N063 Isolated ventricular non-compaction: A rare form of cardiomyopathy

Abstract

Isolated ventricular non-compaction (IVNC) is an increasingly recognized but uncommon form of cardiomyopathy. It is caused by a failure of normal embryogenesis of the endocardium and myocardium in utero resulting in prominent ventricular trabeculations with deep intertrabecular recesses. It was first described in 1984 in a 33 year old woman by Engberding and colleagues. Since then, several case reports in adults and children have been reported. It has recently been classified as genetic cardiomyopathy. The rarity and diverse clinical manifestation of IVNC may prolong diagnosis. The delay in diagnosis poses a potential risk to the individual and may delay genetic testing of family members. The purpose of this abstract is to present a real case study of IVNC including a review of the pathogenesis, clinical presentation, diagnostic procedures, prognosis, potential complications and recommended follow up. The management including the issue of controversial use of primary prevention implantable defibrillator and the importance of genetic work-up and counseling will also be reviewed. The rarity of an IVNC diagnosis in cardiac care poses a unique challenge for cardiac nurse to remain current in their knowledge related to IVNC. Updates, such as this case study, may create an interest in the topic and subsequent future readings that may be later used by nurses in teaching patients and families regarding this genetic disorder.