Spade shaped Left Ventricle in Apical variant of Hypertrophic Cardiomyopathy (or Yamaguchi HCM)

 

 

RAO projection demonstrating a "spade"-shaped left ventricular (LV) cavity, suggestive of apical variant hypertrophic cardiomyopathy (HCM). Contrast agent appears dark and defines the narrow channel-like apical cavity (white arrow). At the apex, there is increased myocardial thickness (black arrows). Also seen are the dual-chamber pacemaker leads (asterisks). Watch Video below:

 

Pulse Wave Doppler of HCM Vs Aortic Stenosis:

PW doppler pattern differs because of dynamic obstruction of LVOT as compared to fixed aortic valve stenosis, resulting in dagger shapped profile in HCM as shown in the image below.

Spade shaped Left Ventricle in Apical variant of Hypertrophic Cardiomyopathy (or Yamaguchi HCM)

Apical HCM is uncommon form of HCM which is seen most frequently in Japanese patients (25% of all HCM cases in Japan and 1-2% outside japan). It is characterized by localised hypertrophy of LV apex, causing a “spade-shaped” configuration of the LV cavity on ventriculography. Classic ECG finding in apical HCM is giant T-wave inversion in the precordial leads.

 

VIEW ECHOCARDIOGRAPHY INTRESTING CASE OF HCM HERE: