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Updated: Jul 6, 2026
06:34
Evaluation of Left Ventricular Structure and Function using 3D Echocardiography
Published on: October 28, 2020
Left ventricular noncompaction: case report and literature review
Pedro Magno1, António Freitas, Victor M Gil
1Serviço de Cardiologia, Hospital Fernando Fonseca, Amadora-Lisboa, Portugal. pedromagno@spc.pt
Summary
Isolated left ventricular noncompaction (ILVNC) is a rare cardiomyopathy. This case highlights ILVNC
Area of Science:
- Cardiology
- Genetics
- Embryology
Background:
- Isolated left ventricular noncompaction (ILVNC) is a rare congenital cardiomyopathy characterized by a spongy left ventricle.
- It is widely believed to result from an arrest in normal myocardial compaction during embryonic development.
- Familial forms suggest a genetic basis, with implicated genes involved in cytoskeletal and extracellular matrix interactions.
Observation:
- A 29-year-old man presented with severe decompensated heart failure (NYHA IV) and a dilated left ventricle with systolic dysfunction.
- Echocardiography revealed prominent trabeculations, a spongy appearance, and an apical thrombus.
- Initial treatment with heart failure medications and levosimendan led to clinical improvement.
Findings:
- Despite initial recovery and partial improvement in left ventricular function, the patient experienced sudden cardiac death 18 months later.
- This case underscores the potential for severe outcomes in ILVNC, even with apparent treatment response.
- The review discusses etiopathogenesis, diagnostic criteria, and therapeutic strategies for ILVNC.
Implications:
- ILVNC poses significant risks including heart failure, arrhythmias, and embolic events, contributing to morbidity and mortality.
- Current diagnostic reliance on clinical and echocardiographic data may require further refinement.
- Therapeutic strategies emphasize heart failure management, anticoagulation, and arrhythmia prevention, but long-term outcomes remain a concern.
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