ArticlePediatric surgery international2017
Onset ages of hepatopulmonary syndrome and pulmonary hypertension in patients with biliary atresia.
Article in Pediatric surgery international, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed, 20 citations in OpenAlex.
- Hepatopulmonary syndrome in children and adolescents with portal hypertension in Brazil: A multicenter study.Journal of pediatric gastroenterology and nutrition · 2026Article
- Pulmonary vascular complications in children who survive long-term after Kasai portoenterostomy for biliary atresia: a narrative review.World journal of pediatric surgery · 2025Review
- Fibrotic liver injury in biliary atresia: long-term implications.World journal of pediatric surgery · 2025Review
- Imaging in hepatopulmonary syndrome-case report. A multicenter approach during the coronavirus pandemic.Journal of radiology case reports · 2023Article
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purposeHepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) are extrahepatic complications of biliary atresia (BA). Their detection is sometimes delayed, which may result in missed opportunities for liver transplantation. The aim of this study was to determine the onset ages of HPS and PoPH in BA patients.
methodsBA patients followed at our institution were identified. Patients visited our clinic for routine blood work, as well as regular electrocardiography, chest X-rays, and arterial blood gas tests. Lung perfusion scintigraphy and cardiac ultrasound were performed to diagnose HPS. Cardiac catheterization was conducted to diagnose PoPH.
resultsThe study population consisted of 88 BA patients. The median follow-up duration was 11.6 years (range 0.8-26.0 years). Six patients (6.8%) developed HPS and three patients (3.4%) developed PoPH. The median age of onset of HPS was significantly younger than that of PoPH (HPS: 4 years, PoPH: 15 years, P < 0.019). Two patients (66%) with PoPH died, while all patients with HPS survivied.
conclusionThe onset of HPS was significantly earlier than that of PoPH. The mortality rate was high in patients with PoPH. Teenagers with BA should receive routine cardiac echocardiograms to detect PH in its early stages.
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