The Forgotten Spongy Myocardium: Clinical Trajectory of Left Ventricular Noncompaction Cardiomyopathy in an Asymptomatic Adult

Leonardo Paskah Suciadi; Dony Yugo Hermanto; Surya Sinaga Immanuel; Jason Wirandy Haryanto; Harvian Satya Dharma

doi:10.30701/ijc.2180

Leonardo Paskah Suciadi Siloam Heart Institute, Siloam Hospitals Kebon Jeruk, Jakarta, Indonesia https://orcid.org/0000-0001-8986-9068
Dony Yugo Hermanto Division of Arrhythmia and Electrophysiology, Department of Cardiology, Pondok Indah Hospital, Jakarta, Indonesia https://orcid.org/0000-0002-6540-6103
Surya Sinaga Immanuel Advanced Cardiac Care Clinic, Siloam Hospitals Kebon Jeruk, Jakarta, Indonesia
Jason Wirandy Haryanto Advanced Cardiac Care Clinic, Siloam Hospitals Kebon Jeruk, Jakarta, Indonesia https://orcid.org/0009-0001-1384-7484
Harvian Satya Dharma General Practitioner, Jakarta, Indonesia https://orcid.org/0009-0000-6633-8264

Keywords: Isolated Noncompaction of the Ventricular Myocardium, Cardiomyopathies, Bundle-Branch Block, Magnetic Resonance Imaging, Heart Failure

Abstract

Background: Left Ventricular Noncompaction (LVNC) is a rare cardiomyopathy characterized by a thin compacted epicardial layer and an extensive noncompacted endocardial layer with prominent trabeculations and deep intertrabecular recesses that communicate with the Left Ventricular (LV) cavity. The classic triad of complications includes chronic heart failure, ventricular arrhythmias, and systemic embolic events. At present, evidence-based management guidelines remain limited.

Case Illustration: We report a 42-year-old man with LVNC, initially detected as an incidental Left Bundle Branch Block (LBBB) on Electrocardiogram (ECG) during a routine medical checkup. Although he remained asymptomatic, LV Ejection Fraction (LVEF) progressively declined, accompanied by rising N-Terminal pro-B-type Natriuretic Peptide (NT-proBNP) levels. Coronary artery disease was excluded by coronary computed tomography angiography. Given worsening LV systolic function over 2 years, Cardiac Magnetic Resonance (CMR) demonstrated an LVNC phenotype consistent with cardiomyopathy. Guideline-Directed Medical Therapy (GDMT) for heart failure was initiated, along with oral anticoagulation for primary prevention of LV thrombus. After medication optimization, LVEF improved markedly, and NT-proBNP normalized.

Conclusions: This case illustrates the value of comprehensive evaluation and multimodality imaging in patients with unexplained LBBB, even when asymptomatic. Early diagnosis, phenotype-guided treatment, and longitudinal surveillance may help prevent clinical progression and future heart-failure, arrhythmic, or thromboembolic complications.

Downloads

Download data is not yet available.

References

1. Singh DP, Horenstein MS, Rout P, et al. Left Ventricular Noncompaction Cardiomyopathy. 2025.
2. Heidenreich PA, Bozkurt B, Aguilar D, et al. 2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol 2022; 79: e263–e421.
3. Arbelo E, Protonotarios A, Gimeno JR, et al. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J 2023; 44: 3503–3626.
4. Negri F, De Luca A, Fabris E, et al. Left ventricular noncompaction, morphological, and clinical features for an integrated diagnosis. Heart Fail Rev 2019; 24: 315–323.
5. Wengrofsky P, Armenia C, Oleszak F, et al. Left Ventricular Trabeculation and Noncompaction Cardiomyopathy: A Review. EC Clin Exp Anat.
6. Towbin JA, Jefferies JL. Cardiomyopathies Due to Left Ventricular Noncompaction, Mitochondrial and Storage Diseases, and Inborn Errors of Metabolism. Circ Res 2017; 121: 838–854.
7. Bustea C, Bungau AF, Tit DM, et al. The Rare Condition of Left Ventricular Non-Compaction and Reverse Remodeling. Life (Basel); 13. Epub ahead of print 1 June 2023. DOI: 10.3390/life13061318.
8. Martínez-Tittonel LE, Ciorba FR, Bayona-Huguet X, et al. Left Ventricular Non-Compaction Cardiomyopathy: A Review of the Pathophysiology, Epidemiology, Diagnosis, Genetics, and Clinical Management. J Pers Med; 15. Epub ahead of print 1 October 2025. DOI: 10.3390/jpm15100484.
9. Chimenti C, Lavalle C, Magnocavallo M, et al. A proposed strategy for anticoagulation therapy in noncompaction cardiomyopathy. ESC Heart Fail 2022; 9: 241–250.
10. Gerecke BJ, Engberding R. Noncompaction Cardiomyopathy-History and Current Knowledge for Clinical Practice. J Clin Med; 10. Epub ahead of print 1 June 2021. DOI: 10.3390/jcm10112457.
11. Paluszkiewicz J, Milting H, Kałużna-Oleksy M, et al. Left Ventricular Non-Compaction Cardiomyopathy-Still More Questions than Answers. J Clin Med; 11. Epub ahead of print 1 July 2022. DOI: 10.3390/jcm11144135.
12. Pittorru R, De Lazzari M, Migliore F, et al. Left Ventricular Non-Compaction: Evolving Concepts. J Clin Med; 13. Epub ahead of print 1 October 2024. DOI: 10.3390/jcm13195674.
13. McDonagh TA, Metra M, Adamo M, et al. 2023 Focused Update of the 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure. Eur Heart J 2023; 44: 3627–3639.
14. Park S, Cheung CWS, Raj V, et al. Cardiac Magnetic Resonance Imaging in Asia: 2025 Status Update. Korean J Radiol 2025; 26: 1149–1156.
15. Sugiyanto S, Sabarudin H, Fatimah F, et al. Current Situation of Magnetic Resonance Imaging Services in Indonesia. J Med Imaging Radiat Sci 2022; 53: S52.
16. Towbin JA, Ballweg J, Johnson J. Left Ventricular Noncompaction Cardiomyopathy. Heart Failure in the Child and Young Adult: From Bench to Bedside 2024; 269–290.
17. Ross SB, Jones K, Blanch B, et al. A systematic review and meta-analysis of the prevalence of left ventricular non-compaction in adults. Eur Heart J 2020; 41: 1428–1436b.
18. Akhbour S, Fellat I, Fennich N, et al. Electrocardiographic findings in correlation to magnetic resonance imaging patterns in African patients with isolated ventricular noncompaction. Anatol J Cardiol 2015; 15: 550–555.
19. Grigoratos C, Barison A, Ivanov A, et al. Meta-Analysis of the Prognostic Role of Late Gadolinium Enhancement and Global Systolic Impairment in Left Ventricular Noncompaction. JACC Cardiovasc Imaging 2019; 12: 2141–2151.
20. Casas G, Limeres J, Oristrell G, et al. Clinical Risk Prediction in Patients With Left Ventricular Myocardial Noncompaction. J Am Coll Cardiol 2021; 78: 643–662.
21. Aung N, Doimo S, Ricci F, et al. Prognostic Significance of Left Ventricular Noncompaction: Systematic Review and Meta-Analysis of Observational Studies. Circ Cardiovasc Imaging 2020; 13: E009712.
22. Bennett CE, Freudenberger R. The Current Approach to Diagnosis and Management of Left Ventricular Noncompaction Cardiomyopathy: Review of the Literature. Cardiol Res Pract 2016; 2016: 5172308.
23. Hong J, Huang L, Jin N, et al. Effect of dapagliflozin on left ventricular structure and function in patients with non-ischemic dilated cardiomyopathy: An observational study. Medicine 2024; 103: E37579.
24. Cerar A, Poglajen G, Zemljič G, et al. SGLT2 Inhibitors Are Associated with Left Ventricular Reverse Remodeling in Patients with Non-Compaction Cardiomyopathy-A Prospective Observational Cohort Trial. Biomedicines; 13. Epub ahead of print 1 November 2025. DOI: 10.3390/biomedicines13112773.
25. Hirono K, Takarada S, Miyao N, et al. Thromboembolic events in left ventricular non-compaction: comparison between children and adults - a systematic review and meta-analysis. Open Heart; 9. Epub ahead of print 25 May 2022. DOI: 10.1136/openhrt-2021-001908.
26. Huizar JF, Ellenbogen KA, Tan AY, et al. Arrhythmia-Induced Cardiomyopathy: JACC State-of-the-Art Review. J Am Coll Cardiol 2019; 73: 2328–2344.
27. Lorca R, Martín M, Pascual I, Astudillo A, Díaz Molina B, Cigarrán H, Cuesta-Llavona E, Avanzas P, Rodríguez Reguero JJ, Coto E, Morís C. Characterization of left ventricular non-compaction cardiomyopathy. Journal of Clinical Medicine. 2020 Aug 5;9(8):2524.