increased owing to timely treatment of heart failure and the use of oral anticoagulants, antiarrhythmic drugs, cardiac resynchronization therapy, cardioverter-defibrillator implantation, and heart transplantation when other treatment options are ineffective.

Keywords

non-compact myocardium of the left ventricle / ventricular extrasystole / echocardiography / Holter monitoring of the electrocardiogram / magnetic resonance imaging

References

[1] Hirono K, Ichida F. Left ventricular noncompaction: a disorder with genotypic and phenotypic heterogeneity-a narrative review. Cardiovasc Diagn Ther. 2022;12(4):495–515. doi: 10.21037/cdt-22-198

[2] Hirono K., Ichida F. Left ventricular noncompaction: a disorder with genotypic and phenotypic heterogeneity-a narrative review // Cardiovasc Diagn Ther. 2022. Vol. 12, N 4. P. 495–515. doi: 10.21037/cdt-22-198

[3] Álvarez Pérez LF, Sandelis Pérez JE, Nieves-Rivera J, et al. Left ventricle non-compaction cardiomyopathy admitted with multiorgan failure: a case report. Cureus. 2020;12(6):e8787. doi: 10.7759/cureus.8787

[4] Álvarez Pérez L.F., Sandelis Pérez J.E., Nieves-Rivera J., et al. Left ventricle non-compaction cardiomyopathy admitted with multiorgan failure: a case report // 2020. Vol. 12, N 6. P. e8787. doi: 10.7759/cureus.8787

[5] Oechslin EN, Attenhofer Jost CH, Rojas JR, 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. doi: 10.1016/s0735-1097(00)00755-5

[6] Oechslin E.N., Attenhofer Jost C.H., Rojas J.R., 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. Vol. 36, N 2. P. 493–500. doi: 10.1016/s0735-1097(00)00755-5

[7] Aras D, Tufekcioglu O, Ergun K, et al. Clinical features of isolated ventricular nonompaction in adults long-term clinical course, echocardiographic properties, and predictors of left ventricular failure. J Card Fail. 2006;12(9):726–733. doi: 10.1016/j.cardfail.2006.08.002

[8] Aras D., Tufekcioglu O., Ergun K., et al. Clinical features of isolated ventricular nonompaction in adults long-term clinical course, echocardiographic properties, and predictors of left ventricular failure // J Card Fail. 2006. Vol. 12, N 9. P. 726–733. doi: 10.1016/j.cardfail.2006.08.002

[9] Barakin AO, Tolstikova TV, Bregel LV, Knjazeva NA. Clinical case: non-compact left and right ventricular myocardium combined with pulmonary arteriovenous bypass. Siberian Medical Review. 2020;(3):15. EDN: QEGIZB doi: 10.20333/2500136-2020-3-106-110

[10] Баракин А.О., Толстикова Т.В., Брегель Л.В., Князева Н.А. Клинический случай: некомпактный миокард левого и правого желудочков сердца в сочетании с легочным артериовенозным шунтированием // Сибирское медицинское обозрение. 2020. № 3. С. 15. EDN: QEGIZB doi: 10.20333/2500136-2020-3-106-110

[11] Asfalou I, Boulaamayl S, Mouine M, et al. Left ventricular noncompaction — a rare form of cardiomyopathy: revelation modes and predictors of mortality in adults through 23 cases. J Saudi Heart Assoc. 2017;29(2):102–109. doi: 10.1016/j.jsha.2016.02.004

[12] Asfalou I., Boulaamayl S., Mouine M., et al. Left ventricular noncompaction — a rare form of cardiomyopathy: revelation modes and predictors of mortality in adults through 23 cases // J Saudi Heart Assoc. 2017. Vol. 29, N 2. P. 102–109. doi: 10.1016/j.jsha.2016.02.004

[13] Di Toro A, Giuliani L, Smirnova A, et al. Myths to debunk: the non-compacted myocardium. Eur Heart J Suppl. 2020;22 (Suppl. L):L6-L10. doi: 10.1093/eurheartj/suaa124

[14] Di Toro A., Giuliani L., Smirnova A., et al. Myths to debunk: the non-compacted myocardium // Eur Heart J Suppl. 2020. Vol. 22, Suppl. L. P. L6–L10. doi: 10.1093/eurheartj/suaa124

[15] Ogah OS, Iyawe EP, Okwunze KF, et al. Left ventricular noncompaction cardiomyopathy: a scoping review. Ann Ib Postgrad Med. 2023;21(2):8–16.

[16] Ogah O.S., Iyawe E.P., Okwunze K.F., et al. Left ventricular noncompaction cardiomyopathy: a scoping review // Ann Ib Postgrad Med. 2023. Vol. 21, N 2. P. 8–16.

[17] Vakhrushev YuA, Vershinina TI, Fedotov PA, et al. Left ventricular noncompaction associated with titin-truncating variants in the TTN gene. Russian Journal of Cardiology. 2020;25(10):63–69. EDN: KVRMDM doi: 10.15829/1560-4071-2020-4027

[18] Вахрушев Ю.А., Вершинина Т.Л., Федотов П.А., и др. Некомпактный миокард левого желудочка, ассоциированный с укорачивающими вариантами в гене тайтина (TTN) // Российский кардиологический журнал. 2020. Т. 25, № 10. С. 63–69. EDN: KVRMDM doi: 10.15829/1560-4071-2020-4027

[19] Gaysyonok OV, Grebenyuk TB, Kurnosov PA, Shatalova IV. Left ventricular non-compaction: an example of a rare disease in clinical practice. The Clinician. 2017;11(4-1):60–64. EDN: ZHGDRV doi: 10.17650/1818-8338-2016-10-4-60-64

[20] Гайсёнок О.В., Гребенюк Т.Б., Курносов П.А., Шаталова И.В. Некомпактный миокард левого желудочка: пример редкого заболевания в клинической практике // Клиницист. 2017. Т. 11, № 4-1. С. 60–64. EDN: ZHGDRV doi: 10.17650/1818-8338-2016-10-4-60-64

[21] Okan T, Lodeen H, Abawkaw M, et al. Left ventricular noncompaction cardiomyopathy in an elderly patient: a case report and literature review. Cureus. 2023;15(4):e38305. doi: 10.7759/cureus.38305

[22] Okan T., Lodeen H., Abawkaw M., et al. Left ventricular noncompaction cardiomyopathy in an elderly patient: a case report and literature review // Cureus. 2023. Vol. 15, N 4. P. e38305. doi: 10.7759/cureus.38305

[23] Oleynikov VE, Donetskaya NA, Vdovkin AV, et al. Non-compact right ventricular myocardium — diagnostic and clinical features: a review. Terapevticheskii arkhiv. 2022;94(4):558–564. EDN: PXIOKR doi: 10.26442/00403660.2022.04.201482

[24] Олейников В.Э., Донецкая Н.А., Вдовкин А.В., и др. Некомпактный миокард правого желудочка — особенности диагностики и клинической картины // Терапевтический архив. 2022. Т. 94, № 4. С. 558–564. EDN: PXIOKR doi: 10.26442/00403660.2022.04.201482

[25] Cantero-Pérez EM, Sobrino-Márquez JM, et al. Severe heart failure due to noncompaction cardiomyopathy: a case report. Transplant Proc. 2013;45(10):3672–3674. doi: 10.1016/j.transproceed.2013.10.018

[26] Cantero-Pérez E.M., Sobrino-Márquez J.M., Adsuar-Gómez A., et al. Severe heart failure due to noncompaction cardiomyopathy: a case report // Transplant Proc. 2013. Vol. 45, N 10. P. 3672–3674. doi: 10.1016/j.transproceed.2013.10.018

[27] Gerecke BJ, Engberding R. Noncompaction cardiomyopathy-history and current knowledge for clinical practice. J Clin Med. 2021;10(11):2457. doi: 10.3390/jcm10112457

[28] Gerecke B., Engberding R. Noncompaction cardiomyopathy-history and current knowledge for clinical practice // J Clin Med. 2021. Vol. 10, N 11. P. 2457. doi: 10.3390/jcm10112457

[29] Lofiego C, Biagini E, Ferlito M, et al. Paradoxical contributions of non-compacted and compacted segments to global left ventricular dysfunction in isolated left ventricular noncompaction. Am J Cardiol. 2006;97(5):738–741. doi: 10.1016/j.amjcard.2005.09.109

[30] Lofiego C., Biagini E., Ferlito M., et al. Paradoxical contributions of non-compacted and compacted segments to global left ventricular dysfunction in isolated left ventricular noncompaction // Am J Cardiol. 2006. Vol. 97, N 5. P. 738–741. doi: 10.1016/j.amjcard.2005.09.109

[31] Stöllberger C, Blazek G, Dobias C, et al. Frequency of stroke and embolism in left ventricular hypertrabeculation/noncompaction. Am J Cardiol. 2011;108(7):1021–1023. doi: 10.1016/j.amjcard.2011.05.039

[32] Stöllberger C., Blazek G., Dobias C., et al. Frequency of stroke and embolism in left ventricular hypertrabeculation/noncompaction // Am J Cardiol. 2011. Vol. 108, N 7. P. 1021–1023. doi: 10.1016/j.amjcard.2011.05.039

[33] van Waning J, Caliskan K, Hoedemaekers YM, et al. Genetics, clinical features, and long-term outcome of noncompaction cardiomyopathy. J Am Coll Cardiol. 2018;71(7):711–722. doi: 10.1016/j.jacc.2017.12.019

[34] van Waning J.I., Caliskan K., Hoedemaekers Y.M., et al. Genetics, clinical features, and long-term outcome of noncompaction cardiomyopathy/ / J Am Coll Cardiol. 2018. Vol. 71, N 7. P. 711–722. doi: 10.1016/j.jacc.2017.12.019

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