Liberal use of veno-arterial extracorporeal membrane oxygenation in combined sequential heart-liver transplantation.

Combined heart-liver transplantation (CHLT) is a rarely though increasingly performed procedure with evolving indications. Despite CHLT being performed at only a handful of centers, the use of intraoperative mechanical circulatory support to optimize hemodynamics and facilitate dual-organ transplantation varies widely. At our center, we liberally utilize veno-arterial extracorporeal membrane oxygenation (V-A ECMO) when a veno-venous shunt is anticipated to be insufficient in mitigating the hemodynamic perturbations associated with liver reperfusion. In this series, we describe our experience with V-A ECMO in sequential (heart-first) CHLT and demonstrate highly favorable outcomes with this strategy.

Anesthesia management of combined sequential heart-liver transplantation using a caval clamp without venovenous bypass: A case report.

Familial amyloid polyneuropathy, an autosomal-dominant disease due to mutations in the transthyretin gene, often affects the heart and liver, and is treated best with a combined heart-liver transplantation (CHLT). Although it remains an uncommonly performed procedure, the number of patients undergoing CHLT is increasing. Because of the complexity associated with dual pathophysiology, CHLT poses an extraordinary challenge for anesthesia management. Either both heart and liver transplantation are performed on cardiopulmonary bypass (CPB); or heart transplantation is performed on CPB, followed by liver transplantation with venovenous bypass. Recent reports suggested that liver transplantation can be performed without bypass using the inferior vena cava-sparing technique. However, both bypass and caval sparing technique have their own complications. Here, we present the anesthesia management in a case of sequential heart-liver transplantation using a routine caval cross-clamp technique without venovenous bypass. A 48-year-old man complaining of chest tightness, chest pain, and shortness of breath was diagnosed with amyloid cardiomyopathy. Cardiac ultrasonography revealed thickening of ventricular walls and left ventricular systolic insufficiency (ejection fraction decreased from 46% to ∼20% in 6 months), which was refractory to medical therapy. Symptoms occurred repeatedly. Therefore, CHLT was planned. Heart transplantation was performed smoothly under general anesthesia and standard CPB. His heart functioned well with dobutamine and epinephrine infusion. Subsequently, the patient was weaned from CPB. Liver transplantation was planned using the piggyback procedure with the caval sparing technique. However, upon caval clamping, unexpected blood loss occurred. Clamping of the caval was tested followed by cross-clamping. Norepinephrine, epinephrine, and dobutamine were administered. After the hepatic vein was anastomosed, the clamp was released and nitroglycerin was administered. Hemodynamics was stable, and the patient was discharged after 37 days of hospitalization. The case indicates that CHLT could be performed using caval clamp without venovenous bypass in selected patients.

Heterogeneity of liver fibrosis in patients with congestive hepatopathy: A biopsy and explant comparison series.

PURPOSE: Patients with end-stage heart failure and concomitant irreversible liver injury may be candidates for combined heart liver transplant (CHLT). Determining appropriate candidates for CHLT is essential given organ scarcity. Transjugular liver biopsy (TJLB) is used to evaluate the severity of parenchymal liver injury in transplant candidates. In patients with congestive hepatopathy (CH), the fibrosis pattern may be heterogenous. METHODS: We reviewed all CHLT cases between 2007 and 2017, as well as lone-heart transplant cases with post-mortem autopsy. Pre-transplant TJLB was compared to explant to assess the performance of biopsy fibrosis staging. RESULTS: 12 patients were included. Median age at time of transplant was 58 and the cohort was predominantly male (75%). Seven (64%) TJLB were predominantly stage 4 fibrosis and 4 (36%) were stage 1. Advanced fibrosis was the dominant pattern in 7 (70%) explants and 5 (50%) explants had heterogenous fibrosis. In 50% of CH cases, there was discordance between the TJLB and explant. In the autopsy cases, the TJLB and autopsy findings differed. CONCLUSIONS: In this series of matched TJLB and explanted livers, we found variable performance of TJLB in predicting the predominant fibrosis stage present in the liver.

Non-invasive biomarkers of Fontan-associated liver disease.

BACKGROUND & AIMS: Fontan-associated liver disease (FALD) has emerged as an important morbidity following surgical palliation of single ventricle congenital heart disease. In this study, non-invasive biomarkers that may be associated with severity of FALD were explored. METHODS: A retrospective cohort of paediatric patients post-Fontan who underwent liver biopsy at a high volume at a paediatric congenital heart disease centre was reviewed. RESULTS: Among 106 patients, 66% were male and 69% were Hispanic. The mean age was 14.4 ± 3.5 years, and biopsy was performed 10.8 ± 3.6 years post-Fontan. The mean BMI was 20.8 ± 5 kg/m2, with 27.4% meeting obesity criteria. Bridging fibrosis was observed in 35% of patients, and 10.4% of all patients had superimposed steatosis. Bridging fibrosis was associated with lower platelet counts (168.3 ± 58.4 vs. 203.9 ± 65.8 K/µl for congestive hepatic fibrosis score [CHFS] 0-2b, p = 0.009), higher bilirubin (1.7 ± 2.2 vs. 0.9 ± 0.7 mg/dl, p = 0.0090), higher aspartate aminotransferase-to-platelet ratio index [APRI] and fibrosis-4 [FIB-4] scores (APRI: 0.5 ± 0.3 vs. 0.4 ± 0.1, p <0.01 [AUC: 0.69] and FIB-4: 0.6 ± 0.4 vs. 0.4 ± 0.2, p <0.01 [AUC: 0.69]), and worse overall survival (median 2 years follow-up post-biopsy, p = 0.027). Regression modelling of temporal changes in platelet counts before and after biopsy correlated with fibrosis severity (p = 0.005). CONCLUSIONS: In this large, relatively homogeneous adolescent population in terms of age, ethnicity, and Fontan duration, bridging fibrosis was observed in 35% of patients within the first decade post-Fontan. Bridging fibrosis was associated with worse survival. Changes in platelet counts, even years before biopsy, and APRI/FIB-4 scores had modest discriminatory power in identifying patients with advanced fibrosis. Steatosis may represent an additional risk factor for disease progression in obese patients. Further prospective studies are necessary to develop strategies to screen for FALD in the adolescent population. LAY SUMMARY: In this study, the prevalence of Fontan-associated liver disease (FALD) in the young adult population and clinical variables that may be predictive of fibrosis severity or adverse outcomes were explored. Several lab-based, non-invasive markers of bridging fibrosis in FALD were identified, suggesting that these values may be followed as a prognostic biomarker for FALD progression in the adolescent population.

Adult Combined Heart-Liver Transplantation: The United States Experience.

Background: We aimed to review the indications and outcomes of adults undergoing combined heart-liver transplantation (CHLT) in the US using national registry data. Methods: Adult (≥18 years) CHLT recipients in the United Network for Organ Sharing database were included (09/1987-09/2020; era 1 = 1989-2000, era 2 = 2001-2010, era 3 = 2011-2020). Survival analysis was conducted by means of Kaplan-Meier method, log-rank test, and Cox regression. Results: We identified 369 adults receiving CHLT between 12/1989-08/2020. The number of adult CHLT recipients (R2 = 0.75, p < 0.001) and centers performing CHLT (R2 = 0.80, p < 0.001) have increased over the study period. The most common cardiac diagnosis in the first two eras was restrictive/infiltrative cardiomyopathy, while the most common in era 3 was congenital heart disease (p = 0.03). The 1-, 3-, and 5-years patient survival was 86.8, 80.1, and 77.9%, respectively. In multivariable analysis, recipient diabetes [adjusted hazard ratio (aHR) = 2.35, 95% CI: 1.23-4.48], CHLT between 1989-2000 compared with 2011-2020 (aHR = 5.00, 95% CI: 1.13-22.26), and sequential-liver first CHLT compared with sequential-heart first CHLT (aHR = 2.44, 95% CI: 1.15-5.18) were associated with increased risk of mortality. Higher left ventricular ejection fraction was associated with decreased risk of mortality (aHR = 0.96, 95% CI: 0.92-0.99). Conclusion: CHLT is being increasingly performed with evolving indications. Excellent outcomes can be achieved with multidisciplinary patient and donor selection and surgical planning.

The first case of combined heart-liver transplantation in a patient with alveolar echinococcosis.

We report a rare case of liver alveolar echinococcosis with an invasion of the hepaticocaval confluence, inferior vena cava, pericardium, right atrium, atrial septum, and superior vena cava, and its successful treatment by combined heart-liver transplantation.

Indications and outcomes of combined heart-liver transplant: A systematic review and met-analysis.

BACKGROUND: Combined heart-liver transplantation (CHLT) has become a viable option for treating concomitant heart and liver failure. However, data are lacking with respect to long-term outcomes. METHODS: An electronic search was performed to identify all studies on CHLT. Following application of inclusion and exclusion criteria, a total of seven studies consisting of 99 CHLT patients were included from the original 1864 articles. RESULTS: CHLT recipient mean age was 53.0 years (95% CI 48.0-58.0), 67.5% of which (95% CI 56.5-76.9) were male. 65.5% (95% CI 39.0-85.0) of patients developed heart failure due to amyloidosis whereas 21.6% (95% CI 12.3-35.2) developed heart failure due to congenital causes. The most common indication for liver transplant was amyloidosis [65.5% (95% CI 39.0-85.0)] followed by liver failure due to hepatitis C [13.8% (95% CI 2.1-54.4)]. The mean intensive care unit length of stay was 8 days (95% CI 5-11) with a mean length of stay of 24 days (95% CI 17-31). Cardiac allograft rejection within the first year was 24.7% (95% CI 9.5-50.7), including antibody mediated [5% (95% CI 1.7-15.2)] and T-cell mediated rejection [22.7% (95% CI 8.8-47.1)]. Overall survival was 87.5% (95% CI 78.6-93.0) at 1 year and 84.3% (95% CI 75.4-90.5) at 5 years. CONCLUSIONS: CHLT in select patients with coexisting end-stage heart and liver failure appears to offer high survival and low rejection rates.

Sequential Versus Combined Heart-Liver Transplantation in the USA.

INTRODUCTION: Combined heart-liver transplantation (CHLT) has resulted in acceptable survival rates compared to orthotopic liver transplantation (OLT) alone and orthotopic heart transplantation alone. Using the US transplant registry, we compared outcomes following sequential and combined HLT. METHODS: We conducted a retrospective cohort study. De-identified data were obtained from the United Network Organ Sharing Registry. The primary outcome was patient survival from the date of OLT. Secondary outcomes included liver allograft survival and heart allograft survival. RESULTS: The study cohort included 301 CHLT recipients and six sequential heart-liver transplantation (SHLT) recipients. Patient survival after CHLT was 88% at 1 year, 84% at 3 years, and 82% at 5 years compared to 83%, 67%, and 50% in the SHLT group (p = 0.010). Liver allograft survival at 1, 3, and 5 years was 88%,83% and 82%, respectively, in the CHLT group compared to 83% and 67%, and 50%, respectively, in the SHLT group (p = 0.009). After OLT, heart allograft survival at 1, 3, and 5 years was 86%, 79%, and 74% in the CHLT group, respectively, compared to 83%, 67%, and 50% in the SHLT group (p = 0.037). CONCLUSIONS: Despite the limited size of the SHLT cohort, we found that CHLT was superior to SHLT in survival rate and graft survival. The better outcomes noted in CHLT may relate to immunoprotection provided by liver transplantation from the same donor.

Combined heart and liver transplantation: State of knowledge and outlooks.

Various types of liver impairment have been described in patients with end-stage heart failure who are awaiting heart transplantation. The liver impairment may be severe, characterized by a high model for end-stage liver disease (MELD) Score and/or the presence of ascites, both of which are associated with a high risk of failure after single heart transplantation. A liver function assessment is therefore necessary before registration on the heart transplant list, moreover in case of long-developing heart failure, such as with congenital heart disease or in the presence of risk factors for chronic liver disease including excessive alcohol consumption, metabolic syndrome or chronic viral hepatitis B or C. In these instances, screening for cirrhosis with liver biopsy and for hepatocellular carcinoma through imaging must be systematic and when present, the indication for combined heart-liver transplantation must be considered. Its benefits, however, in case of liver failure with a high MELD score or multi-organ failure remains to be demonstrated. An exception in which the liver shows no morphological or functional alteration is with familial amyloid neuropathy, during which moderate to severe heart failure implies surgical treatment consisting of a liver or even heart-liver transplantation. These must be done early and are mainly contraindicated according to the level of neurological damage.

Anesthetic management during the first combined heart-liver transplant performed in Korea: a case report.

Herein, we describe the anesthetic management during the first combined heart-liver transplant (CHLT) performed in Korea. Though CHLT is a rare procedure, accumulating evidence suggests that it is a feasible option for patients with coexisting heart and liver failure. A 45-year-old female patient presented with severe cardiac dysfunction requiring extracorporeal membrane oxygenation (ECMO) support and secondary congestive hepatopathy. The patient underwent consecutive heart and liver transplantation using extracorporeal circulatory devices-heart transplant with cardiopulmonary bypass, and liver transplant with peripheral ECMO. In this case report, we focus on the specific anesthetic considerations for CHLT pertaining to the challenges associated with dual pathophysiology.

Anesthetic management during the first combined heart-liver transplant performed in Korea: a case report / 대한마취과학회지

Herein, we describe the anesthetic management during the first combined heart-liver transplant (CHLT) performed in Korea. Though CHLT is a rare procedure, accumulating evidence suggests that it is a feasible option for patients with coexisting heart and liver failure. A 45-year-old female patient presented with severe cardiac dysfunction requiring extracorporeal membrane oxygenation (ECMO) support and secondary congestive hepatopathy. The patient underwent consecutive heart and liver transplantation using extracorporeal circulatory devices—heart transplant with cardiopulmonary bypass, and liver transplant with peripheral ECMO. In this case report, we focus on the specific anesthetic considerations for CHLT pertaining to the challenges associated with dual pathophysiology.

Fontan-associated liver disease: Implications for heart transplantation.

Chronic liver diseases are associated with multiple complications, including cirrhosis, portal hypertension, ascites, synthetic dysfunction and hepatocellular carcinoma, and these processes are increasingly recognized in post-Fontan patients. Fontan-associated liver disease (FALD) can be defined as abnormalities in liver structure and function that result from the Fontan circulation and are not related to another disease process. FALD arises due to chronic congestion of the liver created by the elevated venous pressure and low cardiac output of the Fontan circulation, which may be superimposed on previous liver injury. Pathology studies have generally shown that FALD worsens as time post-Fontan increases, but the prevalence of FALD is not well defined because the majority of Fontan patients, even those with significant hepatic fibrosis, appear to be asymptomatic and biochemical or functional hepatic abnormalities are usually subtle or absent. Alternate non-invasive investigations, derived from the study of other chronic liver diseases, have been tested in small series of pediatric and adult Fontan patients, but they have been confounded by congestion and do not correlate well with liver biopsy findings. Liver disease can complicate Fontan circulatory failure and may even be significant enough to be considered a contraindication to heart transplantation or require combined heart-liver transplantation. The search for the optimal management strategy continues in the setting of increasing numbers of Fontan patients surviving to adulthood and being referred for heart transplantation. Thus, in this review we attempt to define the scope and significance of FALD and address transplant-related assessment and management of this challenging disorder.

Intensive care on one case of combined heart-liver transplantation / 中华器官移植杂志

Objective To summarize the diagnosis and treatment of one case of combined heart liver transplantation. Methods On November 24, 2011, one case of combined heart-liver transplantation was performed on a patient with Ebstein's anomaly and tricuspid valve replacement after 5 years,complicated with congestive cirrhosis,liver failure dccompensation,preoperative heart failure Ⅲ degree and B grade of liver function Child-Pugh score. The operation was done with the graded cardiopulmonary bypass assisted mode:first creating the vena cava-aortic bypass to complete heart transplantation, second creating the femoral vein-ascending aorta bypass to complete liver transplantation,and third stopping and neutralizing.The aortic cross-clamping time was 54 min and the an hepatic phase was 38 min.The total time of three times of cardiopulmonary bypass was 199 min and the total time-consuming of operation was 517 min. The patient was given basiliximab +methylprednisolone for immune induction therapy, and tacrolimus + mycophenolate mofetil +prednisone solution for anti-rejection. After operation, liver protecting treatment, anti-infection therapy and nutrition support therapy were given.Results The recipient died of multiple organ failure after 78 days.The mechanical ventilation treatment duration for this recipient was 78 days and ECMO adjuvant therapy for postoperative hypoxemia time lasted 63 days.Conclusion The combined heart liver transplantation is an effective measures for treatment of heart and liver failure.