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Published on: February 17, 2018
Case Report: Sudden cardiac death due to ventricular myocardial non-compaction
Syrine Mannoubi1, Med Amin Mesrati1, Ibn Hadj Amor Hassen2
1Department of Forensic Medicine, Taher Sfar Hospital, University of Monastir, Mahdia, 5100, Tunisia.
Insights
Ventricular non-compaction (VNC) is a rare heart muscle disorder. Early diagnosis and treatment are crucial to prevent fatal outcomes like sudden cardiac death from arrhythmias.
Area of Science:
- Cardiology
- Genetics
- Pathology
Background:
- Ventricular non-compaction (VNC) is a rare congenital cardiomyopathy.
- It results from impaired embryonic myocardial development, leading to excessive trabeculation and deep recesses.
Observation:
- A 20-year-old male presented with dyspnea and was diagnosed with VNC.
- Echocardiography revealed reduced left ventricular ejection fraction and right ventricular hypertrabeculation, confirmed by cardiac MRI.
Findings:
- The patient, untreated and lost to follow-up, died suddenly.
- Forensic autopsy determined the cause of death as acute ventricular arrhythmia secondary to VNC.
Implications:
- This case highlights the severe, potentially fatal consequences of VNC.
- Emphasizes the critical need for early diagnosis and specific therapeutic interventions in VNC patients to prevent mortality.
Abstract:
Ventricular non-compaction (VNC) is a rare myocardium disorder, which can be both genetic and sporadic. A poor wall compaction process or an excessive trabeculae formation may be at the genesis of myocardial hypertrabeculation with multiple recesses. It is often complicated by ventricular dysfunction, arrhythmias and cardiac embolism. Herein we report a case of a 20-year-old male patient with no particular past medical history who was followed up at the cardiology department for dyspnea. Echocardiography showed reduced ejection fraction of the left ventricle with potential hypertrabeculation in the right ventricle, confirmed by cardiac MRI. The patient was not put under medication and was later lost to follow-up. He died few months later without a clear cause explaining death. A forensic autopsy was performed that attributed death to acute ventricle arrhythmia secondary to VNC, emphasizing the major role of an early and specific treatment to avoid such a fatal outcome.
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