[Hepatopulmonary syndrome in children: evaluation and treatment]
S Rovira Amigo1, C Martín de Vicente, J Bueno Recio
1Unidad de Neumología Pediátrica y Fibrosis Quística, Hospital Universitari Vall d'Hebron, Barcelona, España. 42402sra@comb.cat
Insights
Hepatopulmonary syndrome, a rare liver disease complication, presents with hypoxemia and intrapulmonary shunting. This study details five pediatric cases, highlighting varied disease types and tailored therapeutic strategies for improved outcomes.
Area of Science:
- Medicine
- Cardiology
- Pulmonology
Background:
- Hepatopulmonary syndrome (HPS) is a rare complication of liver disease, affecting patients of all ages.
- Diagnosis relies on identifying hypoxemia and intrapulmonary shunting via imaging techniques.
Observation:
- This study presents five pediatric cases of hepatopulmonary syndrome.
- The cases exhibited diverse natural histories and clinical evolutions.
Findings:
- Two patients were diagnosed with HPS type 1, and two with HPS type 2.
- One patient presented with features of both HPS type 1 and type 2.
Implications:
- The distinct HPS types necessitate individualized therapeutic approaches.
- Understanding these classifications is crucial for effective pediatric HPS management.
Abstract:
Hepatopulmonary syndrome is a rare disease that affects patients of any age with acute or chronic liver disease. Its diagnosis is based on the presence of hypoxemia and the demonstration of an intrapulmonary shunting by echocardiography with contrast or perfusion lung scanning. Pulmonary angiography is useful to demonstrate macroscopic arteriovenous communications. We describe five paediatric cases with a different natural history and evolution. Two of them were diagnosed with hepatopulmonary syndrome type 1, another two with type 2 and a fifth one with type 1 and 2, which required a different therapeutic approach in each case.
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