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Left Ventricular Non-compaction Cardiomyopathy: A Report of a Rare Case From Saudi Arabia
Husna Irfan Thalib1, Sayeeda Mehveen2, Sariya Khan1
1General Medicine and Surgery, Batterjee Medical College, Jeddah, SAU.
Insights
Left ventricular non-compaction cardiomyopathy (LVNC) is a rare congenital heart defect. This case highlights diagnosis and management of LVNC, a condition involving abnormal heart muscle development.
Area of Science:
- Cardiology
- Genetics
- Pathology
Background:
- Left ventricular non-compaction cardiomyopathy (LVNC) is a rare congenital heart disease.
- Characterized by deep intertrabecular recesses and hypertrabeculations of the left ventricle.
- Results from incomplete myocardial compaction during embryogenesis.
Abstract:
Left ventricular non-compaction cardiomyopathy (LVNC) is an unusual congenital heart disease that predominantly affects the heart's left ventricle. This disease is characterized by deep intertrabecular recesses and hypertrabeculations of the myocardial wall that link with the ventricle cavity. During embryogenesis, the fetal myocardium has to undergo a compaction process, wherein the trabeculated and spongy myocardial tissue compacts into a dense, solid form. An incomplete compaction process results in persistent non-compacted spongy myocardial tissue and trabeculations prominent in the left ventricle. This disease could be marked alone or be present in coexistence with other congenital heart abnormalities. We present a rare case of a 57-year-old Saudi male who presented to the ER with chest pain and dyspnea. Due to severe chest pain, he was admitted to the coronary care unit. On further investigation, an echocardiogram revealed heavy trabeculations in the dilated left ventricle and a reduced ejection fraction. The case was diagnosed as LVNC and possible heart failure. The patient was discharged after he was kept under guideline-directed medical therapy (GDMT) along with certain medications and will be evaluated after six months of GDMT to decide on implantable cardiac resynchronization therapy. Although LVNC is rare, it can lead to severe heart conditions like arrhythmias, thromboembolism, and heart failure.
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