HEPATOPULMONARY SYNDROME IN PEDIATRIC PATIENTS WITH PORTAL HYPERTENSION - AN INTEGRATIVE REVIEW.

BACKGROUND: Hepatopulmonary syndrome (HPS) is characterized by the triad of abnormal arterial oxygenation caused by intrapulmonary vascular dilatations (IPVD) in the setting of advanced liver disease or portal hypertension, impacting the patient's quality of life and survival. There are still many gaps in the literature on this topic, especially in pediatrics, with practices frequently based on extrapolation of data obtained from adults. OBJECTIVE: Provide a synthesis of the current knowledge about HPS in children. METHODS: The research was carried out through narrative review. The databases used for the search include Medline, Embase, Elsevier, Lilacs and Scielo. The keywords used were "hepatopulmonary syndrome" AND child, children, infant, preschool, pediatric. RESULTS: In cirrhotic children, the prevalence of HPS can reach up to 42.5%, and it is even more common in those whose underlying condition is biliary atresia, reaching up to 63%. Screening with pulse oximetry (O2 saturation <96%), unlike in adults, has low sensitivity in the pediatric age group. Management involves supportive care with oxygen therapy; liver transplantation is the only definitive treatment to reverse the condition and HPS is considered an exceptional criterion for waitlist. The waitlist mortality is similar among children listed by HPS as a special criterion when compared to those listed for other reasons. The reported rates of complete resolution of hypo-xemia after liver transplantation are close to 100% in children. The post-liver transplantation survival is similar or slightly lower in children with HPS when compared to those without HPS. Contrary to findings from adults, no differences were found in post- liver transplantation mortality between children of different hypoxemia ranges, although longer mechanical ventilation time and hospital stay were observed in children with PaO2 <50 mmHg. CONCLUSION: HPS is not an uncommon complication of cirrhosis in children and adolescents, particularly when biliary atresia is the underlying condition. There are still many gaps to be filled regarding the condition, and this article demonstrates that not all data obtained in studies with adults reflects the disease's behavior in pediatrics, especially concerning prognosis.

Síndrome hepatopulmonar em pacientes pediátricos com hipertensão portal - uma revisão integrativa / Hepatopulmonary syndrome in pediatric patients with portal hypertension - an integrative review

ABSTRACT Background: Hepatopulmonary syndrome (HPS) is characterized by the triad of abnormal arterial oxygenation caused by intrapulmonary vascular dilatations (IPVD) in the setting of advanced liver disease or portal hypertension, impacting the patient's quality of life and survival. There are still many gaps in the literature on this topic, especially in pediatrics, with practices frequently based on extrapolation of data obtained from adults. Objective: Provide a synthesis of the current knowledge about HPS in children. Methods: The research was carried out through narrative review. The databases used for the search include Medline, Embase, Elsevier, Lilacs and Scielo. The keywords used were "hepatopulmonary syndrome" AND child, children, infant, preschool, pediatric. Results: In cirrhotic children, the prevalence of HPS can reach up to 42.5%, and it is even more common in those whose underlying condition is biliary atresia, reaching up to 63%. Screening with pulse oximetry (O2 saturation <96%), unlike in adults, has low sensitivity in the pediatric age group. Management involves supportive care with oxygen therapy; liver transplantation is the only definitive treatment to reverse the condition and HPS is considered an exceptional criterion for waitlist. The waitlist mortality is similar among children listed by HPS as a special criterion when compared to those listed for other reasons. The reported rates of complete resolution of hypo­xemia after liver transplantation are close to 100% in children. The post-liver transplantation survival is similar or slightly lower in children with HPS when compared to those without HPS. Contrary to findings from adults, no differences were found in post- liver transplantation mortality between children of different hypoxemia ranges, although longer mechanical ventilation time and hospital stay were observed in children with PaO2 <50 mmHg. Conclusion: HPS is not an uncommon complication of cirrhosis in children and adolescents, particularly when biliary atresia is the underlying condition. There are still many gaps to be filled regarding the condition, and this article demonstrates that not all data obtained in studies with adults reflects the disease's behavior in pediatrics, especially concerning prognosis.

RESUMO Contexto: A síndrome hepatopulmonar (SHP) é caracterizada pela tríade de oxigenação arterial anormal causada por dilatações vasculares intrapulmonares (DVIP) no contexto de doença hepática ou hipertensão portal, com impacto na qualidade de vida e sobrevida dos pacientes. Há ainda muitas lacunas na literatura sobre este tema, especialmente na Pediatria, cujas práticas são frequentemente extrapoladas de dados obtidos entre pacientes adultos. Objetivo: Apresentar uma síntese do conhecimento atual sobre a SHP na faixa etária pediátrica. Métodos: Trata-se de uma revisão narrativa. As bases de dados para pesquisa foram Medline, Embase, Elsevier, Lilacs e Scielo. As palavras-chave utilizadas foram "hepatopulmonary syndrome" AND child, children, infant, preschool, pediatric. Resultados: Nas crianças cirróticas, a prevalência da SHP pode chegar a 42,5%, sendo ainda mais comum naquelas com atresia biliar, atingindo até 63%. A triagem com oximetria de pulso (saturação de O2 <96%), diferentemente dos adultos, tem baixa sensibilidade na faixa etária pediátrica. O manejo envolve cuidados de suporte com oxigenoterapia; o transplante de fígado é o único tratamento definitivo para reverter o quadro e a SHP é considerada situação especial para alocação na lista de espera. A mortalidade em lista é semelhante entre as crianças com SHP elencadas como situação especial quando comparada àquelas elencadas por outros motivos. As taxas de resolução completa da hipoxemia após o transplante de fígado são próximas de 100% em crianças. A sobrevida pós-transplante de fígado é semelhante ou ligeiramente menor em crianças com SHP quando comparadas àquelas sem SHP. Ao contrário dos achados em adultos, não foi observada diferença na mortalidade pós-transplante nas crianças de diferentes faixas de gravidade da hipoxemia, embora tenha sido observado maior tempo de ventilação mecânica e internação hospitalar em crianças com PaO2<50mmHg. Conclusão: A SHP não é uma complicação incomum na cirrose em crianças e adolescentes, principalmente quando a atresia biliar é a condição subjacente. Ainda há muitas lacunas a serem preenchidas em relação ao quadro, e este artigo demonstra que nem todos os dados obtidos em estudos com adultos refletem o comportamento da doença na pediatria, principalmente no que diz respeito ao prognóstico.