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Related Experiment Video

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Postnatal Outcome of Fetal Left Ventricular Hypertrabeculation/Noncompaction.

Claudia Stöllberger1,2, Christian Wegner3, Abraham Benatar4

  • 1Krankenanstalt Rudolfstiftung, Juchgasse 25, 1030, Vienna, Austria. claudia.stoellberger@chello.at.

Pediatric Cardiology
|April 2, 2016
PubMed
Summary

Left ventricular hypertrabeculation/noncompaction (LVHT) diagnosed prenatally indicates ongoing health issues and mortality risks. Further research into survival and genetic causes for fetal LVHT is essential.

Keywords:
CardiomyopathyEchocardiographyGenetics

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Area of Science:

  • Cardiology
  • Genetics
  • Pediatrics

Background:

  • Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality with unknown causes.
  • Prenatal diagnosis is possible via fetal echocardiography.
  • Previous reviews identified 46 live cases postnatally from 106 published cases.

Purpose of the Study:

  • To collect follow-up data on prenatally diagnosed LVHT cases.
  • To assess the long-term morbidity and mortality in these patients.
  • To identify associated genetic and extracardiac abnormalities.

Main Methods:

  • Contacted authors of 46 prenatally diagnosed LVHT cases.
  • Gathered information on vital status, cardiac, and extracardiac morbidity for 18 cases.
  • Analyzed data for differences between cases with and without follow-up.

Main Results:

  • Three of 18 cases died from heart failure, osteosarcoma, and enterocolitis.
  • Six of seven examined patients had mutations or chromosomal abnormalities.
  • Nine patients had extracardiac abnormalities; four required cardiac procedures.

Conclusions:

  • Prenatally diagnosed LVHT cases show continuing morbidity and mortality.
  • LVHT is frequently associated with genetic abnormalities.
  • Further research on survival and genetic causes is warranted.