Fetal cardiomyopathy

Fetal cardiomyopathy refers to a very rare situation where a cardiomyopathy occurs in utero. It is often a diagnosis of exclusion where, by definition, there is an absence of an underlying congenital cardiac morphological anomaly.

The estimated incidence is variable with the high end of the reported spectrum approaching 1 in 5000 births 6.

It often results from a primary dysfunction in cardiac muscle functioning (i.e. no underlying chamber morphological or valvular anomaly)

There are two main subtypes

A fetal cardiomyopathy can have a broad spectrum of syndromic, as well non-syndromic, associations, which include:

Dedicated fetal echocardiography is often required. Radiographic features will depend on the subtype. Often the fetal heart is enlarged resulting in an increased cardiothoracic circumferential ratio.

A poor outcome is observed in many affected fetuses. The presence of diastolic dysfunction in a fetal cardiomyopathy is considered to be associated with the highest risk of mortality 4. Treatment options vary depending on the presence of associated conditions (e.g. control of underlying twin-twin transfusion syndrome, etc.).

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Article information

rID: 13878
Tag: cases
Synonyms or Alternate Spellings:
  • Foetal cardiomyopathies
  • Foetal cardiomyopathy
  • Fetal cardiomyopathies
  • In utero cardiomyopathy
  • In utero cardiomyopathies

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