Related Experiment Video
Updated: Jun 17, 2026
Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
Portopulmonary hypertension in children. A study in pediatric autopsies
Cecilia Ridaura-Sanz1, Carmen Mejía-Hernández, Eduardo López-Corella
1Department of Pathology, National Institute of Pediatrics, Mexico City, Mexico. cridaura@gmail.com
Insights
Idiopathic portal hypertension in adolescent girls is a risk factor for portopulmonary hypertension. Early screening for pulmonary hypertension in these patients is recommended for timely treatment.
Area of Science:
- Pediatric Cardiology
- Pulmonology
- Hepatology
Background:
- Portopulmonary hypertension (POPH) in children is poorly understood, with limited data on its prevalence and risk factors.
- POPH development in pediatric patients with portal hypertension is often undiagnosed during life.
- Autopsy studies are crucial for identifying POPH due to characteristic pulmonary vascular changes.
Purpose of the Study:
- To determine the relative frequency of portopulmonary hypertension in children with portal hypertension.
- To identify potential risk factors for POPH in this pediatric population.
Main Methods:
- Retrospective review of autopsy cases with documented portal hypertension.
- Histopathological examination of pulmonary vasculature for hypertensive arteriopathy.
Main Results:
- Out of 7060 autopsies, 76 cases (1.07%) showed portal hypertension.
- Four cases (5.2% of portal hypertension cases) had histopathological evidence of pulmonary hypertension.
- All four patients were adolescent females with idiopathic portal hypertension; POPH was undiagnosed during their lifetimes.
Conclusions:
- Idiopathic portal hypertension in adolescent females is a significant risk factor for developing portopulmonary hypertension.
- Prolonged survival due to preserved liver function may contribute to POPH development.
- Proactive screening for pulmonary hypertension in these patients is advised for early intervention.
Background And Aims:
The relative frequency and risk factors of portopulmonary hypertension in children have not been defined with precision. The few published reports refer to isolated cases or small series from selected populations. The development of pulmonary hypertension in patients with portal hypertension is seldom suspected or documented during life. The examination of autopsy material can identify these cases because pulmonary hypertension produces characteristic histological changes in the pulmonary vasculature.
Methods:
Autopsy cases with evidence of portal hypertension were retrieved. Slides of pulmonary tissue from these cases were examined in search of histopathological changes of hypertensive arteriopathy
Results:
Seventy six cases of portal hypertension were identified among 7060 autopsies collected between January 1971 and January 2008 (1.07%). Four cases with histopathological evidence of pulmonary hypertension were identified. This represents 5.2% of all cases with portal hypertension. These four patients were females in late childhood or adolescence with idiopathic portal hypertension. Pulmonary hypertension was not diagnosed during their lifetime.
Conclusions:
Idiopathic portal hypertension in adolescent girls should be considered a risk factor for the development of portopulmonary hypertension, very likely as a consequence of the functional preservation of hepatic tissue that allows a prolonged survival. An oriented search for early signs of pulmonary hypertension in these patients would appear advisable in order to install timely treatment.
Related Concept Videos
01:30Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
01:16Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue, improving...