OBJECTIVES: In recent years, the percentage of heart transplantation (HT) with short/medium-term assistance devices has increased. This study aims at analyzing primary graft failure and in-hospital mortality according...OBJECTIVES: In recent years, the percentage of heart transplantation (HT) with short/medium-term assistance devices has increased. This study aims at analyzing primary graft failure and in-hospital mortality according to the type of care. MATERIAL AND METHODS: From January 2013 to December?2017 all patients undergoing urgent HT with circulatory/ventricular assistance?were retrospectively and consecutively recruited. Combined transplants, retransplantations and pediatric transplants were excluded. The sample was divided in 10 groups according to the type of shot/medium term assistance devices. RESULTS: A total of 53 patients were recruited, 79% men, average age 49 ± 13 years. 26 patients (51%) had mechanical ventilation at the time of the HT. Primary graft failure occurred in 20 patients (38.5%), whilst it was more frequent in patients assisted with ECMO (8 patients in group 1 (45%) and 7 in group 2 (54%), p 0.5). 14 deaths (27%) were registered, whereas a higher mortality was observed in the group assisted with ECMO (6 patients in group 1 (34%) and 4 in group 2 (31%), p 0.6). Mortality was 17% in the group of non-urgent Levitronix. CONCLUSIONS: Despite not showing statistical significance due to the low number of patients in some subgroups, hospital?mortality was high in HT patients with circulatory assistance. A trend towards a higher incidence of primary graft failure and mortality in patients assisted with ECMO was observed. The direct implantation of a centrifugal pump as a bridge to urgent transplantation could identify a subgroup with a better prognosis.展开更多
Background: Hereditary transthyretin(ATTRv) amyloidosis is an autosomal dominant disease linked to transthyretin gene mutations which cause instability of the transthyretin tetramer. After dissociation and misfolding ...Background: Hereditary transthyretin(ATTRv) amyloidosis is an autosomal dominant disease linked to transthyretin gene mutations which cause instability of the transthyretin tetramer. After dissociation and misfolding they reassemble as insoluble fibrils(i.e. amyloid). Apart from the common Val30 Met mutation there is a very heterogeneous group of non-Val30 Met mutations. In some cases, the clinical picture is dominated by a rapidly evolving restrictive and hypertrophic cardiomyopathy. Methods: A case series of four liver recipients with the highly clinically relevant, rare and particularly aggressive Val122 del mutation is presented. Medical and surgical therapeutic options, waiting list policy for ATTRv-amyloidosis, including the need for heart transplantation, and status of heart-liver transplantation are discussed. Results: Three patients needed a staged(1 patient) or simultaneous(2 patients) heart-liver transplant due to rapidly progressing cardiac failure and/or neurologic disability. Domino liver transplantation was impossible in two due to fibrotic hepatic transformation caused by cardiomyopathy. After a follow-up ranging from 3.5 to 9.5 years, cardiac(allograft) function was maintained in all patients, but neuropathy progressed in three patients, one of whom died after 80 months. Conclusions: This is the first report in(liver) transplant literature about the rare Val122 del ATTRv mutation. Due to its aggressiveness, symptomatic patients should be prioritized on the liver and, in cases with cardiomyopathy, heart waiting lists in order to avoid the irreversible neurological and cardiac damage that leads to a rapid lethal outcome.展开更多
AIM: To determine the clinical reasons for conversion to everolimus(EVL) and long-term outcomes in heart transplant(HT) recipients.METHODS: A retrospective 12-mo study has been carried out in 14 Spanish centres to ass...AIM: To determine the clinical reasons for conversion to everolimus(EVL) and long-term outcomes in heart transplant(HT) recipients.METHODS: A retrospective 12-mo study has been carried out in 14 Spanish centres to assess the efficacy and safety of conversion to EVL in maintenance HT recipients.RESULTS: Two hundred and twenty-two patients were included(mean age: 53 ± 10.5 years; mean time from HT: 8.1 ± 4.5 years). The most common reasons for conversion were nephrotoxicity(30%), chronic allograft vasculopathy(20%) and neoplasms(17%). The doses and mean levels of EVL at baseline(conversion to EVL) and after one year were 1.3 ± 0.3 and 1.2 ± 0.6 mg/d and 6.4 ± 3.4 and 5.6 ± 2.5 ng/mL, respectively. The percentage of patients receiving calcineurin inhibitors(CNIs) at baseline and on the final visit was 95% and 65%, respectively. The doses and mean levels of CNIs decreased between baseline and month 12 from 142.2 ± 51.6 to 98.0 ± 39.4 mg/d(P < 0.001) and from 126.1 ± 50.9 to 89.2 ± 47.7 ng/mL(P < 0.001), respectively, for cyclosporine, and from 2.9 ± 1.8 to 2.6 ± 1.9 mg/d and from 8.3 ± 4.0 to 6.5 ± 2.7 ng/mL(P = 0.011) for tacrolimus. In the subgroup of patients converted because of nephrotoxicity, creatinine clearance increased from 34.9 ± 10.1 to 40.4 ± 14.4 mL/min(P < 0.001). There were 37 episodes of acute rejection in 24 patients(11%). The most frequent adverse events were oedemas(12%), infections(9%) and gastrointestinal problems(6%). EVL was suspended in 44 patients(20%). Since the database was closed at the end of the study, no further followup data is available.CONCLUSION: Conversion to EVL in maintenance HT recipients allowed minimisation or suspension of the CNIs, with improved kidney function in the patients with nephrotoxicity, after 12 mo.展开更多
文摘OBJECTIVES: In recent years, the percentage of heart transplantation (HT) with short/medium-term assistance devices has increased. This study aims at analyzing primary graft failure and in-hospital mortality according to the type of care. MATERIAL AND METHODS: From January 2013 to December?2017 all patients undergoing urgent HT with circulatory/ventricular assistance?were retrospectively and consecutively recruited. Combined transplants, retransplantations and pediatric transplants were excluded. The sample was divided in 10 groups according to the type of shot/medium term assistance devices. RESULTS: A total of 53 patients were recruited, 79% men, average age 49 ± 13 years. 26 patients (51%) had mechanical ventilation at the time of the HT. Primary graft failure occurred in 20 patients (38.5%), whilst it was more frequent in patients assisted with ECMO (8 patients in group 1 (45%) and 7 in group 2 (54%), p 0.5). 14 deaths (27%) were registered, whereas a higher mortality was observed in the group assisted with ECMO (6 patients in group 1 (34%) and 4 in group 2 (31%), p 0.6). Mortality was 17% in the group of non-urgent Levitronix. CONCLUSIONS: Despite not showing statistical significance due to the low number of patients in some subgroups, hospital?mortality was high in HT patients with circulatory assistance. A trend towards a higher incidence of primary graft failure and mortality in patients assisted with ECMO was observed. The direct implantation of a centrifugal pump as a bridge to urgent transplantation could identify a subgroup with a better prognosis.
文摘Background: Hereditary transthyretin(ATTRv) amyloidosis is an autosomal dominant disease linked to transthyretin gene mutations which cause instability of the transthyretin tetramer. After dissociation and misfolding they reassemble as insoluble fibrils(i.e. amyloid). Apart from the common Val30 Met mutation there is a very heterogeneous group of non-Val30 Met mutations. In some cases, the clinical picture is dominated by a rapidly evolving restrictive and hypertrophic cardiomyopathy. Methods: A case series of four liver recipients with the highly clinically relevant, rare and particularly aggressive Val122 del mutation is presented. Medical and surgical therapeutic options, waiting list policy for ATTRv-amyloidosis, including the need for heart transplantation, and status of heart-liver transplantation are discussed. Results: Three patients needed a staged(1 patient) or simultaneous(2 patients) heart-liver transplant due to rapidly progressing cardiac failure and/or neurologic disability. Domino liver transplantation was impossible in two due to fibrotic hepatic transformation caused by cardiomyopathy. After a follow-up ranging from 3.5 to 9.5 years, cardiac(allograft) function was maintained in all patients, but neuropathy progressed in three patients, one of whom died after 80 months. Conclusions: This is the first report in(liver) transplant literature about the rare Val122 del ATTRv mutation. Due to its aggressiveness, symptomatic patients should be prioritized on the liver and, in cases with cardiomyopathy, heart waiting lists in order to avoid the irreversible neurological and cardiac damage that leads to a rapid lethal outcome.
文摘AIM: To determine the clinical reasons for conversion to everolimus(EVL) and long-term outcomes in heart transplant(HT) recipients.METHODS: A retrospective 12-mo study has been carried out in 14 Spanish centres to assess the efficacy and safety of conversion to EVL in maintenance HT recipients.RESULTS: Two hundred and twenty-two patients were included(mean age: 53 ± 10.5 years; mean time from HT: 8.1 ± 4.5 years). The most common reasons for conversion were nephrotoxicity(30%), chronic allograft vasculopathy(20%) and neoplasms(17%). The doses and mean levels of EVL at baseline(conversion to EVL) and after one year were 1.3 ± 0.3 and 1.2 ± 0.6 mg/d and 6.4 ± 3.4 and 5.6 ± 2.5 ng/mL, respectively. The percentage of patients receiving calcineurin inhibitors(CNIs) at baseline and on the final visit was 95% and 65%, respectively. The doses and mean levels of CNIs decreased between baseline and month 12 from 142.2 ± 51.6 to 98.0 ± 39.4 mg/d(P < 0.001) and from 126.1 ± 50.9 to 89.2 ± 47.7 ng/mL(P < 0.001), respectively, for cyclosporine, and from 2.9 ± 1.8 to 2.6 ± 1.9 mg/d and from 8.3 ± 4.0 to 6.5 ± 2.7 ng/mL(P = 0.011) for tacrolimus. In the subgroup of patients converted because of nephrotoxicity, creatinine clearance increased from 34.9 ± 10.1 to 40.4 ± 14.4 mL/min(P < 0.001). There were 37 episodes of acute rejection in 24 patients(11%). The most frequent adverse events were oedemas(12%), infections(9%) and gastrointestinal problems(6%). EVL was suspended in 44 patients(20%). Since the database was closed at the end of the study, no further followup data is available.CONCLUSION: Conversion to EVL in maintenance HT recipients allowed minimisation or suspension of the CNIs, with improved kidney function in the patients with nephrotoxicity, after 12 mo.