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Intrapulmonary vascular dilation in children with chronic liver diseases: pre- and post-liver transplantation

Anant Khositseth, Suporn Treepongkaruna, Khemika Khemakanok, Sumate Teeraratkul, Vichai Pansrimangkorn, Chollasak Thirapattaraphan, Surasak Leelaudomlipi


Background and study aims. Chronic liver disease (CLD) can cause hepatopulmonary syndrome (HPS), defined as triad of liver disease, hypoxemia, and intrapulmonary vascular dilation (IPVD). The aim of this study was to determine the evidence of IPVD in a cohort of pediatric patients with CLD pre- and post-liver transplantation (LT). Material and methods. All pediatric patients with CLD listed for LT were studied. Pulse oxygen saturation (SpO2), technetium-99m–labeled macroaggregated albumin (99mTc- MAA) perfusión scan (positive test: uptake of the isotope ≥ 6% in the brain), and echocardiography with saline bubble test (SBT) were performed. SBT was re-evaluated at 3-6 months after LT. Grading of SBT included grade 0 (no bubble), I (1-9 bubbles), grade II (10-20 bubbles), and grade III (> 20 bubbles). Results. Eighteen patients, median age 22.5 months (8-108), were enrolled. Most had biliary atresia (77.8%). Pre-LT, all patients had SpO2 of 100% and none had positive 99mTc- MAA perfusion scan. Two patients (11%) had negative SBT (grade 0), 1 (5.5%) had grade I, 3 (16.5%) had grade II, and 12 (67%) had grade III, respectively. Post-LT SBT became negative in all survivors (n = 16), (p = 0.0001). Conclusions. Most cirrhotic children in this cohort study had evidence of IPVD by positive SBT. However, none of these met the criteria for diagnosis of HPS. This evidence of IPVD subsided after LT.

Key words. Echocardiography, Cirrhosis, Cyanosis.

Article Metrics

The Official Journal of the Mexican Association of Hepatology, the Latin-American Association for the Study of the Liver and the Canadian Association for the Study of the Liver

ALEH Hepatología CASL ACEF Médica Sur
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