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Higher Incidence of Protein-Losing Enteropathy in Patients with Single Systemic Right Ventricle

Alyssa M Bernardi1, Sylvestor Moses2, Brent J Barber3

  • 1Department of Pediatrics, University of Arizona, Tucson, USA.

Pediatric Cardiology
|September 25, 2020
PubMed

Insights

Patients with single ventricle congenital heart disease, particularly hypoplastic left heart syndrome (HLHS), face a higher risk of protein-losing enteropathy (PLE) after surgery compared to tricuspid atresia (TA). This suggests a single systemic right ventricle may be an independent risk factor for developing PLE.

Area of Science:

  • Pediatric Cardiology
  • Gastroenterology
  • Congenital Heart Disease

Background:

  • Patients with single ventricle congenital heart disease (SV-CHD) are susceptible to protein-losing enteropathy (PLE) post-surgical palliation.
  • Physiologic differences between single right and left ventricles may influence PLE incidence.

Purpose of the Study:

  • To investigate the hypothesis that right ventricular morphology is associated with a higher incidence of PLE.
  • To compare PLE incidence, outcomes, and costs between hypoplastic left heart syndrome (HLHS) and tricuspid atresia (TA).

Main Methods:

  • Retrospective review of over 15 million pediatric hospitalizations (ages 5-21) from 2000-2012.
  • Utilized Healthcare Cost and Utilization Project KID databases with ICD-9 codes for HLHS and TA.
  • Compared PLE incidence, patient age at admission, age of PLE onset, hospital outcomes, and costs.

Main Results:

  • PLE incidence was significantly higher in HLHS admissions (17.8%) compared to TA admissions (5.9%) (p < 0.001).
  • Admissions with PLE were older (12 vs 10 years, p < 0.001), and PLE onset occurred younger in HLHS (11 years) than TA (14 years).
  • No significant differences were found in hospital outcomes or costs between groups.

Conclusions:

  • Patients with HLHS demonstrate a higher incidence and younger age of onset for PLE compared to those with TA.
  • A single systemic right ventricle may represent an independent risk factor for developing PLE in pediatric patients with SV-CHD.
  • Further research is warranted to elucidate the mechanisms and optimize management of PLE in this population.

Keywords:
Congenital heart defectsFontanProtein-losing enteropathySingle ventricle

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