# Hepatopulmonary syndrome in children and adolescents with portal hypertension in Brazil: A multicenter study

## TL;DR

This study examines hepatopulmonary syndrome in Brazilian children with portal hypertension, highlighting its clinical features and outcomes.

## Contribution

The study provides new insights into the clinical presentation and outcomes of pediatric hepatopulmonary syndrome in Brazil.

## Key findings

- Hepatopulmonary syndrome was asymptomatic in 44.4% of patients at diagnosis.
- Elevated hemoglobin levels were associated with hypoxemia in patients with portal hypertension.
- Liver transplantation survival rates were not affected by the severity of hypoxemia at diagnosis.

## Abstract

To describe the clinical and laboratory characteristics and outcomes of pediatric hepatopulmonary syndrome (HPS) secondary to portal hypertension (PH) in Brazil.

Fifty‐four pediatric patients diagnosed with PH and HPS according to the European Respiratory Society criteria were included in this multicenter retrospective study. Clinical and laboratory data at the time of diagnosing the underlying disease, 12 months before diagnosing HPS, at the time of diagnosing HPS, and at the time of the last consultation were collected from the medical records.

PH was cirrhotic in 87% of patients. Biliary atresia was the predominant etiology (35.2%). The median age at the time of diagnosis was 7.8 years (interquartile range [IQR]: 4.7–10.8). Partial arterial oxygen pressure (PaO2) of asymptomatic patients (44.4%) was higher than that of symptomatic patients (p < 0.0001). Peripheral oxygen saturation (SpO2) was ≥96% in eight patients, seven of whom had PaO2 of <70 mmHg. The hemoglobin levels were elevated at the time of diagnosing HPS (p = 0.009), whereas the platelet count was decreased (p < 0.001). The survival rates of the 66.6% of patients who underwent liver transplantation (LT) at 2 months and 1 year post‐LT were 90.3% and 84.6%, respectively. The severity of HPS did not affect the general post‐LT survival (p = 0.787).

HPS remains asymptomatic during initial stages. SpO2 may not be a reliable screening test in pediatric patients. Elevated hemoglobin levels in PH may be related to hypoxemia. The severity of hypoxemia at the time of diagnosis does not affect post‐LT survival.

The hepatopulmonary syndrome (HPS) is a portal hypertension (PH) complication that affects cirrhotic and non‐cirrhotic pediatric patients. Its prevalence is higher in patients with cirrhosis.Predictive factors for the development of HPS remain to be established.

The hepatopulmonary syndrome (HPS) is a portal hypertension (PH) complication that affects cirrhotic and non‐cirrhotic pediatric patients. Its prevalence is higher in patients with cirrhosis.

Predictive factors for the development of HPS remain to be established.

Elevated hemoglobin levels in patients with PH and hypersplenism may indicate the onset of hypoxemia and HPS.The severity of hypoxemia at the time of diagnosing HPS did not affect the post‐liver transplantation survival rates.

Elevated hemoglobin levels in patients with PH and hypersplenism may indicate the onset of hypoxemia and HPS.

The severity of hypoxemia at the time of diagnosing HPS did not affect the post‐liver transplantation survival rates.