Related Experiment Video
Updated: May 6, 2026
Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
Prevalence and Predictors of Pulmonary Hypertension in Children with Portal Hypertension: A Single Center Study
Nehal El Koofy1, Sawsan Hassan Okasha1, Hala Mounir Agha1
1Department of Pediatric, Faculty of Medicine, Cairo University, Cairo, Egypt.
Insights
Portopulmonary hypertension (POPH) affects 30.9% of children with portal hypertension. High-grade varices and low oxygen saturation are key predictors, highlighting the need for early echocardiography screening.
Area of Science:
- Pediatric Cardiology
- Hepatology
- Pulmonology
Background:
- Portal hypertension in children can lead to serious complications.
- Portopulmonary hypertension (POPH) is a known but understudied condition in pediatric populations.
- Early identification of POPH is crucial for management and prognosis.
Purpose of the Study:
- To determine the prevalence of portopulmonary hypertension (POPH) in pediatric patients with portal hypertension.
- To identify predictors and risk factors associated with POPH in this cohort.
- To assess the clinical manifestations and diagnostic utility of screening tools.
Main Methods:
- A cohort of 94 children (aged 3-15 years) with clinically suspected and endoscopically confirmed portal hypertension was recruited.
- Participants underwent clinical evaluation, 6-minute walk distance (6-MWD) testing, and echocardiography.
- Esophagogastroduodenoscopy (EGD) was used to assess esophageal varices, and pulmonary hypertension was defined by echocardiographic findings.
Main Results:
- The prevalence of POPH was 30.9% among the study participants.
- Children with POPH exhibited more frequent dyspnea on exertion, lower O2 saturation, and higher-grade esophageal varices.
- High-grade varices (p=0.04) and low O2 saturation (p=0.03) were identified as significant risk factors for POPH.
Conclusions:
- POPH is prevalent in children with portal hypertension, affecting nearly one-third of the study group.
- High-grade varices and low oxygen saturation are significant predictors of POPH.
- Echocardiography screening is essential for the early detection and management of POPH in pediatric patients.
Purpose:
This study aimed to estimate the prevalence and predictors of portopulmonary hypertension (POPH) in children with portal hypertension.
Methods:
We recruited children of both sexes aged 3-15 years with portal hypertension that was clinically suspected and confirmed by the presence of varices on esophagogastroduodenoscopy (EGD). The participants underwent clinical examination, 6-min walk distance (6-MWD), and echocardiography.
Results:
We enrolled 94 children with portal hypertension: 26.6% with pre-hepatic causes and 73.4% secondary to chronic liver disease. Among our participants, 13.8% had one or more cardiac manifestations, such as exercise intolerance, dyspnea on exertion, cyanosis, or orthopnea, whereas 86.2% were asymptomatic. EGD examination revealed grade I varices in 54.3% of cases, grade II-III in 43.6%, and grade IV in 2.1%. Pulmonary hypertension (>35 mmHg) was detected in 30.9% of cases using echocardiography; two of them were >45 mmHg. Patients with POPH had significantly more frequent dyspnea on exertion, lower O2 saturation, and more severe variceal grades than those with normal pulmonary artery pressure. Five (6.9%) cases had <300 m 6-MWD, with no significant difference between patients with normal and those with elevated pulmonary artery pressure. The duration of portal hypertension and 6-MWD were correlated significantly with the echocardiographic measures. High-grade varices (p=0.04) and low O2 saturation (p=0.03) were identified as risk factors for POPH.
Conclusion:
POPH was detected in 30.9% of our study group. High-grade varices and low O2 saturation are predictors of POPH. Echocardiography screening is crucial for the early detection of cases.
Related Concept Videos
01:47Lung Capacity
01:30Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
01:18Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
01:23Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
01:26Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
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,...