Prevalence and Predictors of Pulmonary Hypertension in Children with Portal Hypertension: A Single Center Study

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 ofvarices 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 O-2 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 O-2 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 O-2 saturation are predictors of POPH. Echocardiography screening is crucial for the early detection of cases.