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Outcomes for Ectopia Cordis.

Benjamin J Smith1, Jonathan N Flyer2, Erika M Edwards3

  • 1Department of Pediatrics, University of Vermont, Burlington, VT.

The Journal of Pediatrics
|November 1, 2019
PubMed
Summary
This summary is machine-generated.

Survival for very low birth weight (VLBW) infants with ectopia cordis is poor, with 96% mortality. Outcomes are significantly worse than for non-VLBW infants, highlighting disparities in care.

Keywords:
pentalogy of Cantrellpretermvery low birth weight

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Area of Science:

  • Neonatalogy
  • Pediatric Cardiology
  • Congenital Abnormalities

Background:

  • Ectopia cordis is a rare congenital malformation involving the displacement of the heart outside the chest cavity.
  • Pentalogy of Cantrell is a complex syndrome often associated with ectopia cordis, involving multiple congenital anomalies.
  • Outcomes for infants with ectopia cordis, particularly those who are very low birth weight (VLBW), require further elucidation.

Purpose of the Study:

  • To describe the survival and clinical outcomes of very low birth weight (VLBW) or preterm infants diagnosed with ectopia cordis.
  • To compare outcomes between VLBW and non-VLBW infants with ectopia cordis.

Main Methods:

  • A large, prospective, multicenter cohort study involving 2,262,162 infants born between 2000 and 2017 from 845 US centers.
  • Inclusion criteria encompassed infants with diagnoses or descriptors consistent with ectopia cordis and/or pentalogy of Cantrell, stratified by VLBW (401-1500g or 22-29 weeks gestation) and non-VLBW status.
  • Primary outcome was neonatal survival, defined as hospital discharge or an initial length of stay of at least 12 months.

Main Results:

  • 180 infants had ectopia cordis, with 76% also having pentalogy of Cantrell. VLBW infants constituted 52% of this cohort.
  • VLBW infants experienced a 96% mortality rate, with 79% dying within 12 hours, significantly higher than the 59% mortality (36% within 12 hours) in non-VLBW infants.
  • Life support was provided to 33% of VLBW infants versus 65% of non-VLBW infants. Surgical intervention was reported in 34% of VLBW and 68% of non-VLBW infants.

Conclusions:

  • Survival for VLBW infants with ectopia cordis is critically poor and substantially worse compared to non-VLBW infants.
  • Significant disparities exist in resuscitative efforts and surgical interventions between VLBW and non-VLBW groups.
  • While gestational age and weight are strong predictors, detailed characterization of cardiac and noncardiac anomalies is essential for accurate prognosis and counseling.