Orthotopic Heart Transplantation Research Articles

Diabetes mellitus in stable chronic heart failure and the combination with humoral activation, their association, and prediction of 2-year adverse outcomes. Data from the FAR NHL registry.

The study aims to describe the role of diabetes in patients with heart failure. In all, 1052 chronic heart failure patients were included in the FARmacology and NeuroHumoral Activation (FAR NHL) multicenter prospective registry. They had ejection fraction below 50% and were on stable medication for at least 1 month. More than one-third (38.9%) of the patients had diabetes mellitus (DM). Diabetic patients (N = 409) were older (median 67 vs. 64, p < 0.001), had higher body mass index (BMI) (30 vs. 28 kg/m2, p < 0.001), much more frequently had ischemic heart disease (71 vs. 47%, p < 0.001), hypertension (80 vs. 67%, p < 0.001), dyslipidemia (89 vs. 69%, p < 0.001), worse renal function (estimated glomerular filtration rate [eGFR] median 63 vs. 73 mL/min/1.73 m2, p < 0.001), and higher N-terminal pro-brain natriuretic peptide (NT-proBNP) (median 681 vs. 463 pg/mL, p = 0.003). All-cause death, left ventricle assist device implantation, and orthotopic heart transplantation were set as the combined primary end point, which was present in 15.5% (163 patients) within the 2-year follow-up. In the 2-year follow-up, 81.0% of patients with diabetes survived without a primary end point, while 85.4% of the patients without diabetes survived, the difference being on the verge of statistical significance (p = 0.089). DM is a statistically significant predictor of NT-proBNP value in univariate analysis, but it is not an independent predictor in a multivariate analysis. When the NT-proBNP level was high, the presence of DM did not influence the prognosis. The combination of diabetes and NT-proBNP levels may better stratify the prognosis of patients with chronic heart failure.

Donor and Recipient Age Influence Outcomes Following Orthotopic Heart Transplantation in the 2018 US Heart Allocation System.

This study evaluates the interaction of donor and recipient age with outcomes following heart transplantation under the 2018 heart allocation system. The United Network for Organ Sharing registry was queried to analyze adult primary isolated orthotopic heart transplant recipients and associated donors from August 18, 2018, to June 30, 2021. Both recipient and donor cohorts were grouped according to age: <65 and ≥65 y for recipients and <50 and ≥50 y for donors. The primary outcome was survival. Subanalyses were performed to evaluate the impact of donor age. A total of 7601 recipients and 7601 donors were analyzed. Of these, 1584 recipients (20.8%) were ≥65 y old and 560 donors (7.4%) were ≥50 y old. Compared with recipients <65, recipients ≥65 had decreased 1-y (88.8% versus 92.3%) and 2-y (85.1% versus 88.5%) survival rates (P < 0.001). The association of recipient age ≥65 with lower survival persisted after adjusting for potential cofounders (hazard ratio, 1.38; 95% confidence interval, 1.18-1.61; P < 0.001). Recipients <65 with donors ≥50 had comparable 1-y and 2-y survival rates to recipients <65 with donors <50 (P =0.997). Conversely, transplantation of older allografts was associated with lower 1-y (84.2% versus 89.4%) and 2-y (79.5% versus 85.8%) survival rates in recipients ≥65 (P = 0.025). Recipient age ≥65 continues to be associated with worse survival following heart transplantation in the 2018 heart allocation system compared with younger recipients. Donors ≥50 may be acceptable among recipients <65 with comparable outcomes. However, careful donor age selection should be considered for recipients ≥65, as the use of younger donor allografts appears to improve posttransplantation survival.

Implantation of a permanent pacemaker following orthotopic heart transplantation: a systematic review and meta-analysis.

Orthotopic heart transplant (OHT) is among the final armamentariums for end-stage heart disease. Many patients who have had OHT require a post-transplant permanent pacemaker (PPM) implantation due to an abnormal conduction system. We aimed to evaluate the risk of mortality and acute rejection in patients with OHT who had received PPM compared to patients without PPM and to determine predictors for PPM placement in this population. We comprehensively searched for studies from MEDLINE, EMBASE, and Cochrane databases from inception to September 2023. Inclusion criteria focused on patients who had undergone OHT and PPM implantation post-transplant. Data from each study were combined using a random-effects model. Results were expressed as relative risk (RR) or odd ratios (OR) with a 95% confidence interval (CI). A total of 9 studies were included in this meta-analysis incorporating a total of 54,848 patients (3.3% had PPM). The pooled all-cause mortality rate among patients with PPM post-OHT was 26% (95% CI: 19-33%, I2 = 1%). There were no differences between post-heart transplant patients with PPM and those without PPM in risk of all-cause mortality (RR 0.76, 95% CI: 0.43-1.34; I2 = 45%) and acute rejection (RR 1.22, 95% CI: 0.74-2.00, I2 = 59%). Bi-atrial anastomosis was associated with an increased risk of PPM implantation post-OHT (OR 7.74, 95% CI: 3.55-16.91, I2 = 0%), while pre-OHT mechanical circulatory support (MCS) was associated with a decreased risk of PPM implantation post-OHT (OR 0.45, 95% CI 0.27-0.76, I2 = 0%). There were no significant differences in all-cause mortality or acute rejection between post-OHT recipients who required PPM compared to those who did not receive PPM. Further, bi-atrial anastomosis portended the need for PPM implantation, while MCS was associated with a decreased occurrence of PPM.

Dynamic assessment of left ventricular coupling and myocardial reserve in patients with cardiogenic shock.

Pulmonary artery catheter haemodynamics are associated with improved survival in cardiogenic shock (CS). We investigated the utility of aortic pulsatility index (API) and cardiac power output (CPO) as surrogates for left ventricular (LV) coupling and myocardial reserve, respectively, in patients with CS undergoing dynamic assessment after a milrinone bolus. Patients with SCAI Stage C CS underwent a milrinone drug study (50 mcg/kg bolus infused over 10 min) to assess inotropic response. Haemodynamic measurements were obtained at baseline and following the bolus. Aortic pulsatility index and CPO were used to risk-stratify patients with the incidence of LV assist device (LVAD), orthotopic heart transplantation (OHT), or death at 1 year as the primary composite endpoint. Two hundred and twenty-four patients in SCAI Stage C CS underwent haemodynamics prior to milrinone bolus, and 117 patients had low baseline API < 1.45. Of the 117 patients, 88 had a final API < 2.2 after milrinone load, consistent with LV decoupling, in which 73% met the composite endpoint. The remaining 29 patients had a final API ≥ 2.2 consistent with LV recoupling, and only 55% met the composite endpoint (P = 0.046). Of the 117 patients, 40 patients had low myocardial reserve (final CPO < 0.77 W), in which 78% met the composite endpoint. Of the 77 patients who demonstrated myocardial reserve (final CPO ≥ 0.77 W), only 64% met the composite endpoint (P = 0.039). The use of API and CPO in a dynamic assessment after provocative testing led to improved risk stratification in patients with SCAI Stage C CS for clinical outcomes including LVAD, OHT, or death at 1 year.

Heart transplantation under mechanical circulatory support for fulminant myocarditis: a Case Report

Fulminant myocarditis has been defined as the clinical manifestation of cardiac inflammation with rapid-onset heart failure and cardiogenic shock. We report on the case of a 17-year-old boy with hemodynamic derangement and cardiac arrest due to fulminant myocarditis. After about 2 h of intensive cardiopulmonary resuscitation, with 13 days of extracorporeal membrane oxygenation support, the patient finally bridged to orthotopic heart transplantation. The patient recovered uneventfully and was discharged 37 days after transplantation. The explanted heart revealed diffuse lymphocytic infiltration and myocyte necrosis in all four cardiac chamber walls confirming the diagnosis and identifying the underlying cause of fulminant myocarditis.

Reversible cerebral vasoconstriction syndrome post-cardiac transplantation: a therapeutic dilemma: case report

BackgroundReversible cerebral vasoconstriction syndrome (RCVS) is characterized by diffuse, multifocal segmental narrowing of cerebral arteries and can result in ischaemic stroke. Causal factors, identified in 60% of cases, include immunosuppressant pharmacotherapy. The few reports following heart transplantation are almost all in Asian recipients. We report on a Caucasian Australian patient with immunotherapy induced RCVS post heart transplantation to highlight the state of knowledge of the condition and the treatment dilemma it poses.Case presentationA 51-year-old female underwent orthotopic heart transplantation at our institution. Induction immunotherapy comprised basiliximab, mycophenolate mofetil and methylprednisolone. On day 6 post-transplantation the patient was transitioned to oral prednisolone and tacrolimus. On day 7 the patient began to experience bilateral, severe, transient occipital and temporal headaches. On day 9 tacrolimus dose was up-titrated. A non-contrast computed tomography brain (CTB) was normal. Endomyocardial biopsy on day 12 demonstrated moderate Acute Cellular Rejection (ACR), which was treated with intravenous methylprednisolone. That evening the patient experienced a 15-minute episode of expressive dysphasia. The following morning she became confused, aphasic, and demonstrated right sided neglect and right hemianopia. A CT cerebral perfusion scan demonstrated hypoperfusion in the left middle cerebral artery (MCA) territory and cerebral angiography revealed widespread, focal multi-segmental narrowing of the anterior and posterior circulations. A diagnosis of RCVS was made, and nimodipine was commenced. As both steroids and tacrolimus are potential triggers of RCVS, cyclosporin replaced tacrolimus and methylprednisolone dose was reduced. A further CTB demonstrated a large left MCA territory infarct with left M2 MCA occlusion. The patient made steady neurological improvement. She was discharged 34 days post-transplantation with mild residual right lower limb weakness and persistent visual field defect on verapamil, cyclosporine, everolimus, mycophenolate mofetil and prednisolone.ConclusionReversible cerebral vasoconstriction syndrome is rare after orthotopic heart transplantation. Until now, RCVS has been almost exclusively described in Asian recipients, and is typically caused by immunotherapy. The condition may lead to permanent neurological deficits, and in the absence of definitive treatments, early recognition and imaging based diagnosis is essential to provide the opportunity to remove the causal agent(s). Co-existent ACR, can pose unique treatment difficulties.

Endomyocardial biopsy through the left internal jugular vein in heart transplant recipients: a single center experience.

Endomyocardial biopsies for rejection monitoring after heart transplantation are generally performed through the right internal jugular vein. We aimed to assess the feasibility and safety of using the left internal jugular vein approach as a valid alternative to a femoral vein for endomyocardial biopsies whenever thrombosis of the right internal jugular vein precludes insertion of a bioptome. We have reviewed our experience with heart transplantation in the last 2 decades to identify patients in whom surveillance endomyocardial biopsy was performed through the left internal jugular vein. We herein describe the step-by-step procedure and report the preliminary results. From May 1, 2000 to January 31, 2024, 561 orthotopic heart transplants have been performed in our unit. In 49 patients (8.7%), the right internal jugular vein access was found to be unsuitable or occluded at eco-Doppler evaluation; in 15 of them (30%) a total of 206 endomyocardial biopsies have been performed, using the left internal jugular vein, without complications. Our experience demonstrates that the left internal jugular vein is a valid alternative approach for endomyocardial biopsies when the right internal jugular vein is not available. This technique is feasible, safe and reproducible and allows adequate rejection monitoring after heart transplantation.

First cases of transcatheter edge-to-edge repair after orthotopic heart transplantation in Greece

First cases of transcatheter edge-to-edge repair after orthotopic heart transplantation in Greece

Clonal haematopoiesis is associated with major adverse cardiovascular events in patients with hypertrophic cardiomyopathy.

The heterogeneous phenotype of hypertrophic cardiomyopathy (HCM) is still not fully understood. Clonal haematopoiesis (CH) is emerging as a cardiovascular risk factor potentially associated with adverse clinical events. The prevalence, phenotype and outcomes related to CH in HCM patients were evaluated. Patients with HCM and available biospecimens from the Peter Munk Cardiac Centre Cardiovascular Biobank were subjected to targeted sequencing for 35 myeloid genes associated with CH. CH prevalence, clinical characteristics, morphological phenotypes assessed by echocardiogram and cardiac magnetic resonance and outcomes were assessed. All patients were evaluated for a 71-plex cytokines/chemokines, troponin I and B-type natriuretic peptide analysis. Major adverse cardiovascular events (MACE) were defined as appropriate implantable cardioverter-defibrillator shock, stroke, cardiac arrest, orthotopic heart transplant and death. Among the 799 patients, CH was found in 183 (22.9%) HCM patients with sarcomeric germline mutations. HCM patients with CH were more symptomatic and with a higher burden of fibrosis than those without CH. CH was associated with MACE in those HCM patients with sarcomeric germline mutations (adjusted hazard ratio [HR] 6.89, 95% confidence interval [CI] 1.78-26.6; p = 0.005), with the highest risk among those that had DNMT3A, TET2 and ASXL1 mutations (adjusted HR 5.76, 95% CI 1.51-21.94; p = 0.010). Several cytokines (IL-1ra, IL-6, IL-17F, TGFα, CCL21, CCL1, CCL8, and CCL17), and troponin I were upregulated in gene-positive HCM patients with CH. These results indicate that CH in patients with HCM is associated with worse clinical outcomes. In the absence of CH, gene-positive patients with HCM have lower rates of MACE.

Quantification of Aortic Valve Fibrotic and Calcific Tissue from CTA: Prospective Comparison with Histology.

Background Quantifying the fibrotic and calcific composition of the aortic valve at CT angiography (CTA) can be useful for assessing disease severity and outcomes of patients with aortic stenosis (AS); however, it has not yet been validated against quantitative histologic findings. Purpose To compare quantification of aortic valve fibrotic and calcific tissue composition at CTA versus histologic examination. Materials and Methods This prospective study included patients who underwent CTA before either surgical aortic valve replacement for AS or orthotopic heart transplant (controls) at two centers between January 2022 and April 2023. At CTA, fibrotic and calcific tissue composition were quantified using automated Gaussian mixture modeling applied to the density of aortic valve tissue components, calculated as [(volume/total tissue volume) × 100]. For histologic evaluation, explanted valve cusps were stained with Movat pentachrome as well as hematoxylin and eosin. For each cusp, three 5-µm slices were obtained. Fibrotic and calcific tissue composition were quantified using a validated artificial intelligence tool and averaged across the aortic valve. Correlations were assessed using the Spearman rank correlation coefficient. Intermodality and interobserver variability were measured using the intraclass correlation coefficient (ICC) and Bland-Altman plots. Results Twenty-nine participants (mean age, 63 years ± 10 [SD]; 23 male) were evaluated: 19 with severe AS, five with moderate AS, and five controls. Fibrocalcific tissue composition strongly correlated with histologic findings (r = 0.92; P < .001). The agreement between CTA and histologic findings for fibrocalcific tissue quantification was excellent (ICC, 0.94; P = .001), with underestimation of fibrotic composition at CTA (bias, -4.9%; 95% limits of agreement [LoA]: -18.5%, 8.7%). Finally, there was excellent interobserver repeatability for fibrotic (ICC, 0.99) and calcific (ICC, 0.99) aortic valve tissue volume measurements, with no evidence of a difference in measurements between readers (bias, -0.04 cm3 [95% LoA: -0.27 cm3, 0.19 cm3] and 0.02 cm3 [95% LoA: -0.14 cm3, 0.19 cm3], respectively). Conclusion In a direct comparison, standardized quantitative aortic valve tissue characterization at CTA showed excellent concordance with histologic findings and demonstrated interobserver reproducibility. Clinical trial registration no. NCT06136689 Published under a CC BY 4.0 license. Supplemental material is available for this article. See also the editorial by Almeida in this issue.

Variability in Pacemaker Programming Following Orthotopic Heart Transplantation

Variability in Pacemaker Programming Following Orthotopic Heart Transplantation

Transluminal balloon angioplasty and stenting of coronary arteries after prior orthotopic heart transplantation: a case report

Introduction. Today, heart failure (HF) is the most common natural outcome of diseases that are not diagnosed and treated in time. Despite the advances in cardiovascular pharmacotherapy, orthotopic heart transplantation is a generally recognized gold standard for the treatment of end-stage heart failure.Brief description. The article describes a 55-year-old male patient admitted to the Cardiology Dispensary (Kabardino-Balkarian Republic) with a clinical picture of non-ST segment elevation acute coronary syndrome (ACS). Earlier in 2017, the patient underwent orthotopic heart transplantation (OHT) for end-stage HF due to dilated cardiomyopathy. Hypertension was registered 2,5 years after surgery, and clinical manifestations of HF were registered for 5 years. In 2024, coronary angiography revealed subtotal stenosis of 95% in the middle segment of the anterior descending artery. Intraoperatively, the consensus assessed the risk and made a decision on surgical endovascular treatment of the stenosis. The postoperative period was uneventful.Discussion. The presented case is an example of timely diagnosis of coronary artery disease in a transplanted heart and successful transluminal balloon angioplasty and stenting of the anterior descending artery.

Hip replacement in a heart transplant patient: a case report

Solid organ transplantation is considered the most effective treatment for endstage heart, lung, pancreatic, liver, and kidney diseases. The increasing number of transplants performed, and long-term survival of recipients have contributed to an increase in the diagnostic rate of non-transplant-related diseases.Heart transplant patients are at increased risk of complications when undergoing elective or emergency surgery. We report a case of hip replacement in a young man after orthotopic heart transplantation.

Association of Amiodarone Use Prior to Orthotopic Heart Transplant with Post-Transplant Graft Dysfunction and All-Cause Mortality: A Systematic Review and Meta-Analysis

There is conflicting data on the association between pre-orthotopic heart transplant (OHT) amiodarone use and post-OHT graft dysfunction (GD) leading to heterogeneity in clinical practice. We performed a meta-analysis to evaluate whether pre-OHT amiodarone use was associated with meaningful increases in the incidence of GD, 30-day mortality, and 1-year mortality. Studies were identified by searching PubMed and the Cochrane Register of Clinical Trials. The Mantel-Haenszel method was used to calculate odds ratios (OR) and 95% confidence intervals (CI95) for each endpoint. 17 retrospective studies were identified that included 48,782 patients. 14 studies (n = 48,018) reported GD as an outcome. Pre-OHT amiodarone use was associated with increased odds of GD (OR 1.3, CI95 1.2-1.5, p < 0.001). 10 studies (n = 45,875) reported 30-day mortality based on amiodarone use. Pre-OHT amiodarone use was associated with increased odds of 30-day mortality (OR 1.4, CI95 1.2-1.5, p < 0.001). 5 studies (n = 41,404) reported 1-year mortality based on amiodarone use. Pre-OHT amiodarone use was associated with increased odds of 1-year mortality (OR 1.2, CI95 1.1-1.4, p < 0.001). The increase in absolute risk of GD, 30-day mortality, and 1-year mortality for patients with pre-OHT amiodarone use was 1.3%, 1.2%, and 1.4%, respectively. Pre-OHT amiodarone exposure was associated with increased odds of GD, 30-day mortality, and 1-year mortality. The increase in absolute risk for each endpoint was modest, and it is unclear to what extent, if any, pre-OHT amiodarone use should influence assessment of OHT candidacy.

Contemporary Bridge to Heart Transplantation With Venoarterial Extracorporeal Membrane Oxygenation.

Objective: In October 2018, the United Network for Organ Sharing changed their heart allocation criteria to prioritize patients on temporary mechanical circulatory support. This study assesses outcomes of patients bridged to orthotopic heart transplantation (OHT) with venoarterial extracorporeal membrane oxygenation (VA ECMO) since this change. Methods: We conducted a retrospective single-center study of adults (≥18 years) supported with VA ECMO at the time of OHT (October 1, 2018, to December 31, 2021). The primary outcome was midterm survival. Results: During the study period, 117 patients underwent OHT including 52 adults ≥18 years (44%) and 65 children <18 years (56%). Among adults, 8 (15%) were supported with VA ECMO at the time of OHT and are included in this study; 75% were male (n = 6), and the median age was 52.5 (interquartile range [IQR] = 23.5 to 57.25) years. Most patients were peripherally cannulated (75%, n = 6) and supported with an intra-aortic balloon pump during the pretransplant period (87.5%, n = 7). The median ECMO duration was 7 (IQR = 4.5 to 25.25) days. Three patients experienced complications on ECMO (37.5%), including thromboembolic bowel infarction (12.5%, n = 1) and bleeding requiring reintervention (25%, n = 2). All patients survived to discharge without posttransplantation complications and were alive at the latest follow-up. The median follow-up time was 24.8 (IQR = 19.5 to 28.2) months. Conclusions: Patients can be successfully bridged with VA ECMO directly to OHT with excellent midterm results. Key contributors to our outcomes include early extubation, use of bivalirudin over heparin, ambulation, and rehabilitation while on ECMO.

Restenosis rates for drug-eluting stents used in treating small vessel cardiac allograft vasculopathy after orthotopic heart transplantation

BackgroundCardiac allograft vasculopathy (CAV) is associated with increased mortality in patients with orthotopic heart transplantation (OHT). In addition to immunosuppression, CAV can be treated with percutaneous coronary intervention (PCI) with drug eluting stents (DES) for focal lesions. There is a paucity of data on the rate of DES restenosis in patients with small vessel CAV. MethodsThis was a retrospective observational study of 101 coronary vessels treated with a DES diameter of 2.5 mm or less (small vessels) in 61 OHT patients compared to 72 coronary vessels treated with a DES diameter of >2.5 mm (large vessels) in 44 OHT patients at a single center between 2004 and 2022. Baseline demographic data, angiographic characteristics, and clinical outcomes were analyzed. ResultsAt an average of 1.6 years after DES placement, follow-up angiography revealed in-stent restenosis in 36 (39 %) small vessel interventions and 11 (17 %) large vessel interventions (p = 0.003). Long term mortality did not differ between the groups (59 % vs 59 % at a median of 4.7 [IQR 2.4–7.8] years follow up). ConclusionDES restenosis rates are high in small vessel CAV. Additional studies specifically examining PCI in small vessel CAV as well as the potential role for newer treatment strategies for CAV are warranted.

Assessment of structural and functional changes in recipient's transplanted heart in the long-term postoperative period

Introduction. Heart transplantation is an effective way of treating patients with end-stage heart failure. Echocardiography allows for the assessment of the transplanted heart functions at all stages of follow-up. The clinical implementation of myocardial deformation imaging by the speckle tracking echocardiography has made it possible to detect subtle changes in myocardial contractility.Objective. To study the structural and functional state of myocardium in recipients in the long term after orthotopic heart transplantation.Material and methods. The study included 13 orthotopic heart transplant recipients (11 men and 2 women) at a mean age of 54.1±9.1 years who underwent orthotopic heart transplantation at the N.V. Sklifosovsky Research Institute for Emergency Medicine. The mean follow-up period after orthotopic heart transplantation was 6±0.7 years. All patients underwent transthoracic echocardiography according to the standard protocol, including determination of left ventricular myocardial deformation.Results. The median volume of the left atrium was 60 (53;76) ml, the left ventricular end-diastolic volume was 76 (70;90) ml, and the end-systolic volume was 30 (24;36) ml. The median ejection fraction of the left ventricle in the studied sample was 64 (57;66)%. The median interventricular septum thickness was 12 (11;13) mm, the left ventricular posterior wall thickness was 9 (8;10) mm. At the same time, the left ventricular myocardial mass and the left ventricular myocardial mass index were within the normal range and amounted to 140 (121;155) g and 65 (58;76) g/m2, respectively. The right heart chambers were not dilated, as the volume of the right atrium was 41 (40;56) ml, and the right ventricular enddiastolic dimension was 32 (30;33) mm. The right ventricular systolic function was unimpaired: the tricuspid annular plane systolic excursion was 18 (17;19) mm, and the right ventricular fractional area change was 46 (37.5;47.0)%. The calculated systolic pulmonary artery pressure was within the normal range 24 (21;28) mm Hg. The measurements of left ventricle global longitudinal and circumferential strains were -19.6 (-18.6;-21.2)% and -30.9 (-28.8;-32.0) %, respectively. Patients in the study sample showed a decrease in the left ventricular global function index to 25 (24.2;29.6)%. The diastolic dysfunction of a restrictive type was present in 10 patients (76.9%).Conclusions. In the long-term period after heart transplantation, the recipients were found to have a low left ventricular ejection fraction and a diastolic dysfunction of the restrictive type. Considering the normal values of left ventricular myocardial strains, we can assume that after 6 years post-orthotopic heart transplantation, the recipients have restored the heart adaptive functions, and a favorable outcome has been achieved. However, long-term monitoring is required.

ORTHOTOPIC HEART TRANSPLANTATION IN A PATIENT WITH A PERSISTENT LEFT SUPERIOR VENA CAVA: СASE REPORT

Introduction. Persistent left superior vena cava is the most common congenital venous abnormality of the thorax and has a prevalence of 0,3-0,5% among the general population. In most cases, the presence of a persistent left superior vena cava is asymptomatic and is usually a finding during surgery. Most often additional left superior vena cava coexists with superior vena cava in 80-90% cases. This feature is extremely important for choosing the optimal surgical tactics. Case Presentation. Patient Sh., 44 years old, was urgently hospitalized for orthotopic heart transplantation. From the anamnesis it is known that in 2017 the patient was diagnosed congenital heart disease: critical stenosis of the bicuspid aortic valve. However, the patient refused surgery at that moment. Conservative treatment was carried out. In 2022, the patient`s condition progressively worsened: severe exertional dyspnea, increased shortness of breath at rest, pitting edema in the lower extremities, interruptions in the work of the heart. The patient is diagnosed: dilated cardiomyopathy. The patient was included in the waiting list on the heart transplantation. In March 2023, patient underwent orthotopic heart transplantation with reconstruction a persistent left superior vena cava. A tubular conduit from the autopericardium with a diameter of 20 mm and a length of 5 cm was used for reconstruction. An anastomosis was formed between the distal part of the accessory left superior vena cava and the right atrial auricle. The patient was discharged without any complications and no signs of venous congestion in the upper body were detected during the follow-up examination after three months of observation. Conclusions. Our clinical observation demonstrates the feasibility of successful orthotopic heart transplantation using an autologous autopericardium graft in patients with a persistent left superior vena cava and shows encouraging immediate follow-up results.

Impact of institutional variables on centre performance in long-term survival after heart transplant.

The gold standard metric for centre-level performance in orthotopic heart transplantation (OHT) is 1-year post-OHT survival. However, it is unclear whether centre performance at 1 year is predictive of longer-term outcomes. This study evaluated factors impacting longer-term centre-level performance in OHT. Patients who underwent OHT in the USA between 2010 and 2021 were identified using the United Network of Organ Sharing data registry. The primary outcome was 5-year survival conditional on 1-year survival following OHT. Multivariable Cox proportional hazard models assessed the impact of centre-level 1-year survival rates on 5-year survival rates. Mixed-effect models were used to evaluate between-centre variability in outcomes. Centre-level risk-adjusted 5-year mortality conditional on 1-year survival was not associated with centre-level 1-year survival rates [hazard ratio: 0.99 (0.97-1.01, P = 0.198)]. Predictors of 5-year mortality conditional on 1-year survival included black recipient race, pre-OHT serum creatinine, diabetes and donor age. In mixed-effect modelling, there was substantial variability between centres in 5-year mortality rates conditional on 1-year survival, a finding that persisted after controlling for recipient, donor and institutional factors (P < 0.001). In a crude analysis using Kaplan-Meier, the 5-year survival conditional on 1-year survival was: low volume: 86.5%, intermediate volume: 87.5%, high volume: 86.7% (log-rank P = 0.52). These measured variables only accounted for 21.4% of the between-centre variability in 5-year mortality conditional on 1-year survival. Centre-level risk-adjusted 1-year outcomes do not correlate with outcomes in the 1- to 5-year period following OHT. Further research is needed to determine what unmeasured centre-level factors contribute to longer-term outcomes in OHT.

Long-term outcomes after cardiac transplantation in AL amyloidosis.

7572 Background: Systemic AL amyloidosis can lead to progressive multi-organ dysfunction including advanced heart failure which may require orthotopic heart transplantation (OHT). Hematologic outcomes of AL amyloidosis post-OHT are not well described. Methods: We report outcomes for patients with AL amyloidosis with cardiac involvement post-OHT from Nov 2008 - Jun 2023 at Vanderbilt University Medical Center. Hematologic response was graded per the ISA guidelines. Results: Fourteen pts underwent OHT of whom 13 had lambda light chain disease. Three (21%) pts had isolated AL amyloidosis (AL) without myeloma (MM), while 11 (79%) also had MM: 6 with bone marrow plasma cells (BMPCs) 10-19% (AL-PCMM-10) and 5 with BMPC ≥20% (AL-PCMM-20). One each with AL-PCMM-10 and AL-PCMM-20 had lytic lesions (AL-CRAB). Median age at OHT was 55.5 yrs (range 37-74). Median time from diagnosis to OHT was 18 mo (range 3-62). Five (35%) pts had biopsy-proven extracardiac amyloid involvement. Median difference in serum free light chains at first evaluation was 70 mg/dL (range 9-289). Three pts were Mayo 2012 stage II, 4 stage III, and 7 stage IV. Four pts had t(11;14) and 1 had 1q gain. Ten (71%) pts received triplet therapy (VCd or VRd) and 3 (21%) received quadruplet therapy (Dara-VCd or Isa-VCd) pre-OHT. Six (43%) pts had autologous stem cell transplantation (ASCT); 1 pre-OHT and 5 post-OHT. Of ASCT pts, 1 pt achieved CHR, 1 VGPR, 2 PR, and 1 had PD pre-ASCT. Pre-OHT, 4 pts achieved CHR, 6 VGPR, 3 PR, and 1 had PD. Eleven pts (79%) received maintenance therapy post-OHT (5 proteosome inhibitors, 6 anti-CD38). At last follow-up, 8 pts were in CHR, 3 in VGPR, 1 in PR, and 2 had PD. Four (29%) pts died post-OHT: 2 from AL recurrence (1 each with AL and AL-PCMM-10), 1 from cardiac allograft vasculopathy (AL-CRAB), and 1 from renal failure (AL-CRAB). Three (21%) pts had hematologic relapse 5, 21 and 48 mo post-OHT, and of those, 2 had recurrent cardiac amyloid in graft 37 and 71 mo post-OHT. Of those with hematologic relapse, 2 had AL-PCMM-10 and 1 had AL. One pt had post-transplant lymphoproliferative disorder 126 mo post-OHT, requiring treatment. Median follow-up and survival of the cohort post-OHT was 2.25 yrs (range 0.5-13.8). Conclusions: In this single-center analysis of pts undergoing OHT for cardiac AL amyloidosis, we demonstrate feasibility of excellent long-term outcomes, in predominantly high-risk AL pts where majority harbored high-risk disease with MM overlap, either by AL-CRAB or AL-BMPC. Induction alone led to ≥VGPR response in 10 (71%) pts pre-OHT and further therapy deepened this to 11 (79%) pts. Both AL-CRAB pts died during follow-up, highlighting the overall poor prognosis in this specific subgroup. In our cohort, non-relapse related mortality was seen in only 2 (14%) pts, demonstrating acceptable post-OHT risk and overall ability to achieve far improved outcomes in the high-risk cardiac AL amyloidosis patient population that otherwise suffers from rapid and universal mortality.