Abstract
OBJECTIVES: To describe the clinical and laboratory characteristics and outcomes of pediatric hepatopulmonary syndrome (HPS) secondary to portal hypertension (PH) in Brazil. METHODS: 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. RESULTS: 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 (PaO(2)) of asymptomatic patients (44.4%) was higher than that of symptomatic patients (p < 0.0001). Peripheral oxygen saturation (SpO(2)) was ≥96% in eight patients, seven of whom had PaO(2) 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). CONCLUSIONS: HPS remains asymptomatic during initial stages. SpO(2) 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.