Journal of Heart and Lung Transplantation最新文献

筛选
英文 中文
Sports and Supervised Exercise Programmes for Children and Young Adults with Pulmonary Hypertension. 2026 Consensus Statement from the European Pediatric Pulmonary Vascular Disease Network (EPPVDN) and the Association for European Paediatric and Congenital Cardiology (AEPC). 2026年欧洲儿童肺血管疾病网络(EPPVDN)和欧洲儿科和先天性心脏病学会(AEPC)共识声明。
IF 6.7 1区 医学
Journal of Heart and Lung Transplantation Pub Date : 2026-07-29 DOI: 10.1016/j.healun.2026.07.026
Dan-Mihai Dorobantu, Alexander van de Bruaene, Werner Budts, Barbara Cifra, Mark K Friedberg, Peter Fritsch, Michael Khoury, Jan Müller, Alexander Opotowsky, Shubhayan Sanatani, Craig Williams, Georg Hansmann, Guido E Pieles
{"title":"Sports and Supervised Exercise Programmes for Children and Young Adults with Pulmonary Hypertension. 2026 Consensus Statement from the European Pediatric Pulmonary Vascular Disease Network (EPPVDN) and the Association for European Paediatric and Congenital Cardiology (AEPC).","authors":"Dan-Mihai Dorobantu, Alexander van de Bruaene, Werner Budts, Barbara Cifra, Mark K Friedberg, Peter Fritsch, Michael Khoury, Jan Müller, Alexander Opotowsky, Shubhayan Sanatani, Craig Williams, Georg Hansmann, Guido E Pieles","doi":"10.1016/j.healun.2026.07.026","DOIUrl":"https://doi.org/10.1016/j.healun.2026.07.026","url":null,"abstract":"<p><p>Pulmonary Hypertension (PH) in children and adolescents carries a significant mortality and morbidity, despite existing specific therapies. Strategies to ameliorate disease and improve outcome but also quality of life are needed. As in other pathologies, exercise as treatment and rehabilitation tool has been recently investigated also in children and young adults with PH. While evidence is not complete, available studies show its benefits. Exercise programmes can improve quality of life, reduce morbidity and positively impact on outcome parameters. Importantly, there is limited evidence on safety of exercise in PH, in particular in the paediatric population. While there are risks associated to exercise in this patient group, lack of education and guidance are equally barriers to sports and exercise participation in children and young adults with PH. This expert consensus document from EPPVDN describes exercise pathophysiology in PH, reviews the latest evidence for exercise programmes, assesses the safety of exercise and sports and for the first time gives recommendations. It follows current guidance on level of evidence classification, for structured exercise programmes but also eligibility for competitive sports.</p>","PeriodicalId":15900,"journal":{"name":"Journal of Heart and Lung Transplantation","volume":" ","pages":""},"PeriodicalIF":6.7,"publicationDate":"2026-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148620341","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Effect of surgical exposure on outcomes in bilateral lung transplantation: Insight from the international multicenter extracorporeal life support in lung transplantation registry. 手术暴露对双侧肺移植结果的影响:来自国际多中心体外生命支持(ECLS)肺移植登记的见解。
IF 6.7 1区 医学
Journal of Heart and Lung Transplantation Pub Date : 2026-07-29 DOI: 10.1016/j.healun.2026.07.019
Nunzio Davide de Manna, Sophie Kruszona, Andres Leon-Pena, Ramiro Fernandez, Uma Ramamurthy, Matthew Hartwig, Kamrouz Ghadimi, Nathaniel B Langer, Asishana A Osho, Mohammed Kashem, Yoshida Toyoda, Amir Emtiazjoo, Mindaugas Rackauskas, Laurens J Ceulemans, Cedric Vanluyten, Stephen Huddleston, Subramaniam Kathirvel, Silvana Crowley Carrasco, Pablo G Sanchez, Jawad Salman, Khalil Aburahma, Dirk Van Raemdonck, Gabriel Loor, Fabio Ius
{"title":"Effect of surgical exposure on outcomes in bilateral lung transplantation: Insight from the international multicenter extracorporeal life support in lung transplantation registry.","authors":"Nunzio Davide de Manna, Sophie Kruszona, Andres Leon-Pena, Ramiro Fernandez, Uma Ramamurthy, Matthew Hartwig, Kamrouz Ghadimi, Nathaniel B Langer, Asishana A Osho, Mohammed Kashem, Yoshida Toyoda, Amir Emtiazjoo, Mindaugas Rackauskas, Laurens J Ceulemans, Cedric Vanluyten, Stephen Huddleston, Subramaniam Kathirvel, Silvana Crowley Carrasco, Pablo G Sanchez, Jawad Salman, Khalil Aburahma, Dirk Van Raemdonck, Gabriel Loor, Fabio Ius","doi":"10.1016/j.healun.2026.07.019","DOIUrl":"10.1016/j.healun.2026.07.019","url":null,"abstract":"<p><strong>Background: </strong>Surgical access for bilateral lung transplantation (BLTx), including clamshell, sternotomy, and isolated bilateral thoracotomies (IBT), may influence perioperative outcomes. We analyzed the international ECLS Registry to assess the impact of access type on perioperative outcomes and graft survival.</p><p><strong>Methods: </strong>Adult BLTx performed from January 2016 to August 2024 across 12 centers was reviewed. Patients were grouped as being transplanted through clamshell (n = 451, 37.6%), IBT (n = 624, 52.0%), or sternotomy (n = 125, 10.4%). Three 1:1 propensity score-matched comparisons were performed: clamshell vs IBT, clamshell vs sternotomy, and IBT vs sternotomy.</p><p><strong>Results: </strong>Before matching, baseline characteristics differed significantly across surgical approaches, including BMI (25.2 vs 23.6 vs 24.3 kg/m², p < 0.001), diabetes (19.3% vs 14.9% vs 16.8%, p < 0.001), pulmonary hypertension (4.2% vs 4.8% vs 5.6%, p < 0.001), and intraoperative extracorporeal life support (ECLS) use (p < 0.001). After matching, IBT patients showed longer ischemic times (p< 0.001) but less intraoperative ECLS use, shorter ventilation and ICU stays (p < 0.001), lower reintubation (p = 0.021 vs clamshell; p = 0.045 vs sternotomy), fewer reoperations for bleeding (p = 0.190 vs clamshell; p = 0.018 vs sternotomy), and lower transfusion needs (p< 0.001). At 1 year, IBT was associated with higher pulmonary function and improved unadjusted graft survival than clamshell patients (1-year: 91% vs 86%; 3-year: 74% vs 63%; 5-year: 51% vs 49%; p = 0.007).</p><p><strong>Conclusion: </strong>Surgical access in BLTx affects perioperative morbidity and recovery. IBT was associated with improved early/intermediate graft survival compared with clamshell incision, although the absolute difference was attenuated at 5 years. Incision choice should be tailored to patient profile, procedural demands, and institutional expertise.</p>","PeriodicalId":15900,"journal":{"name":"Journal of Heart and Lung Transplantation","volume":" ","pages":""},"PeriodicalIF":6.7,"publicationDate":"2026-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148620271","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Global approaches to chronic lung allograft dysfunction: Toward evidence-based harmonization. 慢性同种异体肺移植功能障碍的全球方法:循证协调。
IF 6.7 1区 医学
Journal of Heart and Lung Transplantation Pub Date : 2026-07-28 DOI: 10.1016/j.healun.2026.07.029
Robin Vos
{"title":"Global approaches to chronic lung allograft dysfunction: Toward evidence-based harmonization.","authors":"Robin Vos","doi":"10.1016/j.healun.2026.07.029","DOIUrl":"10.1016/j.healun.2026.07.029","url":null,"abstract":"<p><p>Chronic lung allograft dysfunction (CLAD) remains the leading cause of late graft failure and mortality after lung transplantation, imposing a substantial societal burden. Over the past decade, international efforts have refined diagnostic criteria and phenotypic classification of CLAD, and advanced our understanding of the multifactorial mechanisms underlying CLAD onset. Contemporary international surveys provide valuable insight into real-world CLAD management, demonstrating practice harmonization alongside persistent geographic variation. Diagnostic evaluation of CLAD has converged through international consensus. Most transplant centers now incorporate spirometry, chest CT, bronchoscopy with bronchoalveolar lavage and transbronchial biopsies, and donor-specific antibody testing for CLAD surveillance and work-up. However, greater international standardization is still needed for pulmonary function test reporting, imaging protocols, and pathology workflows. Therapeutic management, however, remains more heterogeneous. Major differences persist in preventive strategies, particularly for azithromycin prophylaxis and gastroesophageal reflux management, as well as the use of corticosteroid pulse therapy, lymphocyte-depleting agents, extracorporeal photopheresis, and adjunctive treatments such as montelukast and inhaled bronchodilators in established CLAD. Similar variability is observed in other aspects of lung transplantation, including infection-directed strategies. Rather than representing a limitation, these practice variations identify priorities for future studies by highlighting areas where comparative evidence is lacking. The next decade of CLAD research should therefore focus on generating evidence needed to harmonize clinical care, while advancing molecular diagnostics-guided precision medicine and tailored treatments for CLAD. Standardizing therapeutic algorithms, studying biomarker-guided disease-modifying interventions, and pragmatic clinical trials are essential to transform experience-based CLAD management to evidence-based care, and to improve long-term transplant outcomes.</p>","PeriodicalId":15900,"journal":{"name":"Journal of Heart and Lung Transplantation","volume":" ","pages":""},"PeriodicalIF":6.7,"publicationDate":"2026-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148604159","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Successful treatment of bronchial anastomotic dehiscence after lung transplantation: First-in-man use of aerosolized cold atmospheric plasma. 肺移植后支气管吻合口破裂的成功治疗:首次使用雾化冷常压血浆。
IF 6.7 1区 医学
Journal of Heart and Lung Transplantation Pub Date : 2026-07-28 DOI: 10.1016/j.healun.2026.07.028
Frank Langer, Torben Rixecker, Carsten Zeiner, Parviz Aliyev, Migdat Mustafi, Robert Bals, Heinrike Wilkens
{"title":"Successful treatment of bronchial anastomotic dehiscence after lung transplantation: First-in-man use of aerosolized cold atmospheric plasma.","authors":"Frank Langer, Torben Rixecker, Carsten Zeiner, Parviz Aliyev, Migdat Mustafi, Robert Bals, Heinrike Wilkens","doi":"10.1016/j.healun.2026.07.028","DOIUrl":"10.1016/j.healun.2026.07.028","url":null,"abstract":"","PeriodicalId":15900,"journal":{"name":"Journal of Heart and Lung Transplantation","volume":" ","pages":""},"PeriodicalIF":6.7,"publicationDate":"2026-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148604206","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Tissue perfusion pressure as a physiologic indicator of renal failure risk after heart transplantation. 组织灌注压作为心脏移植后肾功能衰竭风险的生理指标。
IF 6.7 1区 医学
Journal of Heart and Lung Transplantation Pub Date : 2026-07-28 DOI: 10.1016/j.healun.2026.07.025
Chen Chia Wang, Walter Navid, Christian Eidson, Awab Ahmad, Mark Petrovic, Nikith Kurella, Kevin McGann, Aaron M Williams, John Trahanas, Swaroop Bommareddi, Tarek Absi, Eric Quintana, Aniket S Rali, Kelly Schlendorf, Matthew Bacchetta, Ashish Shah, Brian Lima
{"title":"Tissue perfusion pressure as a physiologic indicator of renal failure risk after heart transplantation.","authors":"Chen Chia Wang, Walter Navid, Christian Eidson, Awab Ahmad, Mark Petrovic, Nikith Kurella, Kevin McGann, Aaron M Williams, John Trahanas, Swaroop Bommareddi, Tarek Absi, Eric Quintana, Aniket S Rali, Kelly Schlendorf, Matthew Bacchetta, Ashish Shah, Brian Lima","doi":"10.1016/j.healun.2026.07.025","DOIUrl":"10.1016/j.healun.2026.07.025","url":null,"abstract":"<p><strong>Background: </strong>Mean arterial pressure (MAP) guides hemodynamic management following heart transplantation, yet renal failure remains common. Tissue perfusion pressure (TPP), defined as MAP minus critical closing pressure, predicts acute kidney injury in non-transplant cardiac surgery. We hypothesized that postoperative TPP predicts continuous renal replacement (CRRT) requirement after heart transplantation.</p><p><strong>Methods: </strong>We analyzed all adult heart transplants at a single institution (01/2020-06/2025) and excluded multiorgan transplants, congenital heart disease, and patients requiring post-transplant extracorporeal membrane oxygenation support. Hourly TPP, MAP, and organ perfusion pressure (OPP; MAP - central venous pressure) were analyzed over 72 h. Patients were stratified by post-transplant CRRT use. Predictive performance was compared using net reclassification improvement (NRI) across sequential 24-hour epochs.</p><p><strong>Results: </strong>Of the 269 patients included, 47 (17.5%) required CRRT. Those needing CRRT more likely had circulatory death donors (55.3% vs 38.3%, p=0.035) using direct procurement and perfusion (14.9% vs 6.3%, p=0.037). Pre-transplant creatinine was greater in the CRRT group (1.5mg/dL vs 1.2, p=0.002). In the CRRT group, the median and mean TPP, OPP, and MAP within the first 72 h were all lower (p < 0.01). Mean TPP demonstrated better prediction of CRRT compared to OPP (NRI 0.49, 95% CI 0.23-0.76, p<0.001) and MAP (NRI 0.39, 95% CI 0.11-0.68, p=0.007) within 24 h post-transplant. After 24 h, the predictive powers of median TPP, OPP, and MAP were similar.</p><p><strong>Conclusions: </strong>Early postoperative TPP independently predicted the need for CRRT after heart transplantation and demonstrated superior risk reclassification compared with conventional pressure-based hemodynamic metrics.</p>","PeriodicalId":15900,"journal":{"name":"Journal of Heart and Lung Transplantation","volume":" ","pages":""},"PeriodicalIF":6.7,"publicationDate":"2026-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148604153","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Comparative whole-exome sequencing of ambulatory patients and transplant recipients with idiopathic dilated cardiomyopathy. 特发性扩张型心肌病门诊患者和移植受者的全外显子组比较测序。
IF 6.7 1区 医学
Journal of Heart and Lung Transplantation Pub Date : 2026-07-28 DOI: 10.1016/j.healun.2026.07.022
Ju-Hee Lee, Jinhwa Kong, Seulgi Kim, Bong-Jo Kim, Hae Young Lee, Junho Hyun, Jae-Joong Kim, Hyun-Young Park, Myeong-Chan Cho, Sang Cheol Kim, Sang Eun Lee, Ali Torkamani
{"title":"Comparative whole-exome sequencing of ambulatory patients and transplant recipients with idiopathic dilated cardiomyopathy.","authors":"Ju-Hee Lee, Jinhwa Kong, Seulgi Kim, Bong-Jo Kim, Hae Young Lee, Junho Hyun, Jae-Joong Kim, Hyun-Young Park, Myeong-Chan Cho, Sang Cheol Kim, Sang Eun Lee, Ali Torkamani","doi":"10.1016/j.healun.2026.07.022","DOIUrl":"10.1016/j.healun.2026.07.022","url":null,"abstract":"<p><strong>Background: </strong>Idiopathic dilated cardiomyopathy (DCM) is a major cause of advanced heart failure and heart transplantation (HTx), yet the genetic correlates of progression to HTx and transplant-relevant arrhythmic phenotypes remain incompletely defined. We examined the genetics of idiopathic DCM in a Korean population, focusing on HTx/death and arrhythmic outcomes, to identify adverse outcome-linked genotype-phenotype associations.</p><p><strong>Methods: </strong>Whole-exome sequencing was performed in 202 Korean patients with idiopathic DCM, including 56 HTx recipients and 146 ambulatory patients, and compared the findings with 1093 population-based controls. Genotype-phenotype correlations were analyzed for major clinical outcomes, including HTx, death, arrhythmias, and left ventricular functional recovery.</p><p><strong>Results: </strong>Pathogenic/likely pathogenic variants were identified in 32% of patients (38% in HTx vs 30% in ambulatory patients). TTN was the most frequently affected gene overall (12%), but LMNA variants predominated in HTx recipients (20% vs 4%, p = 0.001). LMNA carriers showed substantially higher odds of HTx/death (OR 14.65, 95% CI 3.32-139.31; FDR p<0.001), and strong association with arrhythmias, including ventricular tachyarrhythmias and atrial fibrillation. Both missense and loss-of-function LMNA variants were associated with adverse outcomes. In contrast, TNNT2 variants were observed exclusively in ambulatory patients and identified a favorable functional-recovery phenotype, with a greater likelihood of LVEF recovery ≥10 percentage points (OR 6.03, 95% CI 1.52-28.71; FDR p = 0.016).</p><p><strong>Conclusions: </strong>LMNA variants mark a high-risk transplant-trajectory phenotype in Korean idiopathic DCM. Genetic testing may aid early identification and management of candidates for advanced HF therapies, including HTx and durable MCS.</p>","PeriodicalId":15900,"journal":{"name":"Journal of Heart and Lung Transplantation","volume":" ","pages":""},"PeriodicalIF":6.7,"publicationDate":"2026-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148604150","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Donor disposition and lung utilization following "Not Expected to Progress" coding in donation after circulatory death: A national registry analysis. 循环性死亡后捐赠“不期望进展”编码后的供体处置和肺利用:一项国家登记分析。
IF 6.7 1区 医学
Journal of Heart and Lung Transplantation Pub Date : 2026-07-25 DOI: 10.1016/j.healun.2026.07.018
Brian Jafari, Lauryn Spinetta, Vaidehi Kaza, Matthias Peltz, Christopher Heid, John Murala, Suresh Keshavamurthy
{"title":"Donor disposition and lung utilization following \"Not Expected to Progress\" coding in donation after circulatory death: A national registry analysis.","authors":"Brian Jafari, Lauryn Spinetta, Vaidehi Kaza, Matthias Peltz, Christopher Heid, John Murala, Suresh Keshavamurthy","doi":"10.1016/j.healun.2026.07.018","DOIUrl":"10.1016/j.healun.2026.07.018","url":null,"abstract":"<p><strong>Background: </strong>Donation-after-circulatory-death (DCD) lung transplantation expands access for waitlisted patients, but uncertainty regarding donor progression may lead centers to decline offers using refusal code 753 (\"DCD donor neurological function/not expected to arrest\"). We sought to characterize the disposition of these donors and center practices in their allocation.</p><p><strong>Methods: </strong>Using national registry match-run data from January 2021 through June 2024 linked with Organ Procurement and Transplant Network (OPTN) transplant data, we identified DCD donors from whom lung offers were generated. Donors declined at least once with code 753 were classified as not expected to progress (NEP). Donor progression, organ procurement, and post-transplant survival were compared with standard DCD (S-DCD) donors.</p><p><strong>Results: </strong>Among 6,140 NEP donors, 3,687 (60.0%) progressed to circulatory death and had at least one organ procured. Among progressed NEP donors, lungs were recovered from 908 donors (24.6%), and 670 donors ultimately yielded at least one transplanted lung. Post-transplant survival did not differ between linked recipients of NEP and S-DCD lungs (log-rank p = 0.15).</p><p><strong>Conclusions: </strong>Many DCD donors declined using code 753 ultimately progressed after withdrawal of life-sustaining therapy, with lungs recovered in only a minority of these cases. The uncertainty surrounding donor progression may restrict access to DCD lung transplantation for waitlisted patients. Standardized DCD assessment, donor management, and policy guidance may help centers manage uncertainty when fielding DCD offers.</p>","PeriodicalId":15900,"journal":{"name":"Journal of Heart and Lung Transplantation","volume":" ","pages":""},"PeriodicalIF":6.7,"publicationDate":"2026-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148592060","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Misdiagnosed as idiopathic pulmonary arterial hypertension: Methylmalonic acidemia as a reversible cause of pediatric pulmonary hypertension. 误诊为特发性多环芳烃:甲基丙二酸血症是儿童肺动脉高压的可逆原因。
IF 6.7 1区 医学
Journal of Heart and Lung Transplantation Pub Date : 2026-07-25 DOI: 10.1016/j.healun.2026.07.021
Yan Wu, Yuan-Rui Deng, Xiao-Pei Cui, Sheng-Song Zhu, Jiang-Shan Tan, Song Hu, Lu Hua
{"title":"Misdiagnosed as idiopathic pulmonary arterial hypertension: Methylmalonic acidemia as a reversible cause of pediatric pulmonary hypertension.","authors":"Yan Wu, Yuan-Rui Deng, Xiao-Pei Cui, Sheng-Song Zhu, Jiang-Shan Tan, Song Hu, Lu Hua","doi":"10.1016/j.healun.2026.07.021","DOIUrl":"10.1016/j.healun.2026.07.021","url":null,"abstract":"<p><strong>Background: </strong>Methylmalonic acidemia (MMA)-induced pulmonary hypertension (PH) is a rare but treatable cause of pediatric PH, often misdiagnosed as idiopathic pulmonary arterial hypertension (IPAH).</p><p><strong>Methods: </strong>We conducted a 10-year, multicenter retrospective study of children with clinically unexplained PH. Thirteen patients with MMA-PH were compared to 113 with idiopathic or hereditary PAH (IPAH/HPAH) regarding clinical features, hemodynamics, and outcomes.</p><p><strong>Results: </strong>MMA-PH accounted for 2.7% of pediatric PH cases. Patients presented younger (7±4 vs. 11±5 years, p=0.008) and exhibited higher rates of growth failure (84.6% vs. 15.9%, p<0.001), anorexia/malnutrition (76.9% vs. 14.2%, p<0.001), recurrent pneumonia (46.2% vs. 13.3%, p=0.003), microscopic hematuria (84.6% vs. 1.8%, p<0.001), and proteinuria (31% vs. 4.4%, p=0.007) than IPAH/HPAH. All had the combined MMA subtype with markedly elevated homocysteine (median 87 µmol/L vs. 18 µmol/L, p<0.001). With metabolic therapy (hydroxocobalamin, betaine, etc.) and short-term pulmonary vasodilators, all achieved complete clinical and hemodynamic remission within 1 year. Median follow-up was 7.0 years; no relapse occurred.</p><p><strong>Conclusions: </strong>Elevated total homocysteine and multisystem involvement should prompt metabolic screening in children with unexplained PH. Early diagnosis and metabolite-targeted treatment can lead to sustained, nearly curative outcomes, distinguishing MMA-PH from IPAH/HPAH. Routine homocysteine testing is recommended in the diagnostic workup of pediatric PH.</p>","PeriodicalId":15900,"journal":{"name":"Journal of Heart and Lung Transplantation","volume":" ","pages":""},"PeriodicalIF":6.7,"publicationDate":"2026-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148592032","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
When "not a candidate" becomes a verdict: Reframing intensive care for patients with advanced heart failure. 当“不是候选人”成为判决:重新制定晚期心力衰竭患者的重症监护。
IF 6.7 1区 医学
Journal of Heart and Lung Transplantation Pub Date : 2026-07-25 DOI: 10.1016/j.healun.2026.07.023
Diana De Oliveira Gomes, Shashank S Sinha, Vanessa Blumer
{"title":"When \"not a candidate\" becomes a verdict: Reframing intensive care for patients with advanced heart failure.","authors":"Diana De Oliveira Gomes, Shashank S Sinha, Vanessa Blumer","doi":"10.1016/j.healun.2026.07.023","DOIUrl":"10.1016/j.healun.2026.07.023","url":null,"abstract":"<p><p>The designation \"not an advanced heart failure candidate\" is applied to patients deemed ineligible for heart transplantation or durable left ventricular assist device (LVAD) therapy at a specific point in time. While this designation accurately captures a therapeutic boundary, it is increasingly conflated with broader notions of futility, diminished prognosis, and reduced appropriateness for cardiac intensive care unit (CICU) admission. This conflation is both clinically inaccurate and potentially harmful. Transplant and LVAD candidacy are determined by a narrow, time-sensitive, and often modifiable set of criteria that do not necessarily reflect whether a patient can benefit from invasive hemodynamic monitoring, decongestion, guideline-directed medical therapy initiation, rhythm or conduction interventions, or coronary or peripheral revascularization. Contemporary data demonstrate that acute decompensated heart failure now accounts for most cardiogenic shock cases encountered in contemporary CICUs, a population in which advanced therapy ineligibility is common yet in whom thoughtful critical care may yield meaningful hemodynamic improvement, end-organ recovery, and reassessment of candidacy. This perspective argues for a triage framework that evaluates CICU appropriateness across 3 independent domains: acute physiologic need, reversibility of the index derangement, and longitudinal prognosis aligned with patient preferences. Advanced therapy ineligibility should close one door, not determine how many remain open.</p>","PeriodicalId":15900,"journal":{"name":"Journal of Heart and Lung Transplantation","volume":" ","pages":""},"PeriodicalIF":6.7,"publicationDate":"2026-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148592131","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Distinct metabolomic and lipidomic profiles across donation after circulatory death recovery strategies reveal a common signature associated with primary graft dysfunction. 不同的代谢组学和脂质组学特征在循环死亡恢复策略后的捐赠中揭示了与原发性移植物功能障碍相关的共同特征。
IF 6.7 1区 医学
Journal of Heart and Lung Transplantation Pub Date : 2026-07-01 DOI: 10.1016/j.healun.2026.06.017
Elizabeth J Bashian, Sariah Hyacinth, Charles D Nicoli, Thomas F O'Shea, Samantha L Nelson, Anit Tyagi, Logan Boyle, Nicholas R Teman, Amrut V Ambardekar, Andrew Gelman, Wanzhu Zhao, Michael T Cain, Jordan R H Hoffman, Benjamin J Kopecky
{"title":"Distinct metabolomic and lipidomic profiles across donation after circulatory death recovery strategies reveal a common signature associated with primary graft dysfunction.","authors":"Elizabeth J Bashian, Sariah Hyacinth, Charles D Nicoli, Thomas F O'Shea, Samantha L Nelson, Anit Tyagi, Logan Boyle, Nicholas R Teman, Amrut V Ambardekar, Andrew Gelman, Wanzhu Zhao, Michael T Cain, Jordan R H Hoffman, Benjamin J Kopecky","doi":"10.1016/j.healun.2026.06.017","DOIUrl":"10.1016/j.healun.2026.06.017","url":null,"abstract":"<p><p>Donation after circulatory death (DCD) heart transplantation has expanded the donor pool, but the biologic consequences of different recovery strategies remain incompletely understood. Using coronary sinus blood obtained immediately following reperfusion, we performed metabolomic profiling in 31 DCD heart transplant recipients and lipidomic profiling in a nested cohort of 11 recipients with various DCD procurement methods as follows: thoraco-abdominal normothermic regional perfusion (n=10); normothermic machine perfusion (n=10), or cold oxygenated rapid recovery (n=11). Partial least squares discriminant analysis demonstrated distinct metabolomic clustering according to procurement strategy. Moderate or severe PGD occurred in 16 recipients (51.6%) and was associated with depletion of tricarboxylic acid (TCA) cycle and redox metabolites together with accumulation of acylcarnitines and amino-acid catabolic intermediates, consistent with impaired mitochondrial energetics, oxidative stress, and membrane injury. Despite distinct procurement-specific metabolic phenotypes, PGD was associated with a common metabolic signature characterized by impaired mitochondrial energetics, oxidative stress, acylcarnitine accumulation, and phospholipid remodeling, highlighting potential mechanistic targets for graft preservation and risk stratification.</p>","PeriodicalId":15900,"journal":{"name":"Journal of Heart and Lung Transplantation","volume":" ","pages":""},"PeriodicalIF":6.7,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148361288","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
相关产品
×
本文献相关产品
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术官方微信
小红书