Left ventricular noncompaction: a pathological study of 14 cases

摘要: Summary Left ventricular noncompaction (LVNC) has been recently proposed as a specific form of cardiomyopathy. There have few pathological series describing gross and microscopic findings this entity, especially in children. We present 14 hearts (13 autopsy 1 explant) with LVNC (isolated associated congenital heart disease), defined by poorly developed left (LV) papillary muscles noncompact inner LV myocardial layer comprising more than 50% the thickness. The mean age at death/explant was 3.6 years (median, 2.5 months); there were 6 boys 8 girls. symptoms sudden unexpected death (10) failure (4). diagnosis suspected before only 13 cases. Right involvement (>75% thickness comprised noncompacted area recess adjacent to tricuspid valve) seen hearts. One patient had sibling pulmonary stenosis, but no other known familial Endocardial fibroelastosis characteristic histological feature, well anastomosing or polypoid endocardial trabeculations, which resulted staghorn-shaped, endocardial-lined recesses. high rate cardiac anomalies, often coexisted not clearly related LVNC, cases (nonisolated LVNC): septal defect (4/14), anomalous venous veins (1/14), coronary ostial stenosis histiocytoid cardiomyopathy polyvalvar dysplasia (2/14), (2/14). In isolated 2 malformed atrioventricular valves (1 mitral tricuspid), appeared part maldevelopment. differences patterns regions between nonisolated LVNC. is frequently defects, when causing infants A clear-cut morphological distinction "isolated" "secondary" apparent. pathologist should be aware entity because first established autopsy.