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Congenital Diaphragmatic Hernia Patients with Left Heart Hypoplasia and Left Ventricular Dysfunction Have Highest

María V Fraga1, Holly L Hedrick2, Natalie E Rintoul1

  • 1Division of Neonatology, The Children's Hospital of Philadelphia, Philadelphia, PA; Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania. Philadelphia, PA.

The Journal of Pediatrics
|April 18, 2024
PubMed

Insights

Infants with congenital diaphragmatic hernia (CDH) and both left ventricular (LV) dysfunction and left heart hypoplasia (LHH) face the highest risk of extracorporeal membrane oxygenation (ECMO) and death. This highlights the critical need for early detection and management of these combined conditions.

Area of Science:

  • Pediatric Cardiology
  • Neonatal Surgery
  • Congenital Heart Disease

Background:

  • Congenital diaphragmatic hernia (CDH) is a severe condition often associated with cardiac anomalies.
  • Left ventricular (LV) dysfunction and left heart hypoplasia (LHH) are recognized complications in CDH patients.
  • Understanding the spectrum and impact of these cardiac issues is crucial for patient outcomes.

Purpose of the Study:

  • To define the prevalence of LV dysfunction and LHH in infants with CDH.
  • To investigate associations between cardiac anomalies and CDH severity.
  • To assess the impact of LV dysfunction and LHH on the need for extracorporeal membrane oxygenation (ECMO) and mortality.

Main Methods:

  • Retrospective cohort study of 182 CDH patients from January 2017 to May 2022.
  • Echocardiograms were used to prospectively assess LV function and LHH.
  • Patients were categorized into four groups based on the presence or absence of LV dysfunction and LHH.

Main Results:

  • 28% of CDH patients exhibited both LV dysfunction and LHH.
  • A dose-response relationship was observed between the severity of left heart disease and increased odds of ECMO and death.
  • Infants with both LV dysfunction and LHH had significantly higher odds of requiring ECMO (OR 1.76) and mortality (OR 2.76) after adjusting for CDH severity.

Conclusions:

  • The combination of LV dysfunction and LHH in CDH patients is associated with the highest risk of ECMO use and mortality.
  • These findings underscore the importance of comprehensive cardiac evaluation in neonates with CDH.
  • Early identification and management strategies for combined LV dysfunction and LHH may improve survival rates in CDH.
Abstract

Keywords:
congenital diaphragmatic hernialeft heart hypoplasialeft ventricular dysfunctionpulmonary arterial hypertension

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