Familial dilated hypokinetic cardiomyopathy

Case contributed by Dr Sigmund Stuppner

Presentation

Isolated transient aphasia at emergency presentation.

Moderate cardiac enlargement with signs of pulmonary vessel congestion.

                                                LEFT VENTRICLE                      RIGHT VENTRICLE
EDVi (ml/m²)                            280 (V.N. 47 -  92)                   97 (V.N. 55 - 105)                   
ESVi (ml/m²)                             230 (V.N. 13 - 30)                    55 (V.N. 15 - 43)
SVi (ml/m²)                               50 (V.N. 32 - 62)                       42 (V.N. 32 - 64)
EF (%)                                        18 (V.N. 55 - 78)                       43 (V.N. 47 - 74)
 

The MRI findings are a compatible with dilated hypokinetic cardiomyopaty with severe dilatation of the left ventricle and severe depression of left cardiac systolic function with diffuse hypokinesia.

Right ventricle with normal dimension and mild depression of systolic function.

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