Non compaction cardiomyopathy with LV dysfunction
40-50 year old woman with a non-compaction cardiomyopathy. She was known with this disorder for 10 years after family screening. Her medical history is unremarkable. In the last 5 years her LV systolic function shows a gradual decline. Currently she has a functional performance NYHA class II-IV and an LVEF of approximately 30-35%
The LV is moderately dilated and has an impaired function. The LVEF was calculated at 35% by 3D echocardiography. The parasternal views do not show the typical phenotype (the apical segment suffered from acoustic drop out in the parastrenal views, precluding the implementation of the Jenni criteria)
In the apical views the non-compacted myocardium in the apical and in the posterolateral segments is clearly seen. The LV systolic function is globally impaired. The RV is not dilated and still has a good function. Also appreciate the ICD lead in the RV (given for primary prophylaxis).
Thrombi in the deep apical crypts are not seen but cannot be excluded without the use of contrast.
Also the diastolic function is impaired. Doppler recordings below show a pseudonormal filling pattern with a clear L-wave, an increased E/E`.
Deformation imaging analysis in this patients shows a severely impaired function. The GLS is calculated at -10%. Regional analysis show that the typical predilection sites are most affected; the posterolateral region and the septum. Also note, that in this patient, the other segments also exhibit an impaired longitudinal function.
Unfortunately, the short axis view at the apical level could not be imaged, therefore the rotation could not be calculated