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Updated: Jul 28, 2025

Evaluation of Left Ventricular Structure and Function using 3D Echocardiography
Published on: October 28, 2020
Left Ventricular Noncompaction Cardiomyopathy in an Elderly Patient: A Case Report and Literature Review
Tetyana Okan1, Homayoon Lodeen2, Michael Abawkaw2
1Department of Internal Medicine, Lviv National Medical University, Lviv, UKR.
Insights
Isolated left ventricular noncompaction cardiomyopathy (LVNC) is a rare genetic heart condition often diagnosed late. Early detection through echocardiography and advanced imaging is crucial for managing severe complications like heart failure and arrhythmias.
Area of Science:
- Cardiology
- Genetics
- Medical Diagnostics
Background:
- Isolated left ventricular noncompaction cardiomyopathy (LVNC), a rare congenital disorder, results from impaired fetal myocardial development.
- Classified as a genetic cardiomyopathy, LVNC affects 0.017-0.26% of adults, often leading to left ventricular dysfunction and high mortality.
- Limited knowledge and diagnostic challenges contribute to the underdiagnosis of LVNC, particularly in adults.
Observation:
- A 62-year-old male presented with palpitations, an irregular heartbeat, and a systolic murmur.
- Electrocardiogram revealed atrial fibrillation and ST-T wave changes; echocardiography showed severe left ventricular hypertrophy, reduced ejection fraction (<30%), and prominent trabeculations.
- Cardiac MRI confirmed LVNC, excluding ischemic heart disease.
Findings:
- Transthoracic echocardiography (TTE) is the primary diagnostic tool, revealing characteristic features like a high noncompacted to compacted myocardium ratio (>2.5:1).
- Cardiac magnetic resonance (CMR) imaging provides definitive diagnosis, complementing TTE findings.
- The patient was treated with carvedilol, ramipril, verospiron, torasemide, rivaroxaban, and an implantable cardioverter-defibrillator was recommended.
Implications:
- Delayed diagnosis of LVNC in adults is common due to its resemblance to more prevalent heart conditions.
- Early diagnosis is critical for preventing life-threatening complications, including heart failure, thromboembolic events, and ventricular arrhythmias.
- Further prospective studies are essential to enhance the management strategies and improve outcomes for patients with LVNC.
Abstract:
Isolated left ventricular noncompaction cardiomyopathy (LVNC), also known as spongy myocardium, is an extremely rare congenital disorder belonging to unclassified cardiomyopathies by the World Health Organization and classified as a genetic cardiomyopathy by the American Heart Association. Adult prevalence is 0.017-0.26% in observational echocardiographic studies. The disease occurs due to the intrauterine arrest of normal myocardial compaction, leading to left ventricular dysfunction. Reported mortality is high, ranging from 35 to 47% over a 42- to 72-month follow-up period. Knowledge regarding proper diagnosis, morbidity, and prognosis is limited; thus, this disease is subdiagnosed. Our aim is to highlight a diagnostic approach to LVNC in an elderly patient and to stress specific diagnostic signs that make the disease more recognizable. We are reporting a case of noncompaction cardiomyopathy in a 62-year-old male without any significant past medical history who was referred to our clinic for arrhythmia evaluation. The patient had several brief episodes of palpitations over the past two months. On physical examination, he presented a blowing systolic murmur at the apex and an irregularly irregular rhythm. The 12-lead electrocardiogram (ECG) demonstrated atrial fibrillation and ST-T segment depression in the V4-V6 leads. A transthoracic echocardiogram (TTE) showed signs of dilated cardiomyopathy, severe eccentric left ventricular hypertrophy, decreased contractility with an ejection fraction (EF) <30%, moderate mitral and tricuspid regurgitations, and moderate pulmonary hypertension. Multiple prominent trabeculations were noticed in the middle and apical segments of the left ventricle. The noncompacted to compacted myocardium ratio was >2.5:1. Cardiac catheterization excluded ischemic heart disease. Cardiac magnetic resonance (CMR) imaging confirmed the diagnosis of LVNC. The patient started treatment with carvedilol, ramipril, verospiron, torasemide, and rivaroxaban. An implantable cardioverter-defibrillator (ICD) was recommended. In conclusion, the diagnosis of LVNC in the adult population is often delayed because of similarities with more frequently diagnosed diseases. TTE is the initial diagnostic test of choice. Additional imaging modalities (contrast echocardiography, CMR) can help confirm the diagnosis. Early diagnosis is crucial because of the high incidence of life-threatening complications related to heart failure, thromboembolic events, and ventricular arrhythmias. Additional prospective studies are needed to improve the management and outcomes of this rare cardiomyopathy.
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