JHLT Open最新文献

{"title":"First US pediatric heart transplant using XVIVO Heart Assist Transport","authors":"Smith Ngeve BA ,&nbsp;John A. Kucera MD ,&nbsp;Seth E.M. Wolf MD ,&nbsp;Berk Aykut MD ,&nbsp;Tariq M. Omer MS ,&nbsp;Alejandro Murillo-Berlioz MD ,&nbsp;Sarah Casalinova BS ,&nbsp;Douglas M. Overbey MD, MPH ,&nbsp;Joseph W. Turek MD, PhD, MBA","doi":"10.1016/j.jhlto.2025.100381","DOIUrl":"10.1016/j.jhlto.2025.100381","url":null,"abstract":"<div><div>We report the first US pediatric heart transplant utilizing the XVIVO Heart Assist Transport (XHAT) system for donor heart preservation. An 11-year-old recipient successfully received a heart from a 27-year-old donor using hypothermic ex vivo perfusion. The transplant was uncomplicated, with excellent 6-month outcomes. This case highlights the potential of XHAT to expand donor access and improve preservation in pediatric heart transplantation.</div></div>","PeriodicalId":100741,"journal":{"name":"JHLT Open","volume":"10 ","pages":"Article 100381"},"PeriodicalIF":0.0,"publicationDate":"2025-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145026522","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Geographic disparity beyond the physical distance: Heart transplant outcomes in patients living in states without a transplant program","authors":"Toyokazu Endo MD ,&nbsp;Joshua Crane MD ,&nbsp;Isabelle Lytle ,&nbsp;Jaimin Trivedi MD, MPH ,&nbsp;Michele Gallo MD ,&nbsp;Siddharth Pahwa MD ,&nbsp;Mark S. Slaughter MD ,&nbsp;Erin M. Schumer MD, MPH","doi":"10.1016/j.jhlto.2025.100365","DOIUrl":"10.1016/j.jhlto.2025.100365","url":null,"abstract":"<div><h3>Background</h3><div>In the United States, outcomes of adult heart transplant are not well studied in those living in states without an active transplant program.</div></div><div><h3>Methods</h3><div>Adult heart transplant patients were identified using the United Network of Organ Sharing database (2014-2023). Two groups were formed: out-of-state (OOS) for those in states without a program and in-state (IS) for those with a program. The primary outcome is post-transplant survival. Secondary outcomes examine listing characteristics and patterns using the Center for Disease Control WONDER database.</div></div><div><h3>Results</h3><div>The OOS group (14 states) had 1,561 patients, with Nevada having the highest proportion. Fewer non-White individuals and those with government-sponsored insurance programs were in the OOS group (<em>p</em> &lt; 0.05). Additionally, more patients in the OOS moved out of their primary state residence at the time of transplant (9.3% vs 2%, <em>p</em> &lt; 0.01). Most patients traveled to high-volume centers in neighboring states to be listed. There was no difference in waitlist outcome (<em>p</em> = 0.13), but post-transplant survival was slightly higher in the OOS group (<em>p</em> = 0.04). Fewer patients in the OOS group were listed relative to their state population and the heart failure mortality cohort compared to those in the IS group (<em>p</em> &lt; 0.01).</div></div><div><h3>Conclusions</h3><div>Overall, the outcomes for individuals living in states without a transplant program did not differ compared to those in states with a program. However, variations in listing characteristics and patterns suggest a potential geographical disparity. Policy changes are crucial to address these inequalities and improve access to heart transplants in states that lack a transplant program.</div></div>","PeriodicalId":100741,"journal":{"name":"JHLT Open","volume":"10 ","pages":"Article 100365"},"PeriodicalIF":0.0,"publicationDate":"2025-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144925574","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of COVID-19 after lung transplantation: A retrospective multicenter comparison of clinical outcomes in Denmark and Sweden","authors":"Embla Bodén ,&nbsp;Michael Perch ,&nbsp;Regitze Hertz Liebermann ,&nbsp;John Mackay Søfteland ,&nbsp;Jesper M. Magnusson ,&nbsp;Sandra Lindstedt","doi":"10.1016/j.jhlto.2025.100377","DOIUrl":"10.1016/j.jhlto.2025.100377","url":null,"abstract":"<div><h3>Background</h3><div>The coronavirus disease-2019 (COVID-19) pandemic posed pronounced challenges in the care of lung transplant (LTx) recipients. Global variations in containment strategies and the introduction of messenger ribonucleic acid (mRNA) vaccines have sparked extensive debate in both scientific and public arenas.</div></div><div><h3>Methods</h3><div>This retrospective study compared outcomes among LTx recipients in Denmark, which implemented a more restrictive COVID-19 containment strategy, and Sweden, which adopted a less restrictive approach. A total of 318 LTx recipients with at least 1 episode of polymerase chain reaction (PCR)-confirmed COVID-19 were included. Propensity score weighting was applied to balance covariates, and survival outcomes were analyzed using weighted Cox proportional hazards and Kaplan-Meier analyses.</div></div><div><h3>Results</h3><div>No significant differences in mortality or risk of chronic lung allograft dysfunction (CLAD) were found between countries (hazard ratios [HR] for death, Sweden = 1.49, 95% confidence intervals [CI]: 0.68-3.26, <em>p</em> = 0.314; HR for CLAD, Sweden = 0.63, 95% CI: 0.32-1.25, <em>p</em> = 0.187). Unvaccinated patients had a significantly higher risk of death compared to vaccinated patients (HR = 3.49, 95% CI: 1.46-8.34, <em>p</em> = 0.005), and infections with the original Wuhan strain carried a higher risk than Omicron (HR = 3.59, 95% CI: 1.53-8.44, <em>p</em> = 0.003). CLAD development or progression was not significantly associated with any subgroup.</div></div><div><h3>Conclusions</h3><div>Despite differences in timing of infections and case load between Sweden and Denmark, clinical outcomes among infected LTx recipients were comparable. mRNA vaccination was strongly associated with improved survival. The results of the current study highlight the importance of continued vaccination efforts and tailored containment strategies in vulnerable populations.</div></div>","PeriodicalId":100741,"journal":{"name":"JHLT Open","volume":"10 ","pages":"Article 100377"},"PeriodicalIF":0.0,"publicationDate":"2025-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144988635","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Transition from pediatric to adult care in thoracic transplantation—Challenges and opportunities","authors":"Nicole Gaffney MBBS, MPH ,&nbsp;Melissa K. Cousino PhD ,&nbsp;Miranda A. Paraskeva MBBS, MPH","doi":"10.1016/j.jhlto.2025.100375","DOIUrl":"10.1016/j.jhlto.2025.100375","url":null,"abstract":"<div><h3>Background</h3><div>Advances in medical care and improved transplant outcomes have led to a increasing number of adolescent and young adult solid organ transplant recipients entering adult-orientated services. Adolescence is a period associated with rapid physical, cognitive and psychosocial change. For thoracic transplant recipients it is a period associated with higher rates of acute and chronic rejecyion and mortality. These factors make transition and transfer particularly high-risk periods.</div></div><div><h3>Methods</h3><div>This paper explores the challenges encountered in the transition and transfer of heart and lung transplant recipients from pediatric to adult care. Examining the structural, psychosocial, developmental and systemic barriers that influence outcomes and appraises best-practice statements and emerging models of care to identify actionable opportunities.</div></div><div><h3>Results</h3><div>Key barriers include fragmented service structure, gaps in continuity, developmental vulnerabilities (executive functioning, autonomy, risk-taking) and system-level constraints (policies, funding, access). Evidence and consensus highligh the importance of a planned, graduated, patient-centered transition process. Opportunities to improve outcomes included dedicated transition programs, intentional pediatric-adult interdisciplinary collaboration, provision of targeted health information and structured self-management skills training for adolescents and young adults and their carers.</div></div><div><h3>Conclusions</h3><div>Successful transition and management of adolescents and young adults with a heart and/or lung transplant requires coordinated efforts among pediatric and adult providers, patients, their caregivers and health care systems. Implementing structured, youth-appropriate, patient-centred transition pathways, grounded in collaboration, continuity of care and skills building, offers a clear route to improving long-term outcomes for this vulnerable population.</div></div>","PeriodicalId":100741,"journal":{"name":"JHLT Open","volume":"10 ","pages":"Article 100375"},"PeriodicalIF":0.0,"publicationDate":"2025-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145026524","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Intraoperative antithrombotic drug removal during heart transplantation: A case series from the International Safe and Timely Antithrombotic Removal (STAR) registry","authors":"Jan Schmitto MD, PhD ,&nbsp;Filip De Somer PhD ,&nbsp;Matthias Thielmann MD, PhD ,&nbsp;Nandor Marczin MD ,&nbsp;Arjang Ruhparwar MD, PhD ,&nbsp;Anna L. Meyer MD, PhD ,&nbsp;Christian Hagl MD, PhD ,&nbsp;Marijana Matejic-Spasic MD ,&nbsp;Daniel Wendt MD, PhD, MHBA ,&nbsp;Weihong Fan ,&nbsp;Efthymios N. Deliargyris MD ,&nbsp;Robert F. Storey MD, DM ,&nbsp;Michael Schmoeckel MD, PhD","doi":"10.1016/j.jhlto.2025.100369","DOIUrl":"10.1016/j.jhlto.2025.100369","url":null,"abstract":"<div><h3>Background</h3><div>Patients on heart transplant waiting lists are often on antithrombotic (AT) drugs. Emergency orthotopic heart transplantation (OHT) when performed on such patients without optimal washout periods increases the risk of severe perioperative bleeding. Intraoperative AT removal by hemoadsorption may mitigate excess bleeding risks.</div></div><div><h3>Methods</h3><div>The international Safe and Timely Antithrombotic Removal (STAR) registry captures real-world outcomes (ClinicalTrials.gov# NCT05077124). Included patients were on ticagrelor or direct-acting oral anticoagulants (DOACs) undergoing emergent OHT. Hemoadsorption was integrated into the cardiopulmonary bypass (CPB) circuit. Bleeding was assessed with the universal definition of perioperative bleeding (UDPB) and volume of chest tube drainage (CTD).</div></div><div><h3>Results</h3><div>Seven patients were included (3 ticagrelor, 2 apixaban, 2 dabigatran; mean age 39.1 ± 11.1 years; 4 females). Mean time from the last AT dose to surgery was 29.4 ± 13.4 hours. Mean CPB duration was 206.0 ± 56.9 minutes with a mean device flow of 340 ± 126 ml. There were no massive bleeding events (UDPB 4), surgical revisions to control bleeding, or deaths within 30 days. Severe bleeding (UDPB 3) occurred in 1/7 (14.3%). Mean 12-hour and 24-hour CTD were 385.7 ± 263.4 m and 586.1 ± 315.0 ml, respectively. No device-related adverse events were reported.</div></div><div><h3>Conclusions</h3><div>This case series from the ongoing STAR registry shows that intraoperative AT removal is simple and potentially effective in minimizing serious perioperative bleeding in patients on ticagrelor or DOACs undergoing OHT. Prospective, controlled studies in larger cohorts are needed to validate these promising observations.</div></div>","PeriodicalId":100741,"journal":{"name":"JHLT Open","volume":"10 ","pages":"Article 100369"},"PeriodicalIF":0.0,"publicationDate":"2025-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144916488","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Donor-derived infections in heart and lung transplant recipients","authors":"Bradley J. Gardiner ,&nbsp;Michael G. Ison","doi":"10.1016/j.jhlto.2025.100376","DOIUrl":"10.1016/j.jhlto.2025.100376","url":null,"abstract":"<div><div>Heart and lung transplantation are life-saving treatments for patients with end-stage organ disease. Donor-derived infections are common and can be expected or unexpected. The lung is exposed to the external environment and transplanted with an intact microbiome, which can include community and nosocomial bacterial, viral, and fungal pathogens. This includes not only well-recognized scenarios, such as bacteria with or without multidrug resistance, respiratory viruses including SARS-CoV-2, molds, and tuberculous/nontuberculous mycobacteria, but also emerging pathogens, such as the mollicutes. The heart is the only transplanted organ that is a muscle and in direct contact with the bloodstream. These factors make donor-derived endocarditis, toxoplasmosis, and Chagas disease particularly relevant. This article aims to review some key established and emerging donor-derived infections that are of particular significance to heart and lung transplant recipients.</div></div>","PeriodicalId":100741,"journal":{"name":"JHLT Open","volume":"10 ","pages":"Article 100376"},"PeriodicalIF":0.0,"publicationDate":"2025-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144932378","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association between tacrolimus blood levels and biopsy-proven acute cellular rejection in adult heart transplant recipients","authors":"Chengliang Yang MD ,&nbsp;Casey P. Shannon BSc ,&nbsp;Sara Assadian MHS ,&nbsp;Linda Lapp PhD ,&nbsp;Rithika Nair BSc ,&nbsp;Tao Huan PhD ,&nbsp;Nilu Partovi PharmD ,&nbsp;Mustafa Toma MD ,&nbsp;Scott J. Tebbutt PhD","doi":"10.1016/j.jhlto.2025.100373","DOIUrl":"10.1016/j.jhlto.2025.100373","url":null,"abstract":"<div><h3>Background</h3><div>Acute cellular rejection (ACR) is a common complication following heart transplantation (HTx). This study examined the association between tacrolimus whole-blood concentrations and endomyocardial biopsy (EMB)-proven ACR in adult HTx recipients.</div></div><div><h3>Methods</h3><div>We conducted a retrospective analysis of 41 adult HTx recipients enrolled in the HEARTBiT study at St. Paul’s Hospital (Vancouver, Canada) between August 2018 and February 2020. A total of 315 EMB visits were analyzed and matched with tacrolimus whole-blood trough concentrations measured within ±1 day using liquid chromatography-tandem mass spectrometry. Patients were stratified into 2 post-transplant intervals: 0 to 90 days and 91 to 180 days, based on BC Clinical Guidelines for Transplant Medications for target tacrolimus levels.</div></div><div><h3>Results</h3><div>During the first 90 days post transplant, tacrolimus concentrations were significantly lower in 2R rejection episodes compared to both 0R (<em>p</em> = 0.006) and 1R (<em>p</em> = 0.013) groups. No significant differences in tacrolimus levels were observed beyond 90 days. In a linear mixed effects model adjusting for time post transplant (days) and tacrolimus dose, 2R rejection remained independently associated with lower tacrolimus concentrations (−2.73 µg/ml; <em>p</em> = 0.021), despite slightly higher dosing at those visits (+0.10 mg/d; <em>p</em> = 0.047). Clinical review confirmed no concurrent cytomegalovirus infections or major changes in other immunosuppressive therapies.</div></div><div><h3>Conclusions</h3><div>Lower tacrolimus concentrations during moderate ACR episodes were not attributable to underdosing or clinical confounders, suggesting the role of altered pharmacokinetics or patient-specific factors. Taken together, our results emphasize the clinical relevance of tailoring tacrolimus targets to individual pharmacokinetics, especially in early-phase post-transplant care.</div></div>","PeriodicalId":100741,"journal":{"name":"JHLT Open","volume":"10 ","pages":"Article 100373"},"PeriodicalIF":0.0,"publicationDate":"2025-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144932425","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Unlocking the potential of uncontrolled DCD in lung transplantation: A review of 2 decades of experience","authors":"Irene Bello MD, PhD ,&nbsp;Alessandro Palleschi MD ,&nbsp;Marcelo Cypel MD, PhD ,&nbsp;Eduard Argudo MD, PhD ,&nbsp;Alberto Sandiumenge MD, PhD","doi":"10.1016/j.jhlto.2025.100374","DOIUrl":"10.1016/j.jhlto.2025.100374","url":null,"abstract":"<div><div>Uncontrolled donation after circulatory death (uDCD) represents a promising yet underutilized approach to expanding the lung donor pool amid persistent organ shortages. Since the first successful lung transplantation from a uDCD donor in 2001, increasing clinical experience and advancements in organ preservation have demonstrated its feasibility. This review critically explores historical evolution, physiological basis, preservation techniques, ethical and legal considerations, and clinical outcomes of uDCD lung transplantation. The lung's unique ability to maintain viability through passive oxygen diffusion in the absence of perfusion supports its potential in the uDCD context. Compared to donors after brain death (DBD), uDCD donors may avoid systemic inflammatory response, potentially preserving graft quality. However, concerns persist regarding ischemia-reperfusion injury and mitochondrial dysfunction, highlighting the need for mitigation strategies such as ex vivo lung perfusion and normothermic ventilation. Ethical and legal challenges—particularly those related to the determination of death and consent—remain key obstacles. Organizational demands, including rapid coordination between prehospital, hospital teams and transplant teams, further limit broader implementation. Despite these barriers, reported outcomes are encouraging: to date, over 70 transplants from uDCD donors have been documented, with 1-year survival rates ranging from 71% to 87.5% and long-term outcomes comparable to DBD transplants. Integration of uDCD into routine clinical practice will require standardized protocols, robust public engagement, and institutional commitment. When appropriately implemented, uDCD lung transplantation offers a viable opportunity to increase donor availability and improve access to life-saving treatment.</div></div>","PeriodicalId":100741,"journal":{"name":"JHLT Open","volume":"10 ","pages":"Article 100374"},"PeriodicalIF":0.0,"publicationDate":"2025-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144932424","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Management of microaspiration and gastrointestinal dysfunction after lung transplantation: A narrative review","authors":"René Hage ,&nbsp;Carolin Steinack ,&nbsp;Macé M. Schuurmans","doi":"10.1016/j.jhlto.2025.100363","DOIUrl":"10.1016/j.jhlto.2025.100363","url":null,"abstract":"<div><h3>Background</h3><div>Chronic Lung Allograft Dysfunction (CLAD) is the leading cause of late morbidity and mortality following lung transplantation. Increasing evidence implicates microaspiration, often secondary to gastroesophageal reflux disease (GERD) and gastrointestinal (GI) dysfunction, as a critical non-alloimmune driver of CLAD. However, its often silent presentation, diagnostic complexity, and heterogeneous management contribute to persistent knowledge and treatment gaps.</div></div><div><h3>Methods</h3><div>This narrative review synthesizes recent literature on the pathophysiology, diagnosis, and clinical impact of microaspiration and GI dysfunction in lung transplant recipients. We focus on emerging biomarkers (e.g., conjugated bile acids and pepsinogen A4), diagnostic modalities, and both medical and surgical treatment strategies aimed at mitigating aspiration-induced graft injury.</div></div><div><h3>Key Content and Findings</h3><div>Microaspiration leads to epithelial damage, surfactant disruption, immune activation, and microbial dysbiosis, collectively promoting allograft dysfunction. Conjugated bile acids in large airway bronchial wash fluid and pepsinogen A4 have shown superior specificity as aspiration biomarkers compared to pepsin alone. Gastrointestinal disorders, such as GERD, gastroparesis, and esophageal dysmotility, frequently co-exist post-transplant and contribute to aspiration risk. Pharmacologic interventions provide limited benefit, while anti-reflux surgery significantly improves graft outcomes, particularly when performed early. Conservative measures such as head-of-bed elevation also reduce reflux burden and may complement therapeutic strategies.</div></div><div><h3>Conclusions</h3><div>Microaspiration is a modifiable and underrecognized contributor to allograft injury. Integration of aspiration biomarkers, early reflux evaluation, and personalized stepwise management, including surgical intervention when indicated, may improve long-term transplant outcomes. This review provides clinicians with a structured framework for diagnosis and management of microaspiration-related injury in lung transplantation.</div></div>","PeriodicalId":100741,"journal":{"name":"JHLT Open","volume":"10 ","pages":"Article 100363"},"PeriodicalIF":0.0,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144885974","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Fontan circulation and transplantation: Considerations for the complex candidate","authors":"Drishti Tolani ,&nbsp;Sharon Chen ,&nbsp;Edo Bedzra ,&nbsp;Kurt R. Schumacher ,&nbsp;Shahnawaz Amdani","doi":"10.1016/j.jhlto.2025.100366","DOIUrl":"10.1016/j.jhlto.2025.100366","url":null,"abstract":"<div><div>The Fontan operation has transformed survival for patients with single ventricle congenital heart disease, but the long-term durability of this physiology remains limited. Fontan circulatory failure (FCF) is a progressive, heterogeneous condition associated with multiorgan dysfunction, lymphatic failure, and elevated post-transplant risk when not recognized early. With increasing survival into adulthood, heart transplantation has emerged as the definitive “fourth stage” of palliation. However, referrals for advanced therapies are often delayed, with many patients presenting in advanced stages of decline. This review synthesizes current evidence on the pathophysiology of Fontan failure, outlines consensus indications for transplant evaluation, and highlights pre-transplant strategies including management of end-organ dysfunction, collateral embolization, and psychosocial readiness that are essential to optimizing outcomes. Advances in surgical technique and mechanical support has markedly improved post-transplant survival, yet challenges remain in timely referral, equitable access, and transition care. Early recognition and multidisciplinary coordination is key to improving outcomes in this vulnerable and growing population.</div></div>","PeriodicalId":100741,"journal":{"name":"JHLT Open","volume":"10 ","pages":"Article 100366"},"PeriodicalIF":0.0,"publicationDate":"2025-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144916562","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}