Transplant Center Experience Research Articles

How to apply ex-vivo split liver transplantation safely and feasibly: A three-step approach.

Given the current organ shortage crisis, split liver transplantation (SLT) has emerged as a promising alternative for select end-stage liver disease patients. To introduce an ex-vivo liver graft splitting approach and evaluate its safety and feasibility in SLT. A retrospective analysis was conducted on the liver transplantation data from cases performed at our center between April 1, 2022, and May 31, 2023. The study included 25 SLT cases and 81 whole liver transplantation (WLT) cases. Total ex-vivo liver splitting was employed for SLT graft procurement in three steps. Patient outcomes were determined, including liver function parameters, postoperative complications, and perioperative mortality. Group comparisons for categorical variables were performed using the χ²-test. In the study, postoperative complications in the 25 SLT cases included hepatic artery thrombosis (n = 1) and pulmonary infections (n = 3), with no perioperative mortality. In contrast, among the 81 patients who underwent WLT, complications included perioperative mortality (n = 1), postoperative pulmonary infections (n = 8), abdominal infection (n = 1), hepatic artery thromboses (n = 3), portal vein thrombosis (n = 1), and intra-abdominal bleeding (n = 5). Comparative analysis demonstrated significant differences in alanine aminotransferase (176.0 vs 73.5, P = 0.000) and aspartate aminotransferase (AST) (42.0 vs 29.0, P = 0.004) at 1 wk postoperatively, and in total bilirubin (11.8 vs 20.8, P = 0.003) and AST (41.5 vs 26.0, P = 0.014) at 2 wk postoperatively. However, the overall incidence of complications was comparable between the two groups (P > 0.05). Our findings suggest that the total ex-vivo liver graft splitting technique is a safe and feasible approach, especially under the expertise of an experienced transplant center. The approach developed by our center can serve as a valuable reference for other transplantation centers.

Single Pediatric Transplant Center Experience with Belumosudil As a Potential Treatment for Chronic Graft Vs Host Disease in Adolescents and Young Adults

Single Pediatric Transplant Center Experience with Belumosudil As a Potential Treatment for Chronic Graft Vs Host Disease in Adolescents and Young Adults

Interstitial lung disease as an indication for pediatric lung transplant.

Interstitial lung disease can be an indication for lung transplant at any age, but it is a particularly common indication for lung transplant in infants. Nevertheless, not all interstitial lung diseases will lead to lung transplant in childhood. Genetic testing has aided the identification of these diseases in children. In severely affected patients, however, definitive diagnosis is not always necessary to consider referral to a transplant center. At experienced transplant centers, a multidisciplinary team educates patient families and aids in the transplant evaluation of children with interstitial lung disease. Children who have undergone transplant require lifetime immunosuppression and close surveillance, but can enjoy good quality of life for years following surgery.

Review of pediatric combined heart-liver transplantation: A roadmap to success.

Combined heart-liver transplantation (CHLT) is a promising technique to address end stage organ failure in patients with concomitant heart failure and chronic liver disease. While most experience with CHLT has involved adult patients, the expanding population of children born with univentricular congenital heart disease who underwent the Fontan procedure and develop Fontan-associated liver disease (FALD) has emerged as a growing indication for pediatric CHLT. Currently, CHLT is performed at a select subset of experienced transplant centers, especially in the pediatric population. While technically demanding, CHLT may offer survival benefit when compared to heart transplant alone with decreased rejection of both synchronous allografts and equivalent outcomes with respect to waitlist time and post-operative complications. Limitations in the technique can be attributed to need for an appropriate multidisciplinary care center, challenges with donor organ availability and allocation, and the complexity associated with patient selection and peri-operative management. In this review, we summarize the history of CHLT, discuss patient selection, and highlight key facets of peri-operative care in the pediatric population.

Dual organ transplantation: when heart alone is not enough.

The number of dual organ transplantations (DOT) are steadily increasing over the past few years. This is both a reflection of increasing complexity and advanced disease process in the patients and greater transplant center experience with performing dual organ transplants. Due to lack of standardization of the process, there remains significant center-based variability in patient selection, perioperative and long-term management of these patients. Overall posttransplant outcomes for DOT have been acceptable with some immunological advantages because of partial tolerance offered by the second organ. These achievements should, however, be balanced with the ethical implications of bypassing the patients who are listed for single organ transplantation because of the preferential allocation of organs for DOT. The field of DOT is expanding rapidly, with good long-term outcomes. There is an urgent need for guidelines to standardize the process of patient selection and listing dual organ transplantation.

The importance of training in an experienced transplant center for initiation a liver transplant program

The importance of training in an experienced transplant center for initiation a liver transplant program

(704) Risk Factors for Persistent FEV1Decline Following COVID-19 Infection in Lung Transplant Recipients; a Single Australian Transplant Centre Experience

(704) Risk Factors for Persistent FEV1Decline Following COVID-19 Infection in Lung Transplant Recipients; a Single Australian Transplant Centre Experience

(583) Sotrovimab in Heart Transplant Recipients with Covid-19 in the Omicron Era: A National Transplant Center Experience

(583) Sotrovimab in Heart Transplant Recipients with Covid-19 in the Omicron Era: A National Transplant Center Experience

Liver transplantation: a 10-year low-volume transplant center experience in Kazakhstan

Liver transplantation: a 10-year low-volume transplant center experience in Kazakhstan

333.2: Vascular Complications in Pediatric Liver Transplants And Their Management

Introduction: Unlike other organs, the blood supply of the liver occurs through two systems. One of these is the hepatic artery, and it supply approximately one-third to one-fifth of the liver. The rest of the liver is supplied with blood via the portal vein. The outflow of blood circulation in the liver is via the hepatic veins. Any disruption in this blood circulation results in deterioration in liver functions. In this study, we aimed to evaluate early vascular complications in pediatric liver transplants. Methods: From 8 November 1988 to 31 December 2021, we performed 701 LT procedures and 334 of them were pediatric.). We reviewed the medical records of these recipients for the following: primary cause of liver failure, age, and weight at the time of transplantation, type of graft, vascular complications and their management. One hundred and seventy six of the recipients were male and 158 were female. Mean age of 7.34 years (0.5 months – 17 years). Nineteen (5.7%) of the LT were deceased donor LT and 315 (94.3%) were living related liver transplant. Most cause of liver failure was biliary atresia (n=169). Mean weight of recipients was 23.3 kg. Most of graft types was left lateral graft (n=204) Results: Hepatic vein complications occurred in 3 patients. In all three patients, stenosis was detected in the portal vein anastomosis region and was successfully treated with interventional radiological methods by placing a stent in the anastomosis region. Portal vein complications occurred in 3 patients. In one of these patients, hemostasis was performed by surgical method due to bleeding from the portal vein anastomosis. In the second patient, the anastomosis was surgically revised due to thrombus formation in the portal vein. In the third patient, due to a stenosis of more than 50% in the portal vein anastomosis, a stent was placed in the anastomosis region after balloon dilation using interventional radiological methods, and blood flow was successfully maintained. Hepatic artery complications occurred in 54 patients. Hepatic artery thrombosis occurred in 31 patients, hepatic artery stenosis in 13 patients, bleeding from hepatic artery anastomosis in 7 patients, hepatic artery dissection in 2 patients, and pseudoaneurysm in the hepatic artery in 1 patient. 43 of these patients were successfully treated with interventional radiological methods and 11 of them surgically. Conclusion: Vascular complications seen in liver transplants can cause deterioration in hepatic functions and acute liver failure. Especially hepatic artery complications are one of the most important causes of biliary tract complications that will develop in the future. Vascular complications can be successfully treated at an early stage in experienced organ transplant centers.

413.4: The Beginning of the End for Living Unrelated Donation Kidney Transplantations in the Middle East: The Shiraz Transplant Center Experience

Background: The Middle East is a region with much controversy and limitations regarding deceased organ transplantations. In this report we describe our unique experience with the establishment of our deceased donor organ donation program for Central and Southern Iran. Methods: During the initial phases in the 70s and 80s, only living related kidney transplantations were allowed in Iran. However, this policy was not continued and was expanded due to severe shortage of hemodialysis facilities and an urgent need to increase the number of kidney transplantations. Accordingly, some centers in Tehran started unrelated kidney transplantation with the support of the government that offered bonuses to persuade unrelated donors. It should be mentioned that, during this time, permission for deceased organ donation was not yet obtained in Iran. The main focus of the Shiraz Transplant Center was to develop a deceased donation program from the beginning. During three decades this center was able to expand its deceased donation program to a degree that in 2008 it completely replaced all its unrelated kidney donations by deceased donations. Prior to 2008, most transplantations were related living and deceased donations, and only in cases where a patient was on the wait list for more than one year and a compatible donor was not found, they were only allowed to have living unrelated donations. The deceased program in the STC has since expanded and has aimed to stop unrelated living donations to achieve some goals which include: to abide by ethical guidelines in organ donations/transplantation such as the declaration of Istanbul, and to finally change the dominant practice of unrelated living donations within the Middle East. These efforts have resulted in an increase of deceased donations in Iran from 1.6 in 2004 to 14.34 per million population in 2019, and in some areas this has reached to as high as 50. Results: Up to March 2021, in the STC, overall 5389 kidney transplantations have been performed. Figure 1 shows the trend in kidney transplantation in the STC. Conclusion: Living unrelated donations are still an ongoing issue in the Middle East region. Our center, has implemented a wide deceased donor program and as a result has been able to completely stop unrelated living kidney transplantations. The deceased donor organ donation program should further be expanded within Iran and other countries in the region and continuous campaigns should be held up against any type of unethical issues such as living unrelated transplantations to meet with international standards.

S239: COMPARABLE LONG-TERM OUTCOMES BETWEEN UPFRONT HAPLOIDENTICAL AND IDENTICAL SIBLING DONOR HSCT IN APLASTIC ANEMIA: A NATIONAL REGISTRY-BASED STUDY

Background: Allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains a curative option for severe aplastic anemia (SAA), and transplantation from identical sibling donors (ISDs) has been recommended as a first-line treatment. Aims: Haploidentical donor (HID) transplantation for SAA has made great advances; thus, an increased role of HID-SCT in SAA should be considered if an ISD is unavailable. Methods: We performed a national registry-based analysis comparing long-term outcomes in the upfront HID or upfront ISD SCT setting. Results: A total of 342 SAA patients were enrolled, with 183 patients receiving HID SCT and 159 receiving ISD SCT. The estimated 9-year overall survival (OS) and failure-free survival (FFS) were 87.1±2.5% and 89.3±3.7% (P = 0.173) and 86.5±2.6% vs. 88.1±3.8% (P = 0.257) for patients in the HID and ISD SCT groups, respectively. Transplantation from HID or ISD SCT has greatly improved quality of life (QoL) levels post-HSCT compared to pre-HSCT. The occurrence of chronic graft versus host disease was the only identified adverse factor affecting each subscale of QoL. Physical and mental component summaries in adults as well as physical, mental, social, and role well-being in children were all similar between HID and ISD SCT at 5-year points. At the last follow-up, the proportion of returning to society was comparable between the HID and ISD groups, showing 78.0% versus 84.6% among children and 74.6% versus 81.2% among adults. A total of 342 SAA patients were enrolled, with 183 patients receiving HID SCT and 159 receiving ISD SCT. The estimated 9-year overall survival (OS) and failure-free survival (FFS) were 87.1±2.5% and 89.3±3.7% (P = 0.173) and 86.5±2.6% vs. 88.1±3.8% (P = 0.257) for patients in the HID and ISD SCT groups, respectively. Transplantation from HID or ISD SCT has greatly improved quality of life (QoL) levels post-HSCT compared to pre-HSCT. The occurrence of chronic graft versus host disease was the only identified adverse factor affecting each subscale of QoL. Physical and mental component summaries in adults as well as physical, mental, social, and role well-being in children were all similar between HID and ISD SCT at 5-year points. At the last follow-up, the proportion of returning to society was comparable between the HID and ISD groups, showing 78.0% versus 84.6% among children and 74.6% versus 81.2% among adults. Image:Summary/Conclusion: These data suggest the recommendation for upfront transplantation with a haploidentical donor for SAA patients in the absence of an ISD, especially in experienced transplant centers.

Our Pediatric Liver and Kidney Transplant Activities in 2021.

In children with end-stage renal disease, chronic liver failure, or acute liver failure, liver transplant and kidney transplant are the most effective modalities for better clinical outcomes compared with other therapies. However, children are particularly susceptible to surgical complications, so pediatric solid-organ transplants should be reserved for centers with substantial experience and multidisciplinary expertise. Here, we assessed liver and kidney transplants performed at our center in 2021. From November 3, 1975, to December 31, 2021, we performed 701 liver transplants and 3290 kidney transplants. From January 1, 2021, to December 31, 2021, we performed 21 liver transplants (19 in children) and 114 kidney transplants (12 in children). We recorded age, sex, body mass index, comorbidities, etiologies, laboratory values, and clinical outcomes. For the year 2021, we performed 19 pediatric liver transplants and 12 pediatric kidney transplants. Mean age of liver recipients was 3.4 years, and 8 were male patients. The most common etiology was biliary atresia (n = 7). All liver grafts were from living related donors who were first-degree (n = 16) or second- degree (n = 3) relatives of the recipients. Mean hospital stay was 17.6 days. All but 2 liver transplant recipients were discharged successfully (2 died from sepsis in the early postoperative period). Mean age of kidney transplant recipients was 14.1 years, and 4 were male patients. The most common etiology was vesicoureteral reflux (n = 3). One kidney graft was from a deceased donor, with the rest from living related donors who were first-degree relatives of the recipients (n = 11; mother for 8 recipients and father for 3 recipients). Mean hospital stay was 4.3 days. All kidney transplant recipients were discharged successfully. Solid-organ transplants for young children are often complex but can be performed successfully at experienced transplant centers.

Kidney Transplantation in Patients After Bariatric Surgery: High-Volume Bariatric and Transplant Center Experience

Kidney transplantation (KTx) is the best type of treatment for patients with end-stage renal disease (ESRD). Unfortunately, obesity may be a contraindication for transplantation. Our study aimed to evaluate the results of KTx in patients who had bariatric surgery (BS) prior to transplantation. A single center, with experience in bariatric and transplant surgery, presents a retrospective study of 13 patients who received a kidney transplant after a gastric bypass (GB) operation between 2012 and 2019. Thirteen patients, who were potential candidates for KTx, were previously qualified for BS because of a body mass index (BMI) > 35 kg/m2. Additionally, all patients had arterial hypertension, 60% of patients had diabetes, and 30% of patients had coronary artery disease. Patients were activated on the waiting list when their BMI was < 35 kg/m2. KTx was performed between 5 and 29 months after BS. One patient needed reoperation due to a urinary leak and another patient needed reoperation because of a high-pressure lymphocele. We diagnosed 2 delayed graft functions (DGFs) and 1 acute rejection. One patient died for reasons independent of surgery. The KTx observation period ranged from 3 to 8 years. Currently, 11 patients has stable renal function: creatinine concentration is 0.8-1.8 mg/dL and BMI is between 23 and 35 kg/m2. Despite the small group of patients, we can assume that kidney transplantation can be safely performed in patients with end-stage renal disease (ESRD) who have previously undergone gastric bypass (GB) as a graft bridging procedure. In some cases, BS may be the only chance of getting an organ.

Vascular Complications in Pediatric Liver Transplants and Their Management.

The blood supply of the liver occurs through the hepatic artery and portal vein. Outflow of blood circulation in the liver is via the hepatic veins. Any disruption in this blood circulation results in deterioration of liver functions. In this study, we evaluated early vascular complications in pediatric liver transplant recipients seen at our center. From November 1988 to December 2021, our team has performed 701 liver transplant procedures, which have included 334 pediatric liver transplants. Among these pediatric liver transplant recipients (mean age of 7.34 y), 176 were male patients. Nineteen patients (5.7%) were recipients of deceased donors. Reason for liver failure was mainly biliary atresia (n = 169). Mean weight of recipients was 23.3 kg. Most graft types were left lateral grafts (n = 204). Three patients had hepatic vein complications. All 3 patients were successfully treated with interventional radiological methods by placing a stent in the anastomosis region. Portal vein complications occurred in 3 patients. One patient had hemostasis performed surgically. The second patient had surgically revision of the anastomosis because of thrombus formation. Because of stenosis of more than 50% in the portal vein anastomosis, the third patient required stent placement in the anastomosis region. Hepatic artery complications occurred in 54 patients: 31 patients had hepatic artery thrombosis, 13 patients had hepatic artery stenosis, 7 patients had bleeding from hepatic artery anastomosis, 2 patients had hepatic artery dissection, and 1 patient had pseudoaneurysm in the hepatic artery. Forty-three of these patients were successfully treated with interventional radiological methods and 11 required surgical treatment. Vascular complications after liver transplant can cause deterioration in hepatic functions and acute liver failure. Vascular complications can be successfully treated in experienced organ transplant centers.

The role of extracorporeal membrane oxygenation as salvage therapy in adult liver transplant recipients: A high-volume transplantation center experience

The role of extracorporeal membrane oxygenation as salvage therapy in adult liver transplant recipients: A high-volume transplantation center experience

Impact of Center Experience with Donor Type on Outcomes: A Secondary Analysis, Blood and Marrow Transplant Clinical Trials Network 1101

We previously reported the results of Blood and Marrow Transplant Clinical Trials Network (BMT CTN) 1101, a randomized comparison of hematopoietic cell transplantation (HCT) performed with double umbilical cord blood units (dUCB) or with haploidentical bone marrow (haplo-BMT) with post-transplantation cyclophosphamide (PTCy) in the nonmyeloablative setting. Those results showed similar progression-free survival in the 2 treatment groups but lower nonrelapse mortality and better overall survival in the haplo-BM arm. In this secondary analysis, we sought to investigate whether transplantation center's previous experience with haplo-BM and/or dUCB HCT had an impact on outcomes. All patients randomized in BMT CTN 1101 were included. Center experience was assigned based on the number of transplantations with each platform performed in the year before initiation of the study according to the Center for International Blood and Marrow Transplant Research. Centers were then classified as a dUCB center (>10 dUCB HCTs; n=117 patients, 10 centers), a haplo-BM center (>10 haplo-BM HCTs and ≤10 dUCB HCTs; n=110 patients, 2 centers), or other center (≤10 haplo and ≤10 dUCB HCTs; n=140 patients, 21 centers). After adjusting for age, Karnofsky Performance Status, and Disease Risk Index, we found that haplo-BM centers had lower overall mortality with this donor type compared with dUCB centers (hazard ratio [HR], 2.56; 95% confidence interval [CI], 1.44 to 4.56). In contrast, there were no differences in overall mortality between haplo-BM and dUCB in centers that were experienced with dUCB HCT (HR, 1.02; 95% CI, .59 to 1.79) or had limited to no experience with either dUCB or haplo-BM HCT (HR, 1.36; 95% CI, .83 to 2.21). The higher risk of treatment failure and overall mortality in dUCB HCT in haplo BM-experienced centers was driven by a significantly higher risk of relapse (HR, 1.78; 95% CI, 1.07 to 2.97). With the exception of worse outcomes among dUCB HCT recipients in haplo-BM centers, transplantation center experience in the year before initiation of BMT CTN 1101 had a limited impact on the outcomes of this randomized clinical trial.

CFTR Modulators and Lung Transplantation: Factors to Consider - A UK Transplant Centre Experience

<h3>Purpose</h3> Cystic fibrosis transmembrane conductance regulator (CFTR) modulators are drugs that act on the defective CFTR protein in people with cystic fibrosis (pwCF), resulting in improved CFTR protein function and thus improved CF symptoms. Evidence is increasing of their positive impact on extra-pulmonary features of CF, which remains problematic in pwCF post-lung transplantation (LTx). Elexacaftor/tezacaftor/ivacaftor (ele/tez/iva) was licensed in the UK in August 2020. It has been debated since if ele/tez/iva has a role post-LTx for ongoing non-pulmonary CF symptoms. We aim to highlight the practical issues faced when considering this drug in the post-LTx CF population in a single large UK LTx unit. <h3>Methods</h3> Retrospective analysis of electronic records of pwCF post-LTx with ongoing extra-pulmonary manifestations considered for initiation of ele/tez/iva August 2020-2021, including demographics, potential benefits, contraindications and outcomes. <h3>Results</h3> 5 pwCF with LTx have been discussed with their CF physicians to consider initiating ele/tez/iva (some had more than one problem):- sinus (n = 2); bowels (n = 2); weight (n = 2); other (n = 2). All were at least 12 months post-LTx (median 42 months; range 21-191 months). Discussions were initiated by the patient in 1 case, the CF team in 2, and transplant team in 2. 3 were not initiated - 1 due to potential side effects on mental health and 2 were contraindicated with deranged liver tests and ongoing bowel symptoms. 2 were initiated on ele/tez/iva. Of these, one is on tacrolimus, needed no immunosuppression adjustment, but stopped due to drop in FEV1 (CT and biopsy negative for features of rejection) which recovered. The other is on sirolimus, needed significant reduction in immunosuppression (9mg to 4mg) and remains on ele/tez/iva. <h3>Conclusion</h3> Extra-pulmonary manifestations of CF are varied and continue post-LTx. With increasing evidence supporting using CFTR modulators to manage extra-pulmonary symptoms, it is important to establish the role these drugs can have in the post-LTx CF population, but also the potential risks. Our experience so far shows mixed results and that discussion between CF and transplant physicians, with patient involvement is vital to ensure correct decisions are taken. Randomised controlled trials are needed to ensure modulator use in pwCF post-LTx is supported by robust evidence.

Heart Transplantation Strategies In Arrhythmogenic Right Ventricular Cardiomyopathy: The Johns Hopkins Experience

<h3>Background</h3> End stage heart failure necessitating evaluation for heart transplantation is increasingly recognized in arrhythmogenic right ventricular cardiomyopathy (ARVC). These patients present unique challenges in pre- and peri-transplant management given predominantly right ventricle (RV) failure and propensity for ventricular arrhythmias. We utilized a tertiary ARVC referral and heart transplant center experience to describe management of patients with ARVC undergoing heart transplantation. <h3>Methods</h3> We queried the Johns Hopkins ARVC Registry for all patients who underwent heart transplantation and studied the subset undergoing transplantation at Johns Hopkins. Patient demographics, clinical characteristics, and pre-transplant clinical course were obtained from the registry and medical records. <h3>Results</h3> Of the 532 patients in the ARVC Registry, 63 underwent heart transplantation. Nine (3 female) patients had known ARVC prior to transplant and were transplanted at Johns Hopkins Hospital from 2006 to 2020; 5 occurred after the 2018 allocation system revision. Plakophilin-2 (<i>PKP2</i>) pathogenic or likely pathogenic variant was present in 6 (67%) patients; the others were gene elusive. Six patients had a history of catheter ablation for ventricular arrhythmia (5 had at least 3 ablations). All 9 patients had significant right ventricular failure as demonstrated in <b>Figure</b>. Transplant evaluation occurred at a mean age of 42±14 years and was typically initiated within 1 year of developing heart failure. Initial waitlist status was 2 (old system) or 6 (revised system) in 7 (78%) patients; 1 patient underwent transplant at this status, the remaining required upgraded status because of clinical worsening. Bridge to transplant support included inotropes (n=3) and ECMO (n=2). Percutaneous RV mechanical support was unsuccessful in 2 patients due to severe RV dilation. Contraindication to inotropes or left ventricle mechanical support was common due to ventricular arrhythmia and RV predominant cardiomyopathy. <h3>Conclusion</h3> Heart transplantation is a curative treatment for ARVC, but due to frequent ventricular arrhythmia and RV predominant pathology, patients require unique considerations in regards to timing of evaluation, hemodynamic support options and wait listing qualification.

Heart transplantation strategies in arrhythmogenic right ventricular cardiomyopathy: a tertiary ARVC centre experience.

AimsEnd‐stage heart failure necessitating evaluation for heart transplantation is increasingly recognized in arrhythmogenic right ventricular cardiomyopathy (ARVC). These patients present unique challenges in pre‐transplant and peri‐transplant management given their predominantly right ventricular (RV) failure and propensity for ventricular arrhythmias. We sought to utilize a tertiary ARVC referral and heart transplant centre experience to describe management of a series of patients with ARVC undergoing heart transplantation at our centre.Methods and resultsWe queried the Johns Hopkins ARVC Registry for all patients who underwent heart transplantation and further studied the subset undergoing transplantation at the Johns Hopkins Hospital. Patient demographics, clinical characteristics, and pre‐transplant clinical course were obtained from the registry and electronic medical records. Of the 532 patients in the ARVC Registry, 63 (12%) underwent heart transplantation. Nine (six male) of these patients both had known ARVC prior to transplant and were transplanted at Johns Hopkins Hospital between 2006 and 2020 at a mean age of 42 ± 14 years old. Pathogenic ARVC genetic variants were identified in six (67%) patients, all of whom had variants in the plakophilin‐2 (PKP2) gene. RV failure was universal with median right atrial to pulmonary capillary wedge pressure (RA/PCWP) ratio of 1.4 [interquartile range (IQR) 1.2–1.5] and median right ventricular stroke work index (RVSWI) of 0 g·m/m2/beat (IQR 0–0.3). Six had a history of catheter ablation for ventricular arrhythmia with five of the six having at least three ablations. Transplant evaluation was initiated an average of 344 ± 407 days after first developing heart failure symptoms. The most common bridge to transplant support included inotropes (n = 3) and extracorporeal membrane oxygenation (ECMO) (n = 2). Contraindication to inotropes or mechanical support was common due to ventricular arrhythmia and RV predominant cardiomyopathy.ConclusionsHeart transplantation is a curative treatment for ARVC, but due to frequent ventricular arrhythmias and RV predominant pathology, patients require unique considerations in regard to timing of evaluation, haemodynamic support options, and wait listing qualification.