Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy

Abstract

Aim-To determine clear cut echocardiographic criteria for isolated ventricular non-compaction (IVNC), a cardiomyopathy as yet "unclassified" by the World Health Organization. The disease is not widely known and its diagnosis mostly missed. Methods and results-In seven out of a series of 34 patients with IVNC the in vivo echocardiographic characteristics were validated against the anatomical examination of the heart removed after death in four and due to heart transplantation in three patients. Four morphological criteria diagnostic for IVNC were found. (1) Coexisting cardiac abnormalities were absent (by definition). (2) A two layer structure was seen, with a compacted thin epicardial band and a much thicker non-compacted endocardial layer of trabecular meshwork with deep endomyocardial spaces. A maximal end systolic ratio of non-compacted to compacted layers of > 2 is diagnostic. (3) The predominant localisation of the pathology was to mid-lateral (seven of seven patients), apical (six), and mid-inferior (seven) areas. The pathological preparations confirmed the echocardiographic findings. Concomitant regional hypokinesia was not confined to the non-compacted segments. (4) There was colour Doppler evidence of deep perfused intertrabecular recesses. Conclusions-Four clear cut echocardiographic diagnostic criteria were established. It is suggested that the WHO classification of cardiomyopathies be reconsidered to include IVNC as a distinct cardiomyopathy. (Heart 2001;86:666-671)

Effects of in ovo exposure to 3,3′,4,4′‐tetrachlorobiphenyl (PCB 77) on heart development in tree swallow (Tachycineta bicolor)

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“…Particularly in II‐4 and II‐5 patients, deep endocardial recesses were documented with color Doppler, meeting the echocardiographic diagnostic criteria for LVNC (ie, noncompaction/compaction ratio ≥2) 16. A remarkable LV hypertrophy with an LV maximal wall thickness of 12‐, 17‐, and 22‐mm thickness was observed in patients II‐4, II‐5, and III‐2, respectively.…”

Clinical usefulness of SonoVue contrast echocardiography: the Thoraxcentre experience

“…14,15 Noncompaction cardiomyopathy Noncompaction cardiomyopathy is a relatively rare congenital, unclassified cardiomyopathy that is characterised by excessively prominent trabecular meshwork and deep intertrabecular recesses, best evidenced by colour Doppler flow. 16 Currently, we are studying the value of contrast echocardiography in these noncompaction patients. As seen in figure 4, we have described one patient in the literature in whom flow in the intertrabecular recesses could only be detected with the use of SonoVue contrast.…”