Abstract
Introduction: HPS is chronic liver disease with impaired oxygenation caused by intrapulmonary shunting(IPS), pulmonary vessel dilation and angiogenesis. Currently only definitive treatment is liver transplantation(LT).
Aim: To compare non-invasively derived ventilation/perfusion(VA/Q) and MAA scans to quantify respiratory impairment pre&post LT.
Methods: Retrospective study of children meeting diagnostic criteria for HPS between 03/1998-05/2016. Clinical data, MAA and paired SpO2 and FiO2 pre & post LT were collected and ODC constructed to determine VA/Q ratio and R to L IPS.
Results: 16 children with HPS with diagnoses of biliary atresia(11), cryptogenic liver disease(2), progressive intrahepatic cholestasis(2) and Alagille syndrome(1). All had SpO2s of ≤94% in air, other lung pathology excluded. HPS diagnosis at median 8.5(IQR 6-12) yrs.
Symptoms: exertional dyspnoea(13), dyspnoea at rest(8), cyanosis(10) and oxygen dependency(7). MAA show median (IQR) shunting 18(13-31)% with ODC showing 20(17-25)%. 15 received a LT(1 died on LT list), median age 8.8(7-12) yrs. and waiting time 201(90-382)days.
All pulmonary symptoms resolved in median(IQR) 43(14-90)days post LT. Median shunting on ODC pre LT 10(0-24)% & post LT (median 43(17-121)days) was 0(0-0)% p=0.009. Correlation coefficient is 0.91 using mild(<20%) and mod/severe(≥20%) HPS grading. Median VA/Q pre LT 0.50(0.43-0.71) & post LT 0.61(0.54-0.71) p=0.065.
Conclusion: Non-invasive ODC and MAA yield comparable results hence are useful in HPS grading in children. R to L IPS reverses with LT with residual pulmonary dysfunction as VA/Q abnormalities persist in same time.
Footnotes
Cite this article as: European Respiratory Journal 2020; 56: Suppl. 64, 4792.
This abstract was presented at the 2020 ERS International Congress, in session “Respiratory viruses in the "pre COVID-19" era”.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2020
