Sudden death due to biventricular non-compaction cardiomyopathy in a 14-year-old

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 post-mortem, although diagnosis through echocardiogram, CT, or MRI is possible and there is criterion for diagnosis with each of these.

State-of-the art review: Noncompaction cardiomyopathy in pediatric patients

“…NCCM predominantly affects the left ventricle, both in children and adults. To date, few cases of biventricular noncompaction in children are reported [22][23][24][25][26][27]. Furthermore, in the left ventricle, NCCM predominantly affects the apex.…”

Challenges and diagnosis of isolated left ventricular non-compaction: A case series of 4 patients with echocardiographic diagnosis of possible ILVNC

“…(8) Despite the fact that ILVNC is a congenital condition, it may present in adulthood. (5,(9)(10)(11) It is recommended that a meticulous three-generational family history be obtained to evaluate for genetic influence, as a positive family history has an impact on screening of other family members. (12) Follow-up of family members of an index case has identified a 30% familial occurrence.…”

“…The prevalence of ILVNC is unknown, and echocardiographic studies have reported a prevalence of 0.014% -1.3%, (7,11) and the diagnosis may often be missed. A number of cases have been reported from Africa, (9,(22)(23)(24)(25)(26) including South Africa. (10,22) At a cardiomyopathy clinic in Johannesburg, the prevalence of ILVNC was 6.9%, and the diagnosis was based on echocardiographic criteria alone.…”

Left ventricular non - compaction: contemporary view of genetic background, clinical course, diagnostic and treatment

“…Вовлечение в процесс обоих желудочков -редкое явление [5]. В зарубежной литературе есть единичные примеры бивентрикулярного некомпактного миокарда [6]. Описаны два случая сочетания бивентрикулярного некомпактного миокарда со снижением систолической и диастолической функции ЛЖ и легочной гипертензией [7,8].…”