Sudden death due to biventricular non-compaction cardiomyopathy in a 14-year-old
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CASE REPORT
Sudden death due to biventricular non-compaction cardiomyopathy in a 14-year-old Paul J. Cohen • Joseph A. Prahlow
Accepted: 13 November 2014 / Published online: 31 December 2014 Ó Springer Science+Business Media New York 2014
Abstract A 14-year-old African-American female with a long-standing medically and ablation-treated history of tachycardia with associated seizures died suddenly. Upon autopsy, evidence of gross non-compaction involving the left ventricle, as well as possible subtle non-compaction of the right ventricle was discovered. Microscopically, there was focal myocyte hypertrophy as well as myxoid connective tissue and subendocardial fibroelastosis in the areas affected by the non-compaction. Arrhythmia, precipitated by the underlying cardiomyopathy, led to this young girl’s death. Non-compaction cardiomyopathy is a rare cardiac disorder which commonly goes undiagnosed until postmortem, although diagnosis through echocardiogram, CT, or MRI is possible and there is criterion for diagnosis with each of these. Keywords Forensic Autopsy Non-compaction cardiomyopathy Isolated left ventricular non-compaction cardiomyopathy Hypertrabeculation Cardiomyopathy Pediatric Spongiform cardiomyopathy
P. J. Cohen Indiana University School of Medicine-Indianapolis, Indianapolis, IN, USA e-mail: [email protected] J. A. Prahlow (&) The Medical Foundation, and Indiana University School of Medicine-South Bend, 530 N. Lafayette Blvd., South Bend, IN, USA e-mail: [email protected]
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Introduction Non-compaction cardiomyopathy (NCM), also known as isolated ventricular non-compaction cardiomyopathy (IVNC), left ventricular non-compaction cardiomyopathy (LVNC), and spongiform cardiomyopathy, is a rare congenital cardiac disorder, the features of which were initially described in 1932 by Bellet and Gouley [1]. It was not until 1990 that Chin et al. [2] gave it the name left ventricular non-compaction cardiomyopathy. In 2006 it was categorized as a primary cardiomyopathy with genetic cause by the American Heart Association [3]. Just over 80 years since it was first described, there remains much mystery surrounding this disorder. NCM is described as a hypertrabeculation of the ventricle with deep intertrabecular recesses communicating with the ventricular cavity, giving it a spongy appearance [4]. It is most commonly seen in the left ventricle, but has been seen, although much more rarely, in the right ventricle as well as in both ventricles [5]. The most commonly accepted hypothesis of its cause is that of a congenital origin, although there have been reports of acquired cases as well [6]. Ideally, clinicians would diagnose this early in the course of disease, as earlier diagnosis and treatment reportedly leads to better outcome [7], but even increased diagnoses later in the disease course is helpful, as it is commonly misdiagnosed as other cardiomyopathies such as dilated or hypertrophic cardiomyopathy [4, 8, 9]. It has been suggested that the condition may even be, at times, missed at autopsy, as
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