Fetal cardiomyopathy

Dr Henry Knipe and Dr Yuranga Weerakkody et al.

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 dependent the sub type. Often the fetal heart is enlarged resulting 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
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Fetal cardiomyopathies
  • In utero cardiomyopathy
  • In utero cardiomyopathies

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