Progression of cardiomyopathy after liver transplantation in patients with familial amyloidotic polyneuropathy, Portuguese type

BACKGROUND: Transthyretin amyloidosis is today an accepted indication for orthotopic liver transplantation (OLT). For several mutations progression of the cardiomyopathy has been observed after OLT. The aim of this study was to assess the course of cardiac involvement in Swedish familial amyloidotic polyneuropathy (FAP), Portuguese type, after OLT. By comparison of the echocardiographic findings before OLT with those obtained after, the course of the heart involvement was followed. METHODS: Twenty-three patients, who had undergone OLT and were examined with echocardiography 1-12 months before OLT, were available for the study. Twenty-one patients were examined 12-27 months after OLT, and 12 were re-examined 52-71 months after OLT. Two-dimensional and M-mode echocardiography were performed in accordance with the standards of the American Society of Echocardiography. RESULTS: A significantly increased septal and left ventricular posterior wall thickness and a significantly increased left atrial dimension was observed at the post-OLT examinations, indicating a progression of the amyloid heart disease. This increase of the cardiac involvement was neither correlated to waiting time for OLT or to pre-operative signs of cardiomyopathy. CONCLUSIONS: Even though the production of the amyloidogenic-mutated transthyretin is stopped by OLT, the cardiomyopathy may progress after the operation even for the Portuguese type of FAP. The increase of the septal and left ventricular posterior wall thickness after OLT is not restricted to patients with signs of left ventricular hypertrophy before the transplantation. The findings have important implications for the follow-up of FAP patients after OLT.     

肝移植术后近期心血管系统并发症的原因及诊治

目的 总结和探讨肝移植术后近期心血管并发症的发生率、原因及其诊治方法.方法 回顾性分析北京佑安医院2004年6月至2006年1月间121例次肝移植病例术后近期(术后2周内)各种心血管系统并发症的发生率及其诊治资料.结果 在121肝移植病例中有30例(24.8%)病人发生心血管系统并发症,其中术后高血压11例(9.1%),心力衰竭9例(7.4%),心肌缺血性疾病(包括心绞痛和心肌梗死)7例(5.8%),心律失常12例(9.9%),其中阵发性室上性心动过速3例(2.5%),室性心动过速3例(2.5%),严重窦性心动过缓6例(4.9%).由心血管并发症引起的死亡为4例(3.3%).心力衰竭的发生率主要同既往有无心脏病史有明显关系(X2=20.56,P<0.01);与术后液体出入量亦有明显关系(X2=17.14,P<0.01).心肌缺血性疾病的可能原因为术后凝血功能紊乱;术后循环状况不稳定有关.心律失常主要与终末期肝病自主神经功能紊乱、QT间期延长;术后早期电解质及酸碱平衡紊乱;Swan-Ganz导管刺激心内膜有关.术后早期高血压与多种因素有关.结论 心血管系统并发症是肝移植术后常见的并发症,可导致死亡.术前的仔细评估.术后控制液体输入量及输液速度、维持电解质、酸碱平衡及凝血功能稳定,对于具有心血管危险因素的病人应加强监测和评估、并给予预防性用药可降低心血管并发症和相关死亡率.     

Changes in renal function in patients with familial amyloid polyneuropathy treated with orthotopic liver transplantation.

BACKGROUND: Familial amyloid polyneuropathy (FAP) is an autosomal dominant disease caused by a point mutation in the gene encoding transthyretin, which is secreted by the liver. Orthotopic liver transplantation (OLT) has been proposed to prevent disease progression. Little is known about long-term changes in renal function and lesions after OLT. METHODS: The renal function of 33 patients with FAP was evaluated (proteinuria, serum creatinine, creatinine clearance) before OLT and over a period of at least 5 years afterwards. A pre-transplantation renal biopsy was performed in 14 patients and a follow-up biopsy in eight patients. RESULTS: Before transplantation, mean serum creatinine concentration was 86 micromol/l (47-126 micromol/l) and creatinine clearance was 71.9+/-31.6 ml/min/1.73 m(2). Proteinuria was detected in 54% of patients (0.3-4 g/day). Pre-transplant renal biopsies (n = 14) revealed glomerular, tubular and vascular amyloid deposits in 90, 58 and 66% of patients, respectively. Eleven patients (33%) died after OLT. Death occurred most frequently in patients having weight losses >7 kg (P<0.05). After transplantation, 25 patients (76%) suffered acute renal failure but only one required dialysis. One month after transplantation, the mean serum creatinine concentration was 134.1+/-73 micromol/l and remained constant during follow-up. Eight patients underwent a second renal biopsy 2 years after transplantation. No significant changes in deposits or renal toxicity due to calcineurin inhibitors were detected. CONCLUSION: Although liver transplantation in FAP does not affect existing renal amyloid deposits, it prevents the progression of renal disease.     

Liver Transplantation for Familial Amyloidotic Polyneuropathy (FAP): A Single-Center Experience Over 16 Years

Orthotopic liver transplantation (LTx) is currently the only available treatment that has been proven to halt the progress of familial amyloidotic polyneuropathy (FAP). The aim of this study was to assess mortality and symptomatic response to LTx for FAP. All 86 FAP patients transplanted at our hospital between April 1990 and November 2005 were included in the study. Five patients underwent retransplantation. The 1-, 3- and 5-year patient survival rates in patients transplanted during 1996-2005 were 94.6%, 92.3% and 92.3%, respectively, a significant difference from the rates of 76.7%, 66.7% and 66.7%, respectively, during 1990-1995 (p = 0.0003). Multivariate analysis revealed that the age at the time of LTx (>or=40 years), duration of the disease (>or=7 years) and modified body mass index (mBMI) (<600) were independent prognostic factors for patient survival. A halt in the progress of symptoms was noted in most patients, but only a minority experienced an improvement after LTx. To optimize the posttransplant prognosis, LTx should be performed in the early stages of the disease, and close post-LTx monitoring of heart function by echocardiography and of heart arrhythmia by Holter ECG is mandatory.     

Liver transplantation for familial amyloidotic polyneuropathy in Australia

Familial amyloidotic polyneuropathy type 1 (FAP-1) is a type of systemic amyloidosis caused by mutant transthyretin (mTTR) that is mainly produced in the liver. Most patients have progressive peripheral and autonomic neuropathy. Ten patients with FAP underwent orthotopic liver transplantation (OLT) at the Queensland Liver Transplant Service (Princess Alexandra Hospital, Brisbane, Australia). Nine patients are still alive, and one patient died of cardiac failure 10 days after OLT. Some symptoms of FAP were alleviated in some of the patients. OLT seems to be a worthwhile treatment for FAP, because it halts the progression of symptoms and achieves improvement in some patients. Received for publication on July 15, 1999; accepted on April 14, 2000     

Characterization of end-stage renal disease after liver transplantation in transthyretin amyloidosis (ATTR V30M)

Transthyretin (TTR) amyloidosis, an autosomal-dominant disease, is characterized by peripheral and autonomic neuropathy—familial amyloidotic polyneuropathy (FAP). End-stage renal disease (ESRD) occurs at 10 years after the onset of neuropathy. Orthotopic liver transplantation (OLT) is the usual treatment of choice. We evaluated FAP patients, ATTR V30M, before and after OLT who started dialysis within 3 months after surgery. The 11 patients had an age at the onset of neuropathy of 31.9 ± 6.3 years with a mean evolution of disease to OLT of 4.54 ± 2.5 years. The glomerular filtration rate was <60 mL/min in 2 patients, 2 displayed nephrotic range proteinuria, and 3 had microalbuminuria. Elective pacemaker implantation was necessary in 8 subjects. Post-OLT 3 patients developed proteinuria, 2 of whom showed increasing nephrotic syndrome. Dysautonomia worsened leading to bladder catheterization in 6. In patients with previous normal renal function and proteinuria <3 g/d, the evolution of neuropathy to the first dialysis was 14.6 ± 4.2 years versus 7.5 ± 1.1 among the other subjects. Overall, dialysis was implemented at 7.4 ± 4.9 years after surgery. There was no liver graft dysfunction. The heart evaluation post-OLT showed the following: 3 patients with de novo dysrhythmias requiring pacemaker implantation and 3 with N-terminal pro-natriuretic peptide levels >10,000 pg/mL. Death occurred in 4 subjects at an average of 26 months after initiation of dialysis. Concerning ESRD, there was no clear benefit of transplantation in the early stages. Patients with normal renal function and lower levels of proteinuria showed slower progression to ESRD, irrespective of their duration of neuropathy.     

Ten years of international experience with liver transplantation for familial amyloidotic polyneuropathy: results from the Familial Amyloidotic Polyneuropathy World Transplant Registry

BACKGROUND: Transthyretin (TTR) amyloidosis is a group of systemic amyloidoses disorders caused by an amyloidogenic TTR variant. Untreated, it slowly leads to severely disabling symptoms that relentlessly progress until the death of the patient. Because the mutant form of TTR is produced mainly in the liver, successful orthotopic liver transplantation (OLT) results in the elimination of the source of the variant TTR molecule and is presently the only known curative treatment. OLT in patients with familial amyloidotic polyneuropathy (FAP) was first performed in 1990 at the Karolinska Institute in Sweden, and because the results were promising other centers took up the procedure. METHODS: To gain as great an experience as possible regarding this treatment, the Familial Amyloidotic Polyneuropathy World Transplant Registry (FAPWTR) was initiated in 1995, and this article presents the 10-year registry results. RESULTS: A total of 54 centers in 16 countries have performed OLT for FAP, and today approximately 60 OLTs are performed annually worldwide. During the last decade, a total of 539 patients have undergone 579 OLTs. Patient survival is excellent (overall 5-year patient survival 77%) and comparable to the survival with OLT performed for other chronic liver disorders, but longer follow-up is needed to compare the outcome after OLT with the natural course of the disease. The main cause of death was cardiac related (39%). CONCLUSIONS: We believe that the FAPWTR has become a valuable tool that will help to accurately evaluate the potential risks and benefits of OLT in patients with FAP and promote a fruitful collaboration between centers engaged in this field.     

Choice of transplantation techniques and indications for liver transplantation in polycystic liver disease in patients with no signs of end-stage liver disease

OBJECTIVE: Since the initiation of the Liver Transplant Program, 500 liver procedures have been performed. Polycystic liver disease (PLD) and polycystic kidney-liver disease (PKLD) have been rare indications for orthotopic liver transplantation (OLT). Only 7 patients (1.4%) underwent transplantation due to PLD and PKLD. MATERIALS AND METHODS: The group consisted of 4 patients who underwent OLT (0.8%) and 3 patients who received simultaneous liver kidney transplantation (LKT; 0.6%). Our objective was to analyze the indications for either OLT or combined LKT as well as indications for surgical techniques during OLT among patients with PLD or PKLD. RESULTS: The main indication for OLT was massive hepatomegaly causing severe physical handicaps, fatigue, and clinically advanced malnutrition. All 3 patients with indications for combined LKT were dialysis-dependent. None of the patients had symptoms of end-stage liver disease and/or hepatic failure. In 4 cases, a portal bypass was applied, and the piggy-back method used in the other 3 cases. The hepatectomy caused no uncommon difficulty. In cases of simultaneous transplantations, the kidney was implanted separately after OLT. All patients are alive following the transplantation; major surgical complications have occurred. CONCLUSIONS: Patients with PLD can undergo OLT safely with good results. They benefit from the relief of abdominal distension and anorexia. Patients with PKLD who are dialysis-dependent should undergo simultaneous LKT. The surgical technique was solely dependent on the intraoperative conditions determined during the dissection phase.     

Liver transplantation for Wilson's disease: long-term results and quality-of-life assessment

BACKGROUND: Wilson's disease associated with severe liver disease is effectively cured by orthotopic liver transplantation (OLT). However, there are also anecdotal reports of improved or resolved neurologic symptoms after OLT in patients with stable or normal liver function. Side effects with conventional chelating agents are common, and it has been suggested that OLT should be considered in patients with severe progressive neurologic symptoms. However, the decision to apply this therapeutic modality to a subgroup of patients without significant liver disease is a quality-of-life issue. METHODS: Long-term follow-up and quality-of-life data were obtained prospectively for 24 patients who underwent OLT between 1988 and 2000 for Wilson's disease associated with severe liver disease. In long-term survivors, quality of life was assessed using the 36-Item Short Form 36 Health Survey Questionnaire. RESULTS: One patient who had multiorgan failure before OLT died within 24 hr of surgery and two patients died within 1 year because of immunosuppressant-related complications. There have been no deaths or graft loss in patients who have undergone transplantation since 1994, and after a median follow-up of 92 months, all survivors have satisfactory graft function (5-year patient and graft survival, 87.5%), with quality-of-life scores (assessed in 86% of survivors) comparable to age- and sex-matched controls from the general population. CONCLUSIONS: The authors' results suggest that liver transplantation can be safely performed in patients with Wilson's disease, with excellent long-term results and quality of life. Further study of the utility of liver transplantation in the management of patients with severe neurologic symptoms is justified.     

Menstrual cycle and sex hormone profile in perimenopausal women after liver transplantation

Excellent long-term outcomes of transplant patients let many female liver-recipients experience perimenopausal problems. This study assessed menstrual patterns and sex hormone profiles in women of perimenopausal age who experienced end-stage liver failure treated by transplantation (OLT). MATERIALS AND METHODS: Menstrual patterns, sex hormone profiles, and biochemical parameters of liver function were analyzed before and after OLT in 13 liver-transplanted patients of perimenopausal age. Nineteen healthy perimenopausal women served as controls. RESULTS: The most common abnormality of the menstrual cycle observed in the study group was secondary amenorrhea, which affected six liver-transplanted women. Three months after OLT amenorrhea was still observed in six patients, regular menstrual cycles in six and irregular bleeding in one graft recipient. One year after transplantation regular menstruations were noted in four, irregular bleeding in four, and secondary amenorrhea in five liver-transplanted women. Similar levels of follicle stimulating hormone, luteinizing hormone, prolactin, progesterone and testosterone as well as lower levels of estradiol and DHEA-sulfate were observed in patients with liver failure, both before and after grafting, compared with healthy women. After OLT E2 levels increased from 32.05 +/- 18.04 to 49.12 +/- 22.21. CONCLUSIONS: One year after OLT disturbances in menstrual patterns affect most (69%) perimenopausal female liver recipients. Both before and after OLT significantly lower levels of estradiol and DHEA-S were observed in transplanted patients compared with healthy controls. Hormonal therapy of amenorrhea or irregular menstruations may be required in that group of patients.     

肺癌开胸术后病人心律失常原因分析及护理

目的探讨肺癌开胸术后发生心律失常的相关因素,为有效护理提供可靠依据.方法对146例肺癌开胸术病人进行术前常规检查,术后心电监护.结果术后发生心律失常62例(42.47%),其中59例(95.16%)发生在术后3d内.术前心电图异常者术后心律失常发生率显著高于正常者(P＜0.01);全肺切除术后心律失常发生率显著高于其它手术方式(均P＜0.05),年龄＞50岁、有吸烟史、有心血管病史者心律失常发生率均显著高于≤50岁、无吸烟史、无心血管病史者(P＜0.05,P＜0.01).术后低血糖及手术前后电解质紊乱者心律失常发生率差异无显著性意义(均P＞0.05).结论术前心电图异常、肺组织切除范围大、＞50岁、有吸烟史、心血管病史等为肺癌开胸术后心律失常的相关因素.术前应注意心肺功能锻炼、改善心肌供血、戒烟;术后以加强心电监护、充分供氧、止痛、排痰为护理重点.     

Combined heart and liver transplantation for familial amyloidotic neuropathy.

Familial amyloidotic polyneuropathy (FAP) is an inherited disease characterized by an abnormal systemic deposition of a mutant protein called transthyretin (TTR) with elective involvement of the peripheral nervous system, but often determining cardiac, gastrointestinal, and urinary tract dysfunction. FAP commonly affects the liver and the heart until end-organs failure. Transthyretin amyloidosis is today an accepted indication for orthotopic liver transplantation (OLT). Combined heart and liver transplantation (CHLT) may be an attractive and rational treatment option when both organs are contemporary involved by this type of amyloidotic disease. Nowadays, surgical indications and techniques are far from being consolidated because only few cases of CHLT have been previously reported in literature. From November 1999 to May 2006, we performed five orthotopic combined heart and liver transplantations for FAP at our institution. Our surgical experience and clinical outcomes are herein reported.     

Post-liver transplant acute renal failure: factors predicting development of end-stage renal disease

BACKGROUND: Acute renal failure (ARF) occurs in 5-50% of patients undergoing orthotopic liver transplantation (OLT). The aim of this study was to determine factors that might predict the development of end stage renal disease (ESRD) in patients who had ARF after OLT. METHODS: We studied all OLT recipients between 9/1/1988 through 12/31/2000. RESULTS: A total of 1602 patients underwent OLT during the study period. About 350 patients (22%) developed ARF requiring dialysis post-operatively. One hundred and twenty-three (39.8%) died within a year after OLT. Median follow up was 5.8 yr (range 1-12 yr). Forty-three patients (23%) developed ESRD over median of 3.79 yr (range 1-8 yr). Multivariate logistic regression analysis revealed creatinine levels > 1.7 mg/dL at 1 yr (p < 0.001), cyclosporine as immunosuppression (p = 0.026), and the presence of diabetes pre-OLT (p < 0.001) to be associated with the development of ESRD. The development of ESRD did not decrease patient survival (p = 0.111). ESRD patients who received subsequent kidney transplantation had significantly improved survival rates (p = 0.005). CONCLUSIONS: Serum creatinine levels at 1 yr, cyclosporine as immunosuppression, and the presence of diabetes pre-OLT are independent predictive factors for the development of ESRD. ESRD patients who received kidney transplantation had higher 10-yr survival rates when compared with patients maintained on dialysis.     

Analysis of Model for End-Stage Liver Disease (MELD) score in a liver transplantation waiting list

BACKGROUND: The Model for End-Stage Liver Disease (MELD) was introduced in 1999 to quantify the 3-month prognosis of cirrhotic patients after a transjugular intrahepatic portosystemic shunt (TIPS). Because of the imbalance between organ donors and patients on the waiting list, the MELD was adopted by the United States in 2002 to allocate liver grafts for transplantation. Preliminary results have indicated a reduction in waiting list deaths and an increase in transplantation rates for candidates. Seeking to find a new model to predict death on the waiting list and after liver transplantation, retrospective studies have examined MELD scores in waiting list patients. The aim of this study was to analyze the MELD scores of patients on the liver waiting list for comparisons between transplanted patients. PATIENTS AND METHODS: A retrospective study was performed analyzing 131 registrations of 127 orthotopic liver transplant (OLT) patients (4 underwent retransplantation) grafted between November 2000 and January 2006, excluding 24 patients: 2 had urgent retransplantations due to hepatic artery thrombosis and 22 had incomplete data. These patients were divided into 3 groups: group I (transplanted patients)-53 patients underwent 55 OLT; group II-29 patients who died on the waiting list; group III-patients on the waiting list including 23 patients still waiting as of the date of the study. RESULTS: The main indication for OLT was hepatitis C virus cirrhosis (50.50%), followed by alcoholic liver cirrhosis (23.30%), cryptogenic cirrhosis (12.60%), autoimmune hepatitis (5.80%), hepatitis B virus cirrhosis (4.85%), and primary biliary cirrhosis (2.91%). Group I: MELD score 15.62 (range, 6-39) on admission to the list, and 18.87 (range, 7-39) at transplantation. The mean waiting time for OLT was 478.39 days (range, 2-1270 days). The 38 patients who survived underwent 39 OLT (1 retransplantation). The MELD score at entrance to the list was 14.62 (range, 7-30) and at transplantation, 17.70 (range, 7-39). The mean time between admission to the list and transplantation was 505.37 days (range, 6-1270 days). The 15 patients who died had received 16 OLT (1 retransplantation). Their MELD scores were 17.80 (range, 6-39) and 21.81 (range, 9-39) at admission to the list and at transplantation, respectively, with a mean time on the waiting list of 417.93 days (range, 2-872 days). Group II: 29 patients died before OLT, at a mean age of 52.60 years (range, 22-67 years). Their MELD score was 19.24 (range, 7-45), and the interval between admission to the waiting list and death was 249.55 days (range, 3-1247 days). Group III: 23 patients still active on the OLT waiting list at the time of study displayed a mean MELD score of 13.65 (range, 6-28) and 354.30 days (range, 2-905 days) waiting until the moment. In conclusion, MELD score at the time of admission to the waiting list was higher among those patients who died either awaiting a liver graft (19.24) or after OLT (17.80) compared with those who survived after OLT (14.60) or are still awaiting OLT (13.65).     

Presence of autoantibody against ATTR Val30 Met after sequential liver transplantation

BACKGROUND: Recently, sequential liver transplantation has been performed with an explanted liver from a patient with familial amyloidotic polyneuropathy (FAP) because of the shortage of donors. However, metabolism of amyloidogenic transthyretin (ATTR), the pathogenic protein of FAP, has not been well studied in patients who have undergone sequential liver transplantation. The purpose of this study was to examine the changes in serum ATTR levels and to investigate the presence of an autoantibody in patients who underwent sequential liver transplantation with an explanted organ from a patient with heterozygotic FAP (FAP ATTR Val30Met). METHODS: This was a case study performed at the Kumamoto University School of Medicine, Kumamoto, Japan, and Kyoto University School of Medicine, Kyoto, Japan. Intervention occurred by sequential liver transplantation with an explanted FAP patient's liver. Levels of normal TTR and ATTR in the two patients who received the transplanted liver were analyzed by means of an enzyme-linked immunosorbent assay (ELISA) and a matrix-assisted laser desorption/time-of-flight mass spectrometry. In addition, the presence of an autoantibody against ATTR Val30Met was evaluated via ELISA using purified ATTR Val30Met from homozygotic FAP patients' sera. RESULTS: After the operation, the variant TTR levels were unexpectedly lower than levels of normal TTR in serum samples from patients with a transplanted liver from the FAP patient. An autoantibody against the variant TTR was detected on day 3 after the operation in the serum of those patients and continued to be present for at least 2 months after the operation. CONCLUSIONS: An autoantibody against the variant TTR may reduce the serum levels of variant TTR. Although the antibody may play a beneficial role in reducing the pathogenic protein, the long-term effect of the antibody must be investigated further.     

Hematologic aspects of liver transplantation for Budd-Chiari syndrome with special reference to myeloproliferative disorders

BACKGROUND: Patients who undergo orthotopic liver transplantation (OLT) for Budd-Chiari syndrome (BCS) traditionally have been anticoagulated with warfarin postoperatively. Because a significant proportion of BCS patients are found to have an underlying myeloproliferative disorder (MPD), antiplatelet therapy may be a more rational treatment strategy for this subgroup. METHODS: All patients who underwent OLT for the diagnosis of BCS at our institution through March 2000 were included in this analysis. Posttransplant therapy consisted of hydroxyurea and aspirin for those with MPDs. Standard anticoagulation or no antithrombotic treatment was given to BCS patients with other causes. Major posttransplantation complications (thrombosis and bleeding) and mortality were determined. RESULTS: Seventeen patients underwent OLT for BCS at our institution. The mean follow-up was 68.4 months. Two of seventeen patients died; one patient died of recurrent thrombosis (124 months after OLT) and the other patient died of acute hepatitis B (7 months after OLT). Twelve patients (71%) had evidence of a MPD. Two of the MPD patients were treated with warfarin before the initiation of hydroxyurea and aspirin therapy. The remaining 10 MPD patients were placed on only hydroxyurea and aspirin after OLT. Anagrelide was used in place of hydroxyurea in two patients because of cytopenias caused by the latter agent. The mean follow-up of this group of 10 patients was 59.9 months. Only one patient experienced recurrent thrombosis, which occurred more than 10 years after the original transplant. There were no major bleeding complications and posttransplant liver biopsies were well tolerated. CONCLUSIONS: Antiplatelet therapy that consists of hydroxyurea and aspirin is a safe and effective alternative to anticoagulation to prevent recurrent thrombosis in MPD patients with BCS after liver transplantation. For patients with a hypercoagulable state corrected by OLT, antithrombotic therapy probably is not required. For those patients with conditions not corrected by OLT or with idiopathic BCS, anticoagulation or other therapy to control the hypercoagulable state should be given.     

Autopsy findings in early and late postoperative death after partial left ventriculectomy

BACKGROUND: Partial left ventriculectomy (PLV) is an alternative to heart transplantation for patients with severe heart failure. However, this procedure is accompanied by high morbidity and mortality. Therefore, we studied the hearts of 12 patients who underwent this procedure to increase our understanding of the causes of bad outcome. METHODS: We analyzed the autopsy hearts of 11 of 16 patients who died after PLV, and one heart from a patient who underwent heart transplantation. RESULTS: Six patients died less than 30 days postoperatively, 4 of cardiogenic shock, 1 of arrhythmia, and 1 of coagulopathy. Five patients died from 36 to 120 days after the procedure, 4 of cardiogenic shock and 1 of arrhythmia. The patient who underwent heart transplantation had a cardiogenic shock 230 days after PLV. Ten hearts weighed more than 500 g and nine had myocardial infarction that extended to the papillary muscles. Four patients had infarction of both papillary muscles and 3 of them had episodes of arrhythmia, suggesting some relation between these events. CONCLUSIONS: We found several important morphologic clues for bad outcome: infarction of both papillary muscles, which may be associated with the development of arrhythmia, and myocardial infarction and pericardial hemorrhage, which may contribute to the outcome of heart failure.     

Lack of endothelial dysfunction in patients under tacrolimus after orthotopic liver transplantation

Abstract: Background: Endothelial dysfunction is a significant cause of vascular and end‐organ damage after solid organ transplantation. The aim of this study was to compare endothelial function in healthy controls and in patients who received tacrolimus for immunosuppression after orthotopic liver transplantation (OLT). Methods: Eight OLT patients and eight age‐ and BMI‐matched healthy subjects were included in the study. Apart from hemodynamic parameters, enzymatic liver function, fasting plasma glucose levels, creatinine, cholesterol, nitric oxide and endothelin‐1 levels were measured. Flow‐mediated dilatation (FMD) in the brachial artery was determined by bi‐mode ultrasound. Results: Systolic and diastolic blood pressure and heart rate were higher in OLT recipients compared with the control group, but remained within normal limits. Blood results did not differ significantly between the groups. Circulating nitric oxide (152.2 ± 29.7 vs. 180.6 ± 40.1 μmol/L) and endothelin‐1 (20.5 ± 1.0 vs. 18.9 ± 1.3 pmol/L) values were similar, and the FMD was normal in both groups (10.29 ± 0.89 vs. 9.86 ± 2.43% in controls and OLT recipients, respectively). There was a significant positive correlation between plasma tacrolimus levels after OLT and FMD (r = 0.72, p < 0.05). Conclusion: As assessed by both laboratory and functional approaches, endothelial function was unaltered in patients taking tacrolimus after OLT. The positive correlation between tacrolimus plasma levels and FMD suggest that tacrolimus might have beneficial effects on endothelial function after OLT.     

Initial clinical results of orthotopic liver transplantation for hepatic alveolar echinococcosis.

We analyzed the features of 7 patients who underwent orthotopic liver transplantation (OLT) from April 2001 to April 2006 for incurable hepatic alveolar echinococcosis (AE) in view of the technical features of the OLT, incidence, and type of complications, as well as patient survival. All 7 patients had biliary diseases that resulted from parasitic involvement of the hilum or a large parasitic lesion that invaded the lobes of the liver due to parasitic involvement of the hepatic veins, which are all typical syndromes of hepatic AE. Five patients had previously undergone surgery for hepatic AE and were at the end stage of liver functional disorder. Four patients' operations were performed under venovenous bypass. The mean duration of surgery was 9.1 hours. After transplantation, one patient experienced biliary leakage but was successfully treated with reoperation. One patient died 21 days later of septicemia caused by pneumonia and multiple organ failure, and another patient died 3 months after OLT from heart failure. The other 5 patients are alive and in good condition after transplantation. Although the transplantation procedure was more difficult than usual in these patients, most achieved prolonged survival and a good quality of life. This study suggests that OLT is feasible for incurable AE of the liver and that this procedure ensures a good clinical outcome.     

减少肝移植术后胆道缺血性损伤的临床路径

目的：针对肝移植术后并发症缺血性胆道损伤（ITBL）,试图建立区分各种导致ITBL的危险因素的临床路径,降低ITBL的发生率。方法：记录随访335例行原位肝移植术（OLT）病例的可能导致胆道缺血的危险因素,包括供肝热缺血时间、冷缺血时间、温缺血时间及供肝脂肪肝情况等。按照冷缺血时间分两组I：TBL组和正常组。比较其他危险因素在两组间的差别。结果：冷缺血时间控制〈8 h,正常组81例,ITBL组2例,热缺血时间差别有统计学意义（P=0.017）;8～12 h,正常组150例I,TBL组25例,胆道温缺血时间差异有统计意义（P=0.033）;〉12 h,正常组57例I,TBL组20例,供肝脂肪肝发生率差异有统计学意义（P〈0.05）。结论：为避免ITBL,冷缺血时间〈8 hI,TBL的发生率很低,只要控制好热缺血时间即可;冷缺血时间8~12 h,尽量将胆道温缺血时间控制在1 h左右;冷缺血时间〉12 h,对于有严重脂肪变的边缘供体可以考虑弃用。