45 year old gentleman presented with worsening heart failure. No prior investigations were done.

March 1, 2021

Case details

45 year old gentleman presented with worsening heart failure. No prior investigations were done

Fig. 1-5. Radial cine SSFP images. Increased trabeculations with increased depth of intertrabecular recesses are seen in Left ventricle (LV). LV Non-compacted to compacted myocardial ratio is 5.7. There is involvement of right ventricle as well in this case.

Fig. 6. 4CH PSIR. No myocardial delayed gadolinium enhancement.

Diagnosis and Discussion

DIAGNOSIS:

Non-compaction cardiomyopathy.

DISCUSSION:

Congenital cardiomyopathy with arrest of compaction in endocardial myocardium in fetal life. The outer layer consists of normally compacted myocardium.
However, it can be diagnosed at a later age in life when patients get evaluated for etiology for heart failure.
CMR criteria for LV non-compaction:

Non-compacted to compacted myocardial ratio >2.3 (in end-diastole) Trabecular LV mass >20% of total LV mass and NC indexed mass > 15 g/m2

Other features: Deep intertrabecular recesses
Evaluate to rule out:
- Thrombus in LV hidden in deep recesses.
- RV involvement with multiple deep trabeculae.

REFERENCES:

Dreisbach, J.G., Mathur, S., Houbois, C.P. et al. Cardiovascular magnetic resonance based diagnosis of left ventricular non-compaction cardiomyopathy: impact of cine bSSFP strain analysis. J Cardiovasc Magn Reson 22, 9 (2020). https://doi.org/10.1186/s12968-020-0599-3
https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-10/Left-ventricularnoncompaction#:~:text=Criteria%20for%20diagnosis%20by%20CMR,and%20specificity%20of%2099%25).