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Intrapulmonary vascular dilation in children with chronic liver diseases: pre- and post-liver transplantation
Anant Khositseth1, Suporn Treepongkaruna2, Khemika Khemakanok1
1Department of Surgery. Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Insights
Pediatric liver disease patients often show intrapulmonary vascular dilation (IPVD), a key factor in hepatopulmonary syndrome (HPS). This IPVD significantly improved after liver transplantation (LT), indicating LT
Area of Science:
- Pediatric Gastroenterology and Hepatology
- Cardiopulmonary Medicine
- Transplantation Science
Background:
- Chronic liver disease (CLD) can lead to hepatopulmonary syndrome (HPS).
- HPS is characterized by liver disease, hypoxemia, and intrapulmonary vascular dilation (IPVD).
- Evidence of IPVD in pediatric CLD patients pre- and post-liver transplantation (LT) requires investigation.
Purpose of the Study:
- To assess the prevalence of IPVD in pediatric CLD patients.
- To evaluate changes in IPVD following LT.
- To determine the relationship between IPVD and HPS criteria in this cohort.
Main Methods:
- Study included pediatric CLD patients listed for LT.
- Evaluated pulse oxygen saturation (SpO2), technetium-99m-labeled macroaggregated albumin ((99m)Tc-MAA) perfusion scans, and echocardiography with saline bubble test (SBT).
- SBT was reassessed 3-6 months post-LT, with grading from 0 to III based on bubble count.
Main Results:
- Eighteen pediatric patients (median age 22.5 months) were enrolled; most had biliary atresia.
- Pre-LT, all patients had normal SpO2, and no positive (99m)Tc-MAA scans; however, 89% showed positive SBT indicating IPVD (11% grade 0, 5.5% grade I, 16.5% grade II, 67% grade III).
- Post-LT, SBT normalized in all 16 survivors (p=0.0001), indicating resolution of IPVD.
Conclusions:
- The majority of pediatric CLD patients in this cohort exhibited IPVD, detectable by SBT.
- Despite IPVD, none met the full diagnostic criteria for HPS.
- Liver transplantation effectively resolved the evidence of IPVD in surviving patients.
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 (SpO(2)), technetium-99m-labeled macroaggregated albumin ((99m)Tc- 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 SpO(2) of 100% and none had positive (99)mTc- 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.
