Hypertrophic cardiomyopathy - severe

Case contributed by Tim Luijkx

Presentation

Resuscitated. Known hypertrophic cardiomyopathy. Identical twin sister also has HCM.

Patient Data

Age: 20
Gender: Female

Asymmetric left ventricular hypertrophy, most prominent in interventricular septum and varying from 20 to 30 mm. Sparing of the LV apex. Concentric mid-cavity hypertrophy with occlusion during systole. Hypertrophy extends to right ventricle and left ventricle outflow tract.

During systole there is an acceleration jet between the hypertrophic interventricular septum and the anterior leaflet of the mitral valve, indicative of systolic anterior motion (SAM). Slight mitral valve insufficiency. 

Inhomogeneous patchy late enhancement anterior LV wall as well as inferior part of the interventricular septum. 

Good contraction of both ventricles. No dilatation of the right ventricle (41 mm end-diastolic). Dilated left ventricle (63 mm end-diastolic).

Conclusion:
Images in keeping with severe hypertrophic cardiomyopathy.

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