ISSN - 0973-0958

Pediatric Oncall Journal View Article

Biventricular Non-Compaction (Spongiform) Cardiomyopathy
Girish Gopal, Sudha Rudrappa, Rajendra Kumar.
Department of Pediatrics, Mysore Medical College and Research Institute, Mysore, Karnataka, India.
Isolated ventricular non-compaction (VNC) is an exceedingly rare genetic cardiomyopathy. In utero, compaction of the myocardial fibres is arrested during endomyocardial morphogenesis resulting in altered structure of the endomyocardial wall. Although this abnormality is often associated with other congenital cardiac defects, it can also be seen in isolation. Most often non-compaction of the left ventricle is seen. However, right ventricular involvement has also been reported in a few cases. Congestive cardiac failure, arrhythmias and systemic thromboembolic events are the major clinical manifestations of VNC. Two dimensional echocardiography or cardiac magnetic resonance imaging are the standard diagnostic modalities. We report a 1 ½ years old boy who presented to us with supraventricular tachycardia and congestive cardiac failure as a manifestation of isolated VNC.

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