Successful adult-to-adult living donor liver transplantation in a patient with moderate to severe portopulmonary hypertension.

Portopulmonary hypertension (PPHTN) is one of the most devastating consequences of end-stage liver cirrhosis. When a patient has moderate to severe PPHTN, his or her candidature for liver transplantation is denied. Here we report a successful adult-to-adult living donor liver transplantation (LDLT) in a patient with moderate to severe PPHTN. The patient was a 58-yr-old female who was diagnosed with end-stage liver cirrhosis due to chronic hepatitis C. Preoperative evaluation revealed that the patient had moderate to severe PPHTN. Her mean pulmonary artery pressure (mPAP) was 35-47 mmHg without treatment. Continuous epoprostenol therapy was introduced to lower the mPAP. She underwent LDLT using an extended right hepatic lobe graft which was donated by her daughter. Prolonged artificial ventilation was necessary until postoperative day (POD) 25, after which her general condition gradually improved. By POD 72, she was in good condition and was allowed to leave the hospital. Currently, 1 yr after the operation, she visits the outpatient clinic regularly and enjoys a normal life. It should be noted, however, that the PPHTN markedly improved but did not completely resolve, as assessed by right heart catheterization 1 yr after successful LDLT.     

Live-donor liver transplantation for acute-on-chronic hepatitis B liver failure

BACKGROUND: The survival results of patients demonstrating acute-on-chronic liver failure and undergoing live-donor liver transplantation (LDLT) have been reported to be poor. This study evaluates the survival outcomes of patients who underwent LDLT using right-lobe liver grafts for acute-on-chronic hepatitis B liver failure. METHODS: The study comprised 32 patients who demonstrated acute-on-chronic hepatitis B liver failure with mean (+/- standard error of mean) Model for End-Stage Liver Disease scores of 36+/-1.8. The mean preoperative intensive care unit stay was 2.4 days. LDLT using a right-lobe liver graft including the middle hepatic vein was performed in all patients. Oral lamivudine 100 mg daily was used for hepatitis B prophylaxis. RESULTS: The patients received liver grafts that were 52%+/-2% of the estimated standard liver weight. Hospital mortality occurred in two patients, and two other patients died on follow-up. At a median follow-up of 23 months, both patient and graft survival rates were 88%. The survival results were not different from those of 49 patients who underwent right-lobe LDLT for elective conditions during the same study period (graft survival=82%, P=0.55; patient survival=84%, P=0.75). Two (6.3%) patients developed hepatitis B virus DNA breakthrough 47 and 53 months, respectively, after transplantation, but they remained well after treatment with adefovir. CONCLUSION: Right-lobe LDLT is an effective therapeutic option for patients with acute-on-chronic hepatitis B liver failure. It results in satisfactory survival outcomes comparable to those in patients undergoing LDLT for elective conditions.     

A Retransplant Case for Hepatopulmonary Syndrome Without Liver Cirrhosis or Portosystemic Shunt After Living-Donor Liver Transplantation: A Case Report

Hepatopulmonary syndrome (HPS) is a disease of gas exchange caused by intrapulmonary shunting secondary to liver disease-associated intrapulmonary vascular dilation. HPS is characterized by the triad of cirrhosis, chronic liver disease, or portosystemic shunting (PSS); arterial hypoxemia; and intrapulmonary arteriovenous shunting in the absence of a primary cardiopulmonary anomaly. We encountered a rare case of HPS without liver disease or PSS. The patient was an 8-year-old girl who underwent living donor liver transplantation (LDLT) shortly after developing fulminant hepatitis at 11 months of her age. Eight years after LDLT, hypoxemia and shortness of breath developed. The shunt ratio on ^99mTc-macroaggregated albumin (MAA) lung perfusion scintigraphy (^99mTc-MAA lung scan) was 32%. The patient had no cardiopulmonary disease, so we diagnosed her illness as HPS. We did not find cirrhosis, chronic liver disease, or PSS as a cause of HPS. We thought the graft was the cause of HPS. A second transplantation was planned. One year after the diagnosis of HPS, the shunt ratio on ^99mTc-MAA lung scan worsened to 42%, digital clubbing appeared, and hypoxemia was worsening. Thus, we performed a second LDLT. After LDLT the shunt ratio on ^99mTc-MAA lung scan normalized (6%) and cyanosis resolved. We determined that the graft was the cause of HPS; the typical causes of HPS were not clearly revealed in the histologic examination of the second liver explant. Acute rejection occurred twice after LDLT, so we speculated that HPS occurred because the graft became stressed over the long term.     

End-stage liver cirrhosis with severe autoimmune hemolytic anemia, treated by blood type-incompatible living donor liver transplantation: a case report

We present a case of successful living donor liver transplantation (LDLT) for liver cirrhosis caused by hepatitis B virus with severe autoimmune hemolytic anemia (AIHA) using an ABO-incompatible (ABOi) graft. The patient was a 47-year-old woman who had a history of ruptured esophageal varices, accumulation of intractable ascites, frequent hepatic encephalopathy and severe anemia, with a hemoglobin value of approximately 3 g/dL due to AIHA. We treated the patient by LDLT using an ABOi liver graft. The treatment strategy included anti-CD20 antibody, plasma exchange and transfusion before LDLT. The patient's anemia improved after surgery; she required only 2 units of irradiated red blood cell concentrates-leukocytes reduced. The patient was discharged from the hospital on postoperative day 35. Two years after surgery, the patient still shows normal hepatic and hematological findings. The immunomodulation protocol for ABOi LDLT was effective not only to avoid humoral reactions associated with ABOi LDLT, but also those associated with AIHA.     

Right-lobe live donor liver transplantation improves survival of patients with acute liver failure

BACKGROUND: Right-lobe live donor liver transplantation (LDLT) is used by many liver transplant centres for treating adult patients with terminal liver disease, but its incremental benefit for the intended recipient over cadaveric liver graft transplantation has not been determined. The impact of LDLT as a proactive approach on the outcome of patients with acute liver failure was analysed. METHODS: From January 1999 to March 2001, right-lobe LDLT was offered proactively to 50 consecutive patients with acute liver failure and their families. The outcome of those who opted for right-lobe LDLT (n = 34) was compared with that of those who did not opt for LDLT (n = 16). RESULTS: In the group that opted for right-lobe LDLT, 16 patients eventually received a live donor right-lobe graft (14 patients survived) and three patients received a cadaveric liver graft that became available while the potential live donor was undergoing evaluation (all three patients survived). Among the group who did not opt for LDLT, only one patient received a cadaveric liver graft and survived. The former group had a higher overall survival rate (17 of 34 versus one of 16). With a proactive approach, the overall transplant rate was increased from four of 50 to 20 of 50. The morbidity rate among donors was low and none died. CONCLUSION: Right-lobe LDLT improves the overall survival rate of patients with acute liver failure and should be considered as one of the treatment options for adult patients with acute liver failure.     

Risk factors for graft dysfunction after adult-to-adult living donor liver transplantation

The aim of this study was to investigate the risk factors for graft dysfunction after adult-to-adult living donor liver transplantation (LDLT). Thirty-nine adults with chronic cirrhosis underwent LDLT between 1999 and 2004. Their postoperative courses were uneventful with no vascular or bile duct complications early after LDLT, except one mild hepatic artery stenosis. The preoperative MELD scores were significantly higher in the failed graft group (n=5) than the functioning graft group (n=34; P=.004), while the graft liver weight/standard liver volume ratio was similar between these groups. We concluded that a high preoperative MELD score was associated with postoperative graft failure and that graft size had little impact on graft outcome. Although large grafts would seem intuitively more suitable for sick recipients, we did not show a benefit among this cohort; the MELD score was the best predictor, a finding that is also most consistent with donor safety.     

Successful Treatment of Intraoperative Heart Failure Caused by Ampulla Cardiomyopathy by Intra-aortic Balloon Pumping and Percutaneous Cardiopulmonary Support: Report of a Case

An 82-year-old woman underwent total gastrectomy for advanced gastric cancer with invasion to the lower esophagus. Her blood pressure dropped alarmingly during the operation, which was performed via the transabdominal and left-side transthoracic approach. Using echocardiography, we diagnosed intraoperative-oneset reversible heart failure caused by ampulla cardiomyopathy. Because the infusion of catecholamines is associated with secondary heart failure, we gave her calcium antagonists and nicorandil, then started intra-aortic balloon pumping (IABP) and the percutaneous cardiopulmonary support system (PCPS). On postoperative day (POD) 7, the IABP and PCPS were removed and on POD 12, she was extubated successfully. The patient was discharged on POD 54 and has remained well. The factors predisposing her to ampulla cardiomyopathy were left-side thoracotomy, hypoxia caused by one-lung ventilation, and the infusion of high-dose catecholamines. Prompt diagnosis and timely treatment of the heart failure with IABP and PCPS prevented any further complications.     

Successful living donor liver transplantation for liver failure due to maternal T cell engraftment following cord blood transplantation in X-linked severe combined immunodeficiency disease: Case report

Maternal T cells from perinatal transplacental passage have been identified in up to 40% of patients with severe combined immunodeficiency (SCID). Although engrafted maternal T cells sometimes injure newborn tissue, liver failure due to maternal T cells has not been reported. We rescued a boy with X-linked SCID who developed liver failure due to engrafted maternal T cell invasion following living donor liver transplantation (LDLT) following unrelated umbilical cord blood transplantation (UCBT). After developing respiratory failure 3 weeks postpartum, he was diagnosed with X-linked SCID. Pathological findings showed maternal T cells engrafted in his liver and hepatic fibrosis gradually progressed. He underwent UCBT at 6 months, but hepatic function did not recover and liver failure progressed. Therefore, he underwent LDLT using an S2 monosegment graft at age 1.3 years. The patient had a leak at the Roux-en-Y anastomosis, which was repaired. Despite occasional episodes of pneumonia and otitis media, he is generally doing well 6 years after LDLT with continued immunosuppression agents. In conclusion, the combination of hematopoietic stem cell transplantation (HSCT) and liver transplantation may be efficacious, and HSCT should precede liver transplantation for children with X-linked SCID and liver failure.     

Langerhans' cell histiocytosis after living donor liver transplantation: report of a case.

We report a case of Langerhans' cell histiocytosis (LCH) occurring after a living donor liver transplantation (LDLT) for fulminant hepatitis. A 9-month-old girl underwent an LDLT for fulminant hepatitis of an unknown cause. The histology of the native liver did not show any findings of LCH. On postoperative day 42, her Epstein-Barr virus (EBV)-DNA and cytomegalovirus antigenemia were both found to be positive. As a result, she was treated with antiviral agents and a reduction of the immunosuppression dosage. On postoperative day 98, acute rejection occurred, and she was treated with FK506, methylprednisolone, and finally, anti-CD3 murine monoclonal antibody was added. Subsequently, the EBV was re-activated. Thereafter, skin eruptions, swelling of the systemic lymph nodes, and pancytopenia appeared on postoperative day 127. LCH was diagnosed based on the typical histological findings as LCH, CD1a, and S-100-positive cells in her skin and a lymph nodes biopsy. She was treated by chemotherapy. The symptoms disappeared a few weeks after the start of the chemotherapy, and a clinical remission of LCH was obtained. We could not detect any evidence of EBV infection in the tumor cells. In spite of the fact that her LCH lesions thereafter remained in remission, she died of hepatic failure at 22 months after undergoing the liver transplantation. In conclusion, we discuss the factors influencing the occurrence of LCH in our patient after LDLT, while also evaluating the relationship between LCH and the immunosuppressive therapy administered to this patient.     

Monosegmental living donor liver transplantation

BACKGROUND: Living donor liver transplant (LDLT) program has been started from 1990 in Japan, and is still major form of liver transplantation because of the scarcity of cadaveric donor organs. In small infants, implantation of left lateral segment grafts can be a problem because of a large-for-size graft. Until November 2002, we performed 867 transplants for 828 patients (561 children and 306 adults), and 14 cases received monosegment grafts from living donors. METHODS: Fifteen patients, median age 211 days, median weights 5.95 kg, received monosegmental LDLT. The indication for using this technique was infants with an estimated graft-to-recipient weight ratio of over 4.0%. RESULTS: Graft and patient survival is 85.7%. There were no differences in donor operation time and blood loss between monosegmentectomy and left lateral segmentectomy. Segment III grafts were indicated in 13 cases. Two vascular complications were observed (one hepatic artery thrombosis and one portal vein thrombosis). CONCLUSIONS: Monosegental living donor liver transplantation is a feasible option with satisfactory graft survival in small babies with liver failure.     

Adult-to-adult living donor liver transplantation using extended right lobe grafts.

OBJECTIVE: The authors report their experience with living donor liver transplantation (LDLT) using extended right lobe grafts for adult patients under high-urgency situations. SUMMARY BACKGROUND DATA: The efficacy of LDLT in the treatment of children has been established. The major limitation of adult-to-adult LDLT is the adequacy of the graft size. A left lobe graft from a relatively small volunteer donor will not meet the metabolic demand of a larger recipient. METHODS: From May 1996 to November 1996, seven LDLTs, using extended right lobe grafts, were performed under high-urgency situations. All recipients were in intensive care units before transplantation with five having acute renal failure, three on mechanical ventilation, and all with hepatic encephalopathy. The median body weight for the donors and recipients was 58 kg (range, 41-84 kg) and 65 kg (range, 53-90 kg), respectively. The body weights of four donors were less than those of the corresponding recipients, and the lowest donor-to-recipient body weight ratio was 0.62:1. The extended right lobe graft was chosen because the left lobe volume was <40% of the ideal liver mass of the recipient. RESULTS: Median blood loss for the donors was 900 mL (range, 700-1600 mL) and hospital stay was 19 days (range, 8-22 days). Homologous blood transfusion was not required. Two donors had complications (one incisional hernia and one bile duct stricture) requiring reoperation after discharge. All were well with normal liver function 5 to 10 months after surgery. The graft weight ranged from 490 g to 1140 g. All grafts showed immediate function with normalization of prothrombin time and recovery of conscious state of the recipients. There was no vascular complication, but six recipients required reoperation. One recipient died of systemic candidiasis 16 days after transplantation and 6 (86%) were alive with the original graft at a median follow-up of 6.5 months (range, 5-10 months). CONCLUSIONS: When performed by a team with experience in hepatectomy and transplantation, LDLT, using an extended right lobe graft, can achieve superior results. The technique extends the success of LDLT from pediatric recipients to adult recipients and opens a new donor pool for adults to receive a timely graft of adequate function.     

Living domino liver transplantation in an adult with congenital absence of portal vein.

Congenital absence of the portal vein (CAPV) is a rare malformation of the splanchnic venous system. Although CAPV is usually detected in the pediatric age group, our patient was a 35-year-old woman. She had been diagnosed with CAPV in 1996 when she was 27 years old. In 1998, she was placed on hemodialysis due to chronic renal failure. After several episodes of encephalopathy in 2002, liver transplantation (LT) was recommended to her and her family. Since there was no suitable living donor candidate, she was put on the waiting list for a deceased donor liver transplant in Japan. In 2004, her ammonia level increased to around 300 microg/dl, and she went into a coma lasting for three days. After recovering from this event, she underwent a living domino transplantation using a whole liver donated by a familial amyloid polyneuropathy (FAP) patient. Her portal vein, which had drained directly into the inferior vena cava (IVC), was transected together with a cuff of the IVC wall and anastomosed to the graft liver portal vein in an end-to-end fashion. In conclusion, liver transplantation proved to be a safe and effective way to save this patient and improve her quality of life.     

Four hundred thirty consecutive pediatric living donor liver transplants: variables associated with posttransplant patient and graft survival

The availability of living donors allows transplant teams to indicate living donor liver transplantation (LDLT) early in the course of liver disease before the occurrence of life-threatening complications. Late referral to transplant centers is still a problem and can compromise the success of the procedure. The aim of this study was to examine the perioperative factors associated with patient and graft survival for 430 consecutive pediatric LDLT procedures at Sirio-Libanes Hospital/A. C. Camargo Hospital (S?o Paulo, Brazil) between October 1995 and April 2011. The studied pretransplant variables included the following: recipient age and body weight, Pediatric End-Stage Liver Disease score, z score for height/age, bilirubin, albumin, international normalized ratio, hemoglobin, sodium, presence of ascites, and previous surgery. The analyzed technical aspects included the graft-to-recipient weight ratio and the use of vascular grafts for portal vein reconstruction. In addition, the occurrence of hepatic artery thrombosis (HAT), portal vein thrombosis (PVT), and biliary complications was also analyzed. The liver grafts included 348 left lateral segments, 5 monosegments, 51 left lobes, and 9 right lobes. In a univariate analysis, an age < 12 months, a low body weight (≤10 kg), malnutrition, hyperbilirubinemia, and HAT were associated with decreased patient and graft survival after LDLT. In a multivariate analysis, a body weight ≤ 10 kg and HAT were significantly associated with decreased patient and graft survival. The use of vascular grafts significantly increased the occurrence of PVT. In conclusion, a low body weight (≤10 kg) and the occurrence of HAT independently determined worse patient and graft survival in this large cohort of pediatric LDLT patients.     

Critical graft size and functional recovery in living donor liver transplantation

With a high prevalence of chronic hepatitis B and a low cadaveric organ donation rate, living donor liver transplantation (LDLT) remains the only option for many patients in Hong Kong. In such cases, the liver graft volume is smaller owing to a partial liver graft; therefore, a problem of small-for-size grafts often occurs. Between September 1999 and April 2003, 25 cadaveric, 16 living related, and 1 auto-LTs were performed at our center. The outcomes of LDLT were analyzed to assess the critical graft size and functional recovery. Among the 16 LDLT recipients (mean age, 44.4 +/- 14.4 years; mean weight, 61.9 +/- 11.4 kg), 1 patient received a graft from a donor left lobe (weight, 400 g) in an auxillary partial orthotopic LT (APOLT), 12 received right lobes, and 3 received left lobes. Besides the APOLT case, the overall graft/recipient weight ratio (GRWR) for the 15 LDLTs was 1.11 (0.76 to 1.75). The GRWR in the 25 cadaveric LTs was 1.92 (1.05 to 3.69) (P < .001). Among the 12 successful LDLTs, there were 5 (41.7%) cases of small-for-size graft syndrome: 3 of 3 (100%) in GRWR < or = 0.8%; 5 of 6 (83.3%) in GRWR < 1%; and 0 of 6 with GRWR > 1%. The initial post-LT graft function parameters were significantly higher among the LDLT group: International normalized ratio (INR), 1.42 vs 1.24, P = .03; alanine aminotransferase (ALT, 387 vs 201 IU/L, P = .005, and bilirubin, 170 vs 48 micromol/L, P < .001 as compared to the cadaveric transplant group). Small-for-size graft syndrome can be avoided if GRWR > 1%, but often occurs when GRWR < 0.8%. Graft function in LDLT recovers more slowly than in cadaveric liver transplant.     

肝移植多模式策略的初步探讨

目的：总结多种临床肝移植技术的经验。方法：对2000年9月至2002年3月完成的25例临床肝移植（27次移植手术）的资料进行回顾性分析。包括尸肝移植14例,活体肝移植11例,再次尸肝移植1次,减体积再次肝移植1次。结果：11例活体肝移植的供体中,10例为患者之母亲,1例急诊成人右叶活体肝移植供体为患者之妹。供肝方式：扩大左半肝6例,右半肝3例,右半肝2例;切取供肝重量270－620g。全组存活24例,1例成人活体肝移植受体于术后72d死于不可逆转的严重排斥反应。肝炎患者采用拉米呋啶加抗HVB－Ig治疗,10例乙肝、肝硬化患者术后随访时间4－21个月,复查HVB－DNA均为阴性。所有肝豆状核变性受体术后随访复查6－17个月,铜氧化酶、肝功能均正常。本组主要并发症包括：腹腔出血2例,需再次剖腹探查止血;ARDS 5例;急性肾功能衰竭2例;排斥反应4例,其中1例导致死亡。结论：综合开展包括尸体肝移植、活体肝移植、减体积肝移植等在内的各项技术,充分利用有限的供肝,是优化肝移植资源,提高移植疗效的重要途径。     

Long-term outcomes of 600 living donor liver transplants for pediatric patients at a single center.

This report concerns the long-term outcome of living donor liver transplantation (LDLT) for pediatric patients at a single center. Between June 1990 and December 2003, a total of 600 LDLTs, including 568 primary transplantations and 32 retransplantations, were performed for pediatric patients, who were immunosuppressed with FK506 and low-dose corticosteroids. Patient survival at 1, 5, and 10 years were 84.6%, 82.4%, and 77.2%, respectively, and the corresponding findings for graft survivals were 84.1%, 80.9%, and 74.5%. Multivariate analysis demonstrated that fulminant hepatic failure (FHF), a graft vs. body weight (GBWR) ratio of <0.8, and ABO-incompatible transplants were independently associated with both patient and graft survival. The retransplantation rate was 6%, and 55 patients (9.7%) have been completely weaned off immunosuppressants. Long-term patient and graft survival after pediatric LDLT for a large cohort of children at our hospital were found to be as good as those for cadaveric liver transplantation, although this series includes 13% liver transplantations with ABO-incompatible donors, which are obviously inferior in patient and graft survival. To obtain better outcomes for patients with FHF and for patients with ABO-incompatible transplants, immunosuppressive therapy needs to be improved.     

Live donor liver transplantation in adults

Live donor liver transplantation (LDLT) was initiated in 1988 for children recipients. Its application to adult recipients was limited by graft size until the first right liver LDLT was performed in Hong Kong in 1996. Since then, right liver graft has become the major graft type. Despite rapid adoption of LDLT by many centers, many controversies on donor selection, indications, techniques, and ethics exist. With the recent known 11 donor deaths around the world, transplant surgeons are even more cautious than the past in the evaluation and selection of donors. The need for routine liver biopsy in donor evaluation is arguable but more and more centers opt for a policy of liberal liver biopsy. Donation of the middle hepatic vein (MHV) in the right liver graft was considered unsafe but now data indicate that the outcome of donors with or without MHV donation is about equal. Right liver LDLT has been shown to improve the overall survival rate of patients with chronic liver disease, acute or acute-on-chronic liver failure and hepatocellular carcinoma waiting for liver transplantation. The outcome of LDLT is equivalent to deceased donor liver transplantation despite a smaller graft size and higher technical complexity.     

活体肝移植治疗HBV相关性急性亚急性肝功能衰竭

目的 探讨活体肝移植(living donor liver transplantation,LDLT)HBV感染导致的急性肝功能衰竭(acute liver failure,ALF)和亚急性肝功能衰竭(subacute liver failure,SALF)患者的可行性,并评价其疗效.方法 回顾性分析2000年11月至2007年10月完成的10例LDLT治疗ALF、SALF患者的临床资料.10例LDLT的供、受者均为成人,切取右半肝为移植物,8例含肝中静脉(middle hepatic vein,MHV).10例供者的评估均在确定实施LDLT的24 h内完成,供、受者手术均在确定供者后的12 h内完成.移植物质量与受者体质量比为(1.03±0.17)%(0.86%～1.22%),移植物体积与受者标准肝体积比为(52.2±11.8)%(47.6%～70.1%).结果 10例受者中,2例分别于术后7、28 d时因肺部感染、十二指肠球部溃疡穿孔腹腔感染死亡.1例胆管吻合口胆漏,经十二指肠镜下置入鼻胆管引流治愈.2例术后1周出现轻度急性排斥反应,增强免疫抑制强度后肝功能恢复正常.8例中位随访期9.6个月(2～84个月),生存质量优良.10例供者中,1例出现急性门静脉高压症导致脾脏破裂,行脾脏切除术,其后出现胆管断端胆漏,经鼻胆管引流结合经皮穿刺腹腔引流治愈.其余9例无并发症发生.结论 LDLT适宜治疗HBV感染导致的ALF、SALF,而且能获得较好的中、远期疗效.     

Determinants of hospital mortality of adult recipients of right lobe live donor liver transplantation

OBJECTIVE: To define the technical factors that might contribute to hospital mortality of recipients of right lobe live donor liver transplantation (LDLT) so as to perfect the design of the operation. SUMMARY BACKGROUND DATA: Right lobe LDLT has been accepted as one of the treatments for patients with terminal hepatic failure, but the design and results of the reported series vary and the technical factors affecting hospital mortality have not been known. METHODS: The data of 100 adult-to-adult right lobe LDLT performed between 1996 and 2002 were prospectively collected and retrospectively analyzed. All grafts except one contained the middle hepatic vein, which was anastomosed to the recipient middle/left hepatic vein in the first 84 recipients and directly into the inferior vena cava (with the right hepatic vein in form of venoplasty) in the subsequent 15 patients. Venovenous bypass was used routinely in the first 29 patients but not subsequently. RESULTS: Eight patients died within the same hospital admission for liver transplantation. There was no hospital mortality in the last 53 recipients. Comparison of data of patients with or without hospital mortality showed that graft weight/body weight ratio, graft weight/estimated standard liver weight ratio, technical error resulting in occlusion/absence of the middle hepatic vein, use of venovenous bypass, the lowest body temperature recorded during surgery, the volume of intraoperative blood transfusion, fresh frozen plasma, and platelet infusion were significantly different between the two groups. However, the pretransplant intensive care unit status of the recipients, cold and warm ischemic time of the graft, and occurrence of biliary complications were not. By multivariate analysis, low body temperature recorded during operation, low graft weight/estimated standard liver weight ratio (35% of the estimated standard graft weight may be sufficient for recipient survival. Hypothermia, which predisposes to coagulopathy and is enhanced by the use of venovenous bypass and massive blood, and blood product transfusion must be avoided.     

Bevacizumab reverses need for liver transplantation in hereditary hemorrhagic telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease characterized by mucocutaneous and visceral telangiectasia. Hepatic involvement with vascular malformations may lead to portal hypertension, biliary ischemia, and high-output cardiac failure. Liver transplantation is indicated for life-threatening disease but carries significant risk from surgery and chronic immunosuppression. We report a case of a 47-year-old woman with HHT successfully treated with the vascular endothelial growth factor (VEGF) antibody bevacizumab. The patient was referred for consideration of liver transplantation because of hepatic HHT leading to high-output cardiac failure, diuretic resistant ascites, cholestasis, and malnutrition. As she was considered a high-risk candidate for transplantation, she underwent 6 courses of bevacizumab (5 mg/kg) over 12 weeks. A dramatic improvement in her clinical state was observed after 3 months with reversal of cholestasis, resolution of cardiac failure and ascites, and improvement in nutritional status with a 10% dry weight increase. Treatment induced a marked reduction in liver vascularity and halving of her liver volume from 4807 to 2269 mL over 6 months. This was associated with normalization of her cardiac output from 10.2 to 5.1 L/minute. Correspondingly, she ceased diuretic medications, returned to full-time work, and was delisted as a transplant candidate. She remains well 6 months after completing treatment. In conclusion, antagonism of VEGF receptors led to a dramatic regression of hepatic vascular malformations and reversal of high-output cardiac failure and complications of portal hypertension in this patient with HHT. Bevacizumab may potentially alleviate the need for liver transplantation in this group of patients.