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Pulmonary vascular complications in asymptomatic children with portal hypertension

John R Whitworth1, D Dunbar Ivy, Jane Gralla

  • 1Pediatric Gastroenterology, Hepatology and Nutrition, USA.

Journal of Pediatric Gastroenterology and Nutrition
|October 13, 2009
PubMed

Insights

Portopulmonary hypertension and hepatopulmonary syndrome (HPS) are rare in stable pediatric portal hypertension. However, intrapulmonary vascular shunting (IPVS) affects 19%, with elevated b-type natriuretic peptide levels observed.

Area of Science:

  • Pediatric Gastroenterology
  • Pulmonary Medicine
  • Cardiology

Background:

  • Portal hypertension in children can lead to serious complications.
  • Portopulmonary hypertension, hepatopulmonary syndrome (HPS), and intrapulmonary vascular shunting (IPVS) are known complications.
  • Assessing these conditions in stable pediatric patients is crucial.

Purpose of the Study:

  • To determine the prevalence of portopulmonary hypertension, HPS, and IPVS in children with stable portal hypertension.
  • To evaluate the predictive value of vasoactive peptides, biochemical tests, and clinical signs for these conditions.

Main Methods:

  • Prospective, cross-sectional study of 33 children (4-17 years) with stable portal hypertension.
  • Screening included contrast-enhanced echocardiography (cECHO), pulse oximetry, and Doppler echocardiography.
  • Vasoactive peptides, chemistries, and clinical data were compared between groups.

Main Results:

  • No cases of portopulmonary hypertension were found.
  • Six patients (19%) had IPVS, all with intrahepatic portal hypertension; one also had HPS.
  • IPVS patients showed biochemical evidence of advanced liver disease and elevated b-type natriuretic peptide.

Conclusions:

  • Portopulmonary hypertension and HPS are uncommon in clinically stable pediatric portal hypertension.
  • IPVS was present in 19% of studied children.
  • Elevated b-type natriuretic peptide is a novel finding associated with IPVS in this population.
Abstract

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