Publications by authors named "Evan Kransdorf"

Background: Clinical genetic evaluation of dilated cardiomyopathy (DCM) is implemented variably or not at all. Identifying needs and barriers to genetic evaluations will enable strategies to enhance precision medicine care.

Methods: An online survey was conducted in June 2024 among cardiologist investigators of the DCM Consortium from US advanced heart failure/transplant (HF/TX) programs to collect demographics, training, program characteristics, genetic evaluation practices for DCM, and implementation needs.

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Background: Cardiac sarcoidosis is an uncommon but potentially devastating manifestation of sarcoidosis, which is a multisystem inflammatory granulomatous disease. Although corticosteroids are the mainstay of treatment, given the number of complications associated with their long-term use, there is increasing interest in the use of steroid-sparing agents. Recent basic and translational studies have suggested a role for the mechanistic target of rapamycin (mTOR) pathway in cardiac sarcoidosis.

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The number of candidates on the waiting list for heart transplantation (HT) continues to far outweigh the number of available organs, and the donor heart nonuse rate in the United States remains significantly higher than that of other regions such as Europe. Although predicting outcomes in HT remains challenging, our overall understanding of the factors that play a role in post-HT outcomes continues to grow. We observe that many donor risk factors that are deemed "high-risk" do not necessarily always adversely affect post-HT outcomes, but are in fact nuanced and interact with other donor and recipient risk factors.

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Background: Primary graft dysfunction (PGD) remains the leading cause of 30-day mortality post-heart transplantation (HTx). HTx recipients experiencing severe PGD have been found to have high levels of circulating proteins associated with PGD occurrence and post-HTx survival. Whether treating these patients with therapeutic plasma exchange (TPE) can attenuate ongoing immunological and inflammatory processes and improve post-transplant outcomes has not been well-investigated.

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Article Synopsis
  • - The study focuses on developing an automated system to quantify [18F]-fluorodeoxyglucose (FDG) PET activity in diagnosing cardiac sarcoidosis using deep learning for segmenting cardiac chambers from CT scans.
  • - The analysis included 69 patients, revealing that the cardiometabolic activity (CMA) showed the best predictive accuracy for cardiac sarcoidosis, followed by volume of inflammation (VOI) and target to background ratio (TBR).
  • - The findings indicate that this automated method provides rapid, objective measurements of cardiac inflammation, showing high sensitivity and specificity for diagnosing cardiac sarcoidosis.
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Background: Microvascular dysfunction after heart transplantation leads to restrictive cardiac allograft physiology (RCP), which is classified as severe coronary allograft vasculopathy (CAV); however, the prognosis of RCP remains unclear. Therefore, in this study, we aimed to elucidate the prognosis of RCP in comparison with that of severe angiographic CAV.

Methods: We assessed 116 patients with severe CAV who underwent heart transplantation between 2004 and 2023.

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Background: The application of posttransplant predictive models is limited by their poor statistical performance. Neglecting the dynamic evolution of demographics and medical practice over time may be a key issue.

Objectives: Our objective was to develop and validate era-specific predictive models to assess whether these models could improve risk stratification compared to non-era-specific models.

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Background: Metabolic remodeling is a hallmark of the failing heart. Oncometabolic stress during cancer increases the activity and abundance of the ATP-dependent citrate lyase (ACL, ), which promotes histone acetylation and cardiac adaptation. ACL is critical for the de novo synthesis of lipids, but how these metabolic alterations contribute to cardiac structural and functional changes remains unclear.

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The Banff Heart Concurrent Session, held as part of the 16th Banff Foundation for Allograft Pathology Conference at Banff, Alberta, Canada, on September 21, 2022, focused on 2 major topics: non-human leukocyte antigen (HLA) antibodies and mixed rejection. Each topic was addressed in a multidisciplinary fashion with clinical, immunological, and pathology perspectives and future developments and prospectives. Following the Banff organization model and principles, the collective aim of the speakers on each topic was to • Determine current knowledge gaps in heart transplant pathology • Identify limitations of current pathology classification systems • Discuss next steps in addressing gaps and refining classification system.

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Article Synopsis
  • Bortezomib, a proteasome inhibitor, was evaluated alongside plasmapheresis as a method to desensitize highly sensitized heart transplant patients (cPRA > 50%) at a single center from 2010 to 2021, with a focus on their outcomes compared to non-sensitized patients.
  • Despite some changes in antibody levels, the desensitization therapy did not lead to a statistically significant decrease in overall sensitization (mean cPRA remained high), though there was a marginally better response in class I antibodies compared to class II.
  • While one-year survival rates were similar between desensitized patients and non-sensitized controls (95.4% vs. 92
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  • Primary graft dysfunction (PGD) is a significant complication after heart transplantation, and the study examines how pretransplant human leukocyte antigen (HLA) sensitization, indicated by the calculated panel reactive antibody (cPRA) value, influences the risk of PGD in heart transplant recipients.!* -
  • The research involved 596 adult heart transplant patients, evaluating their peak cPRA-LS levels, donor-specific antibodies, and other factors, finding that higher levels of cPRA-LS, particularly for HLA-A, correlated with increased severity of PGD.!* -
  • The findings suggest that HLA sensitization, along with other factors like donor age and recipient medication use, should be considered in pre-trans
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Background: Sensitization to human leukocyte antigens (HLA) is a persistent problem in heart transplant (HT) candidates. We sought to characterize the anti-HLA antibody and circulating B cell repertoire in a cohort of highly sensitized HT candidates.

Methods: We assessed immunoglobulin G (IgG) and immunoglobulin M (IgM) anti-HLA antibodies using Luminex single antigen bead assays in a cohort of 11 highly sensitized (HS; calculated panel reactive antibody ≥ 90%) and 3 mildly sensitized (MS) candidates.

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Article Synopsis
  • The study investigates the genetic factors associated with advanced dilated cardiomyopathy (DCM), particularly focusing on rare genetic variants related to patients requiring devices like left ventricular assist devices (LVAD) or heart transplants (HT).
  • Researchers analyzed data from a diverse group of 1,198 patients enrolled in a precision medicine study, classifying the severity of DCM based on treatment type and assessing genetic variants in 36 related genes.
  • Findings revealed that 26.2% of patients with advanced DCM (LVAD/HT) had pathogenic genetic variants, significantly more than those with only an implantable cardioverter defibrillator (15.9%) or neither treatment (15.0%), indicating a strong genetic link to
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  • - The study investigates the genetic differences in dilated cardiomyopathy (DCM) among Black, Hispanic, and White patients, noting that Black patients face higher familial risk and worse health outcomes compared to White patients, despite most existing genetic data coming from the latter group.
  • - Researchers conducted a cross-sectional study involving over 1,000 patients across various US heart failure centers, focusing on genetic variants in 36 DCM-related genes, classified based on their significance and clinical impact.
  • - Findings revealed that Black patients displayed a lower percentage of clinically actionable genetic variants compared to White patients (8.2% vs 25.5%), particularly in the TTN gene, highlighting potential disparities in genetic influences on DCM severity among different ances
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  • Cardiovascular screening for first-degree relatives (FDRs) of patients with dilated cardiomyopathy (DCM) was studied to determine its effectiveness among those without known familial DCM and across different demographics.
  • The study involved 1,365 adult FDRs who underwent echocardiograms and ECGs, revealing that 14.1% were newly diagnosed with DCM, left ventricular systolic dysfunction (LVSD), or left ventricular enlargement (LVE).
  • The results showed higher diagnosis rates in older FDRs and those with hypertension or obesity, indicating that screening is beneficial for all FDRs, regardless of race or ethnicity.
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Allosensitization represents a major barrier to heart transplantation. We previously reported favorable 1-year outcomes of complement inhibition at transplant in highly sensitized recipients. We now report a longer follow-up.

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We present 3 heart transplant recipients who developed hypertrophic cardiomyopathy years after transplantation. In all 3 cases, the diagnosis was initially made based on echocardiography and confirmed using cardiac magnetic resonance imaging. ().

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Article Synopsis
  • The DCM Precision Medicine Study aimed to improve the communication of genetic disease risk among first-degree relatives of individuals diagnosed with dilated cardiomyopathy (DCM), focusing on enhancing participation in clinical screenings.
  • A booklet was created to assist probands in conveying the importance of cardiovascular screening to their at-risk family members, and its effectiveness was evaluated in a large controlled trial.
  • Results showed that first-degree relatives of probands who received the booklet had a higher screening completion rate (19.5%) compared to those who did not receive it (16.0%), indicating the booklet's success in motivating screenings.
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Background: Transthyretin amyloidosis (TTR) is increasingly implicated as an aetiology of advanced cardiomyopathy. Typically, both genetic variant (TTRv) and wild-type (TTRwt) amyloidosis present with a restrictive phenotype. We present a series of three patients who were found to have cardiac amyloidosis on explant following heart transplant (HT) who had atypical, non-restrictive phenotypes.

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