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Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
08:08

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets

Published on: May 11, 2015

Portopulmonary hypertension in pediatric patients

Adria A Condino1, D Dunbar Ivy, Judith A O'Connor

  • 1Section of Pediatric Gastroenterology, Hepatology & Nutrition, Department of Pathology, The Children's Hospital, University of Colorado Health Sciences Center, Denver, CO, USA.

The Journal of Pediatrics
|July 20, 2005
PubMed

Insights

Portopulmonary hypertension (PPHTN) in children often presents subtly. Early echocardiogram and cardiology evaluation are crucial for diagnosing PPHTN in pediatric patients with portal hypertension.

Area of Science:

  • Pediatric Cardiology
  • Hepatology
  • Pulmonology

Background:

  • Portopulmonary hypertension (PPHTN) is a rare but serious complication of portal hypertension (PH).
  • Diagnosis in pediatric patients can be challenging due to subtle clinical manifestations.
  • Understanding the presentation and outcomes is vital for timely intervention.

Purpose of the Study:

  • To investigate the clinical presentation, manifestations, and response to therapy of PPHTN in pediatric patients.
  • To highlight the diagnostic challenges and essential evaluation methods for PPHTN in this population.

Main Methods:

  • Retrospective chart review of 7 pediatric patients diagnosed with PPHTN.
  • Analysis of clinical data including causes of PH, time to PPHTN diagnosis, presenting symptoms, and diagnostic test results.
  • Review of treatment responses and postmortem findings.

Main Results:

  • Common causes of PH included biliary atresia and cavernous transformation of the portal vein.
  • Presenting symptoms included new heart murmur, syncope, and dyspnea.
  • Echocardiography was diagnostic for pulmonary hypertension in all patients; ECG and chest X-rays were insensitive.
  • Mean pulmonary artery pressure averaged 65 +/- 20 mm Hg.
  • Therapy response was variable, with 4 deaths; postmortem findings showed plexiform lesions and pulmonary arteriopathy.

Conclusions:

  • Pediatric patients with PH presenting with new heart murmur, dyspnea, or syncope require PPHTN evaluation.
  • Electrocardiograms and chest X-rays are not reliable screening tools for PPHTN.
  • Echocardiography and cardiology consultation are essential for accurate PPHTN diagnosis in children.
Abstract

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