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Revisiting Non-Compaction Cardiomyopathy Through a Case with Cyanosis and Complete Heart Block
Achyut Sarkar1, Imran Ahmed1, Arindam Pande1
1Department of Cardiology, Pediatric Cardiology Unit, Institute of Post Graduate Medical Education and Research, Seth Sukhlal Karnani Memorial Hospital, Kolkata, West Bengal, India.
Insights
Left ventricular non-compaction, a rare cardiomyopathy, can present with severe symptoms like heart failure and block. Echocardiography is key for diagnosing this spongy myocardium, confirmed by cardiac MRI.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Medical Imaging
Background:
- Left ventricular non-compaction (LVNC), also known as "spongy myocardium", is a rare congenital cardiomyopathy.
- It carries significant risks of serious complications, necessitating early diagnosis and management.
Abstract:
Left ventricular non-compaction or "spongy myocardium", is a rare congenital cardiomyopathy that should be considered as a possible diagnosis because of its potential complications. Echocardiography is the diagnostic tool of choice, and cardiomagnetic resonance (CMR) can confirm or rule out this disease. Herein, we report the case of an 8-month-old female child who presented with congestive cardiac failure (CCF) and symptomatic complete heart block (CHB). An echocardiogram established the diagnosis as non-compaction cardiomyopathy (NCCM). An associated double outlet right ventricle with ventricular septal defect and valvular pulmonary stenosis was found. Cardiac magnetic resonance study confirmed the findings. This singular case report of NCCM highlights the importance of clinical awareness of this rare abnormality, its varied presentation and associated cardiac anomalies. The article revisits NCCM and focuses on the practical issues for a proper echodiagnosis.
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