Current status of hepatopulmonary syndrome in liver transplantation in Japan: a Japanese multicenter analysis

Abstract

Background Hepatopulmonary syndrome (HPS) negatively affects the outcomes of deceased donor liver transplantation (LT). Methods We retrospectively reviewed the clinical records of patients with HPS who underwent LT and studied the impact of risk factors on clinical outcomes to determine strategies to overcome complications. Patients with symptoms of hypo‐oxygenemia and a shunt ratio >15% on 99mTc‐MAA lung perfusion scintigraphy were defined as having HPS. Results Forty‐eight patients in 10 centers were enrolled. Diseases included biliary atresia, liver cirrhosis, non‐alcoholic steatohepatitis, congenital hepatic fibrosis, and others. The length of ICU stay was 2–170 days. The respirator was used for 41.6% of patients on post‐operative day (POD) 3 and 20.8% on POD 14. The patient survival rate was 87% at 1 year and 82% at 5 years. The causes of hospital mortality were sepsis, thrombotic microangiopathy, intracranial bleeding, pulmonary fibrosis, and transplant rejection. An amount of shunt ratio prior to LT was a significant risk factor for hospital mortality. Hypoxia from POD 3 to POD 14 was a risk factor for biliary stenosis. The shunt ratio of all surviving patients significantly improved. Conclusion Although LT is feasible for patients with HPS, early transplantation and avoiding hypo‐oxygenemia immediately after transplantation are important.

Improved survival in children with HPS: Experience from two high volume liver transplant centers across continents

“…HPS is a rare disease and even a 10‐year multicentric study from 10 centers could recruit only 48 cases that included both adults and children 9 . Ours is a retrospective study on a small cohort, which limits data extraction and statistical analysis; however, it does represent a cohort of patients who have been managed with a uniform protocol, with excellent peri‐operative and long‐term outcomes.…”

“…S HPS is usually considered to carry high mortality with LT, but our series shows that 96% graft survival and 100% patient survival can be achieved, despite 60% of our cases belonging to S/VS disease categories. [8][9][10] Pulse oximetry was the routine screening tool used during the evaluation of children with liver disease, and ABG plus bubble echocardiography was performed only when low capillary oxygen saturation was detected. 11,12 However, it is possible that we may have overlooked M HPS in some children due to the <96% saturation criteria, but since HPS is usually progressive these patients would have presented for LT in due course via the same centers where their follow-up was taking place.…”

“…11,12 However, it is possible that we may have overlooked M HPS in some children due to the <96% saturation criteria, but since HPS is usually progressive these patients would have presented for LT in due course via the same centers where their follow-up was taking place. [8][9][10] We, therefore, recommend clinical assessment with SpO 2 measurement as the preliminary screening tool due to its simplicity, easy availability, and cost-effectiveness. 10 Warner et al showed that despite a majority of children with HPS being on home oxygen, the median SaO 2 of whole cohort had dropped from 91% to 88% during an average waiting period of 9 weeks to LT, illustrating that HPS is progressive.…”

“…Pulse oximetry was the routine screening tool used during the evaluation of children with liver disease, and ABG plus bubble echocardiography was performed only when low capillary oxygen saturation was detected 11,12 . However, it is possible that we may have overlooked M HPS in some children due to the <96% saturation criteria, but since HPS is usually progressive these patients would have presented for LT in due course via the same centers where their follow‐up was taking place 8–10 10 …”

Liver epigenome changes in patients with hepatopulmonary syndrome: A pilot study

“…The hepatopulmonary syndrome (HPS) is defined by the presence of an arterial oxygenation defect caused by intrapulmonary vascular dilatations in patients with chronic liver disease, mostly liver cirrhosis (LC) [1][2][3][4][5]. The prevalence of the HPS in patients assessed for liver transplant (LT) ranges between 5-30% [6][7][8]. Thirty years ago, we were the first to show that patients with LC [9][10][11] exhibit reduced or abolished pulmonary vascular tone, also distinctive of HPS.…”