Apical hypertrophic cardiomyopathy
This case shows the findings of an advanced apical HCM phenotype in a 50-60 year old man. The diagnosis was made 6 years ago when he presented with atrial fibrillation. His prior medical history was unremarkable and his family history revealed a sudden cardiac death of this father below the age of 50 years. The ECG was abnormal with pronounced LVH with deep negative T-wave in all leads. Genetic testing is pending.
The images below show a severe apical hypertrophy and also thickening of the interventricular septum. The maximal septal thickness was 18mm and the apical wall thickness was >20mm. The basal segments posterolateral are normal. The LVEF was 60%. There is no SAM nor any outflowtract obstruction.
The diastolic function was abnormal with an E/A ratio of 4.0, a clear L-wave after the E-wave (see arrow). The E/E`was 26.
In this particular case, the wall thickness shows a clear correlation to the strain values. The most abnormal strain values are seen in the thickest wall segments. In the bulls-eye presentation of the strain analysis, the apical segments have no strain (or are positive) and the anteroseptal segments show a clear reduction in strain accompanied with post systolic shortening (see PSI image)
In the APLAX strain analysis, we see a typical finding in HCM patients (not seen in all). There is an early peak systolic strain (which is reduced) followed by a plateau phase and finally some post systolic shortening (best seen in the green graph). This has also been dubbed as the “double peak sign”.
CMR confirmed the echocardiographic findings. The septal thickness was 20mm and the maximal wall thickness in the apex was approximately 24mm. LV mass 225 gr (114 gr./m²).
The LVEF was 58%. There is diffuse patchy delayed enhancement in the apex and in the anteroseptal wall, corresponding to the segments with the most abnormal strain values.