Hepatic venous reconstruction of the left lateral segment with emphasis on anomalous hepatic vein in pediatric liver transplantation.

Left lateral segment grafts have become a suitable option in pediatric liver transplantation (PLT). The correlation between hepatic vein (HV) reconstruction and outcome is relevant when assessing the safe use of these grafts. We retrospectively reviewed the medical records prospectively collected from a pediatric living donor liver transplantation database and conducted a comparative analysis of the different left lateral segment graft types according to HV reconstruction. Donor, recipient, and intraoperative variables were analyzed. Post-transplant outcomes included vascular complications such as hepatic vein outflow obstruction, early (≤30 d) and late (>30 d) PVT, hepatic artery thrombosis, and graft survival. From February 2017 to August 2021, 303 PLTs were performed. According to venous anatomy, the distribution of the left lateral segment was as follows: single HV (type I) in 174 (57.4%), close HVs, simple venoplasty for reconstruction (type II) in 97 (32.01%), anomalous hepatic vein (AHV) with a distance between the HVs orifices that allowed simple venoplasty (type IIIA) in 25 (8.26%) and AHV with a distance between the HVs orifices requiring homologous venous graft interposition (type IIIB) in 07 (2.31%) grafts. Type IIIB grafts came from male donors ( p =0.04) and had a higher mean donor height ( p =0.008), a higher mean graft weight, and a higher graft-to-recipient weight ratio, both p =0.002. The median follow-up time was 41.4 months. The overall cumulative graft survival was 96.3%, and comparative graft survival showed no difference (log-rank p =0.61). No hepatic vein outflow obstructions were observed in this cohort study. There was no statistically significant difference in the post-transplant outcomes between the graft types. The venous reconstruction of the AHV with homologous venous graft interposition had similar outcomes in the short and long term.

Impact of hypoxemia on pediatric liver transplantation for hepatopulmonary syndrome.

BACKGROUND: The treatment of choice for patients with cirrhosis and HPS is LT. The clinical manifestations associated with hypoxemia result in limitations and a poor health-related quality of life of affected patients. The present report aims to study the differences in outcomes between patients with PaO2  < 50 mm Hg and those with PaO2  ≥ 50 mm Hg. METHODS: This was a retrospective study of 21 patients under 18 years of age conducted from 2001 to 2018; the patients were divided into 2 groups: G1-PaO2  ≥ 50 mm Hg, 11 patients, and G2-PaO2  < 50 mm Hg, 10 patients. Demographic, clinical, laboratory, and perioperative data; outcome variables; and post-transplant survival were compared between the groups. RESULTS: In total, 2/11 (18.2%) patients in G1 and 8/10 (80%) patients in G2 required supplemental oxygen therapy at home (P = .005). Patients in G2 required prolonged MV (median 8.5 days in G2 vs 1 day in G1, P = .015) and prolonged ICU and hospital stays (P = .002 and P = .001, respectively). Oxygen weaning time was longer in G2 (median 127.5 days) than in G1 (median 3 days; P = .004). One (9.1%) patient in G1 and three (30%) patients in G2 died (P = .22). The survival at 90 months was 90.9% in G1 and 70% in G2 (P = .22). CONCLUSION: The survival between groups was similar. Patients with very severe HPS required a longer MV time, longer ICU and hospital stays, and a longer O2 weaning time than those with mild, moderate, or severe HPS.

Living related versus deceased donor liver transplantation for maple syrup urine disease.

Maple syrup urine disease (MSUD) is an inherited disorder of branched chain ketoacid (BCKA) oxidation associated with episodic and chronic brain disease. Transplantation of liver from an unrelated deceased donor restores 9-13% whole-body BCKA oxidation capacity and stabilizes MSUD. Recent reports document encouraging short-term outcomes for MSUD patients who received a liver segment from mutation heterozygous living related donors (LRDT). To investigate effects of living related versus deceased unrelated grafts, we studied four Brazilian MSUD patients treated with LRDT who were followed for a mean 19 ± 12 postoperative months, and compared metabolic and clinical outcomes to 37 classical MSUD patients treated with deceased donor transplant. Patient and graft survival for LRDT were 100%. Three of 4 MSUD livers were successfully domino transplanted into non-MSUD subjects. Following LRDT, all subjects resumed a protein-unrestricted diet as mean plasma leucine decreased from 224 ± 306 µM to 143 ± 44 µM and allo-isoleucine decreased 91%. We observed no episodes of hyperleucinemia during 80 aggregate postoperative patient-months. Mean plasma leucine:isoleucine:valine concentration ratios were ~2:1:4 after deceased donor transplant compared to ~1:1:1.5 following LRDT, resulting in differences of predicted cerebral amino acid uptake. Mutant heterozygous liver segments effectively maintain steady-state BCAA and BCKA homeostasis on an unrestricted diet and during most catabolic states, but might have different metabolic effects than grafts from unrelated deceased donors. Neither living related nor deceased donor transplant affords complete protection from metabolic intoxication, but both strategies represent viable alternatives to nutritional management.

Quality of life, depression and anxiety in potential living liver donors for pediatric recipients: A retrospective single center experience.

BACKGROUND: Living donor liver transplantation is a safe alternative for patients on a liver transplant list. Donor evaluation goes beyond physical variables to include social, emotional, and ethical aspects. The role of pre-donation sociopsychological evaluation of the donor candidate is as important to the success of the procedure as is the medical assessment. Success implies recovery from the operation and prompt engagement in pre-transplant professional and social activities, without leading to psychological or physical distress. Psychological profiling of potential living liver donors (PLLD) and evaluation of quality of life (QOL) can influence outcomes. AIM: To evaluate the socio-demographics and psychological aspects (QOL, depression, and anxiety) of PLLD for pediatric liver transplantation in a cohort of 250 patients. METHODS: This was a retrospective cohort study of 250 PLLD who underwent psychological pre-donation evaluation between 2015 and 2019. All the recipients were children. The Beck anxiety inventory, Beck depression inventory, and 36-item short-form health survey (SF-36) scores were used to evaluate anxiety (Beck anxiety inventory), depression (Beck depression inventory), and QOL, respectively. RESULTS: A total of 250 PLLD were evaluated. Most of them were women (54.4%), and the mean age was 29.2 ± 7.2 years. A total of 120 (48.8%) PLLD were employed at the time of evaluation for donation; however, most had low income (57% earned < 2 times the minimum wage). A total of 110 patients (44%) did not finish the donation process, and 247 PLLD answered a questionnaire to evaluate depression, anxiety, and QOL (SF-36). Prevalence of depression was of 5.2% and anxiety 3.6%. Although most of the PLLD were optimistic regarding the donation process and never had doubts about becoming a donor, some traces of ambivalence were observed: 46% of the respondents said they would feel relieved if a deceased donor became available. CONCLUSION: PLLD had a low prevalence of anxiety and depression. The foundation for effective and satisfactory results can be found in the pre-transplantation process, during which evaluations must follow rigorous criteria to mitigate potential harm in the future. Pre-donation psychological evaluation plays a predictive role in post-donation emotional responses and mental health issues. The impact of such findings on the donation process and outcomes needs to be further investigated.

Perioperative blood transfusion decreases long-term survival in pediatric living donor liver transplantation.

BACKGROUND: The impact of perioperative blood transfusion on short- and long-term outcomes in pediatric living donor liver transplantation (PLDLT) must still be ascertained, mainly among young children. Clinical and surgical postoperative complications related to perioperative blood transfusion are well described up to three months after adult liver transplantation. AIM: To determine whether transfusion is associated with early and late postoperative complications and mortality in small patients undergoing PLDLT. METHODS: We evaluated the effects of perioperative transfusion on postoperative complications in recipients up to 20 kg of body weight, submitted to PLDLT. A total of 240 patients were retrospectively allocated into two groups according to postoperative complications: Minor complications (n = 109) and major complications (n = 131). Multiple logistic regression analysis identified the volume of perioperative packed red blood cells (RBC) transfusion as the only independent risk factor for major postoperative complications. The receiver operating characteristic curve was drawn to identify the optimal volume of the perioperative RBC transfusion related to the presence of major postoperative complications, defining a cutoff point of 27.5 mL/kg. Subsequently, patients were reallocated to a low-volume transfusion group (LTr; n = 103, RBC ≤ 27.5 mL/kg) and a high-volume transfusion group (HTr; n = 137, RBC > 27.5 mL/kg) so that the outcome could be analyzed. RESULTS: High-volume transfusion was associated with an increased number of major complications and mortality during hospitalization up to a 10-year follow-up period. During a short-term period, the HTr showed an increase in major infectious, cardiovascular, respiratory, and bleeding complications, with a decrease in rejection complications compared to the LTr. Over a long-term period, the HTr showed an increase in major infectious, cardiovascular, respiratory, and minor neoplastic complications, with a decrease in rejection complications. Additionally, Cox hazard regression found that high-volume RBC transfusion increased the mortality risk by 3.031-fold compared to low-volume transfusion. The Kaplan-Meier survival curves of the studied groups were compared using log-rank tests and the analysis showed significantly decreased graft survival, but with no impact in patient survival related to major complications. On the other hand, there was a significant decrease in both graft and patient survival, with high-volume RBC transfusion. CONCLUSION: Transfusion of RBC volume higher than 27.5 mL/kg during the perioperative period is associated with a significant increase in short- and long-term postoperative morbidity and mortality after PLDLT.