Nehal El Koofy, Sawsan Hassan Okasha, Hala Mounir Agha, Noha Ali, Ahmed Said Behairy, Hanan Mina Fouad, Rehab Hamdy Zawam
Cairo University, Helwan University.
Egypt
Pediatric Gastroenterology Hepatology and Nutrition
Pediatr Gastroenterol Hepatol Nutr 2025; 28: 101-111
DOI: 10.5223/pghn.2025.28.2.101
Abstract
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.
Category
Class I. Pulmonary Hypertension Associated with Liver Disease
Symptoms and Findings Associated with Pulmonary Vascular Disease
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes