Clinical Research in Cardiology最新文献

{"title":"Outpatient diuretic intensification: a simple prognostic marker in cardiac transthyretin amyloidosis.","authors":"Richard J Nies, Svenja Ney, Jasper F Nies, Katharina Seuthe, Lukas Klösges, Monique Brüwer, Stephan Nienaber, Sascha Macherey-Meyer, Matthieu Schäfer, Roman Pfister","doi":"10.1007/s00392-025-02617-4","DOIUrl":"10.1007/s00392-025-02617-4","url":null,"abstract":"<p><strong>Background: </strong>Currently, simple clinical parameters indicating disease progression are lacking in patients with transthyretin amyloid cardiomyopathy (ATTR-CM). This study aimed to evaluate the prognostic value of outpatient diuretic intensification (ODI) in ATTR-CM patients.</p><p><strong>Methods: </strong>This retrospective study examined ATTR-CM patients at a tertiary care center between August 1, 2020, and June 30, 2023. ODI was defined as any loop diuretic increase within 6 months after baseline visit, and its impact on all-cause mortality and hospitalization for heart failure (HF) was analyzed.</p><p><strong>Results: </strong>Altogether, 182 patients were included (median age 80 [76; 84] years; 88% male), and 25% experienced ODI (median increase 10 [10; 40] mg furosemide equivalent). Independent predictors of ODI were higher baseline New York Heart Association (NYHA) class and polyneuropathy. Both any ODI and the magnitude of furosemide equivalent increase were significantly associated with mortality and HF hospitalization during a median follow-up of 17 months. After adjusting for baseline NYHA class and National Amyloidosis Centre stage, significantly increased risk of all-cause mortality (hazard ratio [HR] 2.38, 95% confidence interval [CI] 1.03-5.53; p = 0.043) and HF hospitalization (HR 3.27, 95% CI 1.41-7.60; p = 0.006) persisted in patients with ODI. Its prognostic value was similar in strata of age, ATTR subtype, previous cardiac decompensation, biomarkers, left ventricular ejection fraction, six-minute walk distance, and tafamidis treatment.</p><p><strong>Conclusion: </strong>ODI occurred in one in four ATTR-CM patients within 6 months and was associated with more severe baseline amyloid organ manifestations. ODI and the magnitude of diuretic dose increase provide easily assessable clinical markers of disease progression in patient monitoring.</p>","PeriodicalId":10474,"journal":{"name":"Clinical Research in Cardiology","volume":" ","pages":"752-762"},"PeriodicalIF":3.7,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13083326/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143540339","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical and economic burden associated with symptomatic and asymptomatic obstructive hypertrophic cardiomyopathy in Germany.","authors":"Farbod Sedaghat-Hamedani, Carla L Zema, Michael Schultze, Tarcyane B Garcia, Nils Kossack, Julia Borchert, Ervant J Maksabedian Hernandez, Yue Zhong, Tobias Bluhmki, Taryn Krause, Johanna Schmoelders, Benjamin Meder","doi":"10.1007/s00392-025-02776-4","DOIUrl":"10.1007/s00392-025-02776-4","url":null,"abstract":"<p><strong>Background: </strong>The clinical and economic burden of obstructive hypertrophic cardiomyopathy (HCM) in Germany for symptomatic versus asymptomatic patients has not been comprehensively assessed.</p><p><strong>Methods: </strong>This retrospective observational study analyzed nationally representative WIG2 Benchmark Database data, identifying adults diagnosed with obstructive HCM from 2012 to 2018. Study index was the first date when a participant met eligibility criteria. An algorithm based on coded symptoms and pharmacological treatments was used to divide patients into symptomatic and asymptomatic subgroups. Annual prevalence, patient characteristics, outcomes, resource utilization, and costs were assessed during follow-up.</p><p><strong>Results: </strong>Overall, 1141 patients were included (649 symptomatic patients and 492 asymptomatic patients during the 1-year pre-index period [baseline]). In total, 1042 patients had symptomatic disease at some point during follow-up. Annual obstructive HCM prevalence increased between 2011 and 2019 and was higher for symptomatic than asymptomatic patients. Compared with asymptomatic patients, symptomatic patients: were older (mean age: 62.8 vs. 55.5 years), were less likely to be male (59% vs. 67%), and had higher Charlson Comorbidity Index (mean: 2.82 vs. 1.69) at baseline; had higher rates of all-cause mortality (0.05 vs. 0.02 per 100 patient-years) and cardiovascular events (mean follow-up: 4.7 years); had more outpatient visits (13.3 vs. 10.8 per patient-year [PPY]), inpatient visits (0.7 vs. 0.5 PPY), and longer mean length of stay (8.7 vs. 7.4 days); and had higher mean costs PPY for outpatient, inpatient, and pharmacy services (total: €5975 vs. €4399).</p><p><strong>Conclusions: </strong>Patients with symptomatic obstructive HCM experienced greater clinical and economic burden than asymptomatic patients in Germany.</p>","PeriodicalId":10474,"journal":{"name":"Clinical Research in Cardiology","volume":" ","pages":"852-861"},"PeriodicalIF":3.7,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13083345/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145376192","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Real-world experience in initiation of treatment with the selective cardiomyosin inhibitor mavacamten in an outpatient clinic cohort during the 12-week titration period.","authors":"Finn Becker, Julia Novotny, Nadine Jansen, Sebastian Clauß, Florian Möller-Dyrna, Birge Specht, Madeleine Orban, Steffen Massberg, Stefan Kääb, Daniel Reichart","doi":"10.1007/s00392-024-02544-w","DOIUrl":"10.1007/s00392-024-02544-w","url":null,"abstract":"<p><strong>Introduction: </strong>Lately, mavacamten emerged as a new therapeutic option for symptomatic patients with obstructive hypertrophic cardiomyopathy (oHCM). Clinical trials revealed reduction of serum biomarkers, and left ventricular outflow tract (LVOT) obstruction, as well as an improvement in clinical symptoms and exercise capacity. Nevertheless, clinical experience and manageability of patients in a real-world setting is still lacking.</p><p><strong>Material and methods: </strong>22 patients with symptomatic oHCM (54.5% male, age 58.5 ± 16.2 years) and elevated LVOT gradients were started on mavacamten between March 2023 and June 2024. All patients were New York Heart Association (NYHA) class II or higher. Seven patients were excluded from primary analysis due to comedication with Angiotensin-converting-enzyme-inhibitors or Angiotensin-II receptor blockers. Cardiac imaging, laboratory work-up and clinical evaluation were assessed at three visits during the 12 weeks initiation phase; Dosing of mavacamten was adjusted according to manufacturer's recommendations.</p><p><strong>Results: </strong>At 12 weeks, the majority of patients described a significant improvement of their quality of life. Work-up at 12 weeks revealed a significant reduction of serum biomarkers and LVOT gradients. In four patients, mavacamten needed to be temporarily paused due to clinical complaints or transient left ventricular ejection fraction deterioration below 50% with subsequent full recovery.</p><p><strong>Conclusion: </strong>We provide first insights into the usage of mavacamten in oHCM patients during the titration period in a real-world setting. Clinical findings are in line with previous clinical trials. In accordance with current recommendations, we highlight the need for standardized follow-up of patients on mavacamten treatment.</p>","PeriodicalId":10474,"journal":{"name":"Clinical Research in Cardiology","volume":" ","pages":"725-731"},"PeriodicalIF":3.7,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13083493/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142388635","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Temporal trends, patient characteristics and hospital volume outcomes after transcoronary ablation of septal hypertrophy and septal myectomy for treatment of obstructive hypertrophic cardiomyopathy.","authors":"Peter Moritz Becher, Fabian J Brunner, Jessica Weimann, Marko Remmel, Benedikt Schrage, Monica Patten, Christian Detter, Hermann Reichenspurner, Paulus Kirchhof, Stefan Blankenberg, Moritz Seiffert","doi":"10.1007/s00392-025-02773-7","DOIUrl":"10.1007/s00392-025-02773-7","url":null,"abstract":"<p><strong>Background: </strong>Hypertrophic obstructive cardiomyopathy (HOCM) is a hereditary cardiac disease associated with poor prognosis. Data on septal reduction therapy (SRT), including transcoronary ablation of septal hypertrophy (TASH) and surgical myectomy (SM), are limited. We aimed to evaluate temporal trends, patient characteristics, in-hospital outcomes, and the influence of institutional procedural volumes in a nationwide cohort.</p><p><strong>Methods: </strong>We used nationwide administrative data from the German Federal Bureau of Statistics to identify HOCM patients undergoing TASH or SM between 2006 and 2019. Primary outcomes were in-hospital mortality and pacemaker implantation. Multivariable logistic regression assessed associations with institutional procedure volume.</p><p><strong>Results: </strong>Among 8,514 SRT procedures, 5,293 (62.2%) were TASH and 3,221 (37.8%) SM. Annual volumes increased for both procedures between 2006 and 2019. SM patients were older (67.4 vs. 60.2 years), more often male, and had a higher comorbidity burden. Of all centers, 49.3% performed ≤ 20 SM per year, and 76.3% performed ≤ 20 TASH per year. Mitral surgery occurred in 36.1% of SM cases. In-hospital mortality was higher for SM than TASH (6.9% vs. 0.8%), while pacemaker implantation was more frequent with TASH (19.3% vs. 13.1%). Risk of in-hospital mortality after SM was higher in low-volume centers than in large centers (adjusted odds ratio 2.64; 95% confidence interval, 1.49-4.66).</p><p><strong>Conclusion: </strong>SM was performed in older, more comorbid patiens and frequently included concomitant valve surgery. Higher in-hospital mortality at low-volume centers may partially reflect surgical complexity or case mix rather than volume alone. Our findings underscore the need for individualized referral strategies to experienced centers.</p>","PeriodicalId":10474,"journal":{"name":"Clinical Research in Cardiology","volume":" ","pages":"842-851"},"PeriodicalIF":3.7,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13083484/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145250189","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mechanisms of damage and therapies for cardiac amyloidosis: a role for inflammation?","authors":"Ilaria Anna Bellofatto, Panagiota Efstathia Nikolaou, Ioanna Andreadou, Marco Canepa, Federico Carbone, Alessandra Ghigo, Gerd Heusch, Petra Kleinbongard, Christoph Maack, Bruno K Podesser, Kimon Stamatelopoulos, Konstantinos Stellos, Gemma Vilahur, Fabrizio Montecucco, Luca Liberale","doi":"10.1007/s00392-024-02522-2","DOIUrl":"10.1007/s00392-024-02522-2","url":null,"abstract":"<p><p>The term cardiac amyloidosis (CA) refers to the accumulation of extracellular amyloid deposits in the heart because of different conditions often affecting multiple organs including brain, kidney and liver. Notably, cardiac involvement significantly impacts prognosis of amyloidosis, with cardiac biomarkers playing a pivotal role in prognostic stratification. Therapeutic management poses a challenge due to limited response to conventional heart failure therapies, necessitating targeted approaches aimed at preventing, halting or reversing amyloid deposition. Mechanisms underlying organ damage in CA are multifactorial, involving proteotoxicity, oxidative stress, and mechanical interference. While the role of inflammation in CA remains incompletely understood, emerging evidence suggests its potential contribution to disease progression as well as its utility as a therapeutic target. This review reports on the cardiac involvement in systemic amyloidosis, its prognostic role and how to assess it. Current and emerging therapies will be critically discussed underscoring the need for further efforts aiming at elucidating CA pathophysiology. The emerging evidence suggesting the contribution of inflammation to disease progression and its prognostic role will also be reviewed possibly offering insights into novel therapeutic avenues for CA.</p>","PeriodicalId":10474,"journal":{"name":"Clinical Research in Cardiology","volume":" ","pages":"685-699"},"PeriodicalIF":3.7,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13083513/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142016541","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Endomyocardial biopsy in patients with myocarditis-still justified in the CMR era? A single-centre experience.","authors":"Katharina Seuthe, Roman Pfister, Lenhard Pennig, Ute Mons, Karin Klingel, Henrik Ten Freyhaus","doi":"10.1007/s00392-024-02574-4","DOIUrl":"10.1007/s00392-024-02574-4","url":null,"abstract":"<p><strong>Background: </strong>In the past decades, cardiovascular magnetic resonance (CMR) was established as a non-invasive tool supporting the diagnosis of myocarditis and there is often reluctance in performing EMB due to potentially severe complications. We sought to identify patient subgroups that could still benefit from EMB in the CMR era.</p><p><strong>Methods: </strong>Data of patients presenting with myocarditis between 01/2016 and 06/2023 were analysed according to patient risks. Prespecified risk factors were (i) left ventricular ejection fraction (LVEF) ≤ 30%; (ii) severe arrhythmias; or (iii) pre-existing autoimmune disease. Furthermore, the subgroup of recurrent myocarditis cases was analysed separately.</p><p><strong>Results: </strong>A total of 137 patients (35.5 ± 14.8 years, 80.3% male) were included. 26/137 patients had a documented LVEF ≤ 30%, 13/137 a LVEF > 30% with at least one other risk factor and 98/137 a LVEF > 30% without risk factors. EMB was performed in 21/26 patients with LVEF ≤ 30% (80.8%), in 7/13 patients with LVEF > 30% and risk factors (53.8%) and in 16/98 (16%) patients without risk factors. EMB led to the initiation of immunosuppressive therapy in 11/28 patients with risk factors (39.3%) and in none of the patients without risk factors (0/16, 0%, p = 0.003). With respect to the subgroup of patients presenting with recurrent myocarditis (n = 10), no specific therapy was initiated.</p><p><strong>Conclusions: </strong>Due to a high therapeutic yield for initiation of immunosuppressive therapy in non-infectious myocarditis, performing EMB should be considered in all high-risk patients. In patients without clinical risk factors including cases of recurrent or relapsing myocarditis no specific therapy was initiated.</p>","PeriodicalId":10474,"journal":{"name":"Clinical Research in Cardiology","volume":" ","pages":"743-751"},"PeriodicalIF":3.7,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13083329/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142680981","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Incremental prognostic utility of congestion markers in cardiac transthyretin amyloidosis.","authors":"Sandra Michaela Ihne-Schubert, Caroline Morbach, Vladimir Cejka, Maximilian Johannes Steinhardt, Aikaterini Papagianni, Stefan Frantz, Hermann Einsele, Thomas Wehler, Klaus Martin Kortüm, Claudia Sommer, Stefan Störk, Torben Schubert, Andreas Geier","doi":"10.1007/s00392-024-02512-4","DOIUrl":"10.1007/s00392-024-02512-4","url":null,"abstract":"<p><strong>Background/aims: </strong>Congestion is prognostically relevant in cardiac transthyretin amyloidosis (ATTR-CA), but whether congestion has an incremental prognostic value beyond the well-established, congestion-sensitive NT-proBNP is unknown. Therefore, we aimed to comparatively evaluate the prognostic utility of several congestion surrogates over NT-proBNP.</p><p><strong>Methods: </strong>We estimated hazard ratios by Cox proportional hazards regressions with time-varying covariates from a panel data set of the local amyloidosis cohort study AmyKoS. Different models were compared by using chi(χ)²-statistics measuring overall model significance.</p><p><strong>Results/conclusion: </strong>131 ATTR-CA patients (wild-type 84.0%, hereditary 6.9%, without genetic testing 9.2%; median age 78.7 (quartiles 73.3, 82.1) years; 85.5% male) with 566 observations across a median follow-up of 38.2 (30.6; 48.2) months were analyzed. 83.2% received disease-modifying treatment; 20.6% participated concurrently in placebo-controlled gene silencer trials. Information on congestion improved biomarker-driven risk stratification and identified patients at the highest risk. Echocardiographic congestion markers performed better than clinical findings and daily diuretic use/dosage. Relevant adjusters were daily diuretic dosage, disease-modifying treatment, eGFR, and right atrial volume. NT-proBNP and the tricuspid regurgitation peak velocity (tr-v_max) provided an easy-to-use stratification with overall model performance similar to NAC and Mayo staging systems. Further analyses are necessary for validation and to identify the optimal cut points of the congestion markers.</p>","PeriodicalId":10474,"journal":{"name":"Clinical Research in Cardiology","volume":" ","pages":"714-724"},"PeriodicalIF":3.7,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141893061","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"CMR-based assessment of long-term effects of tafamidis in patients with cardiac transthyretin amyloidosis.","authors":"Alexandru Zlibut, Michael Bietenbeck, Nuriye Akyol, Volker Vehof, Redouane Bouras, Khuraman Isgandarova, Claudia Meier, Maria Theofanidou, Philipp Stalling, Ali Yilmaz","doi":"10.1007/s00392-025-02691-8","DOIUrl":"10.1007/s00392-025-02691-8","url":null,"abstract":"<p><strong>Objectives and background: </strong>The aim of the study was to evaluate the long-term effects of tafamidis on cardiac disease progression beyond 12 months of treatment by performing (among others) serial multi-parametric cardiovascular magnetic resonance (CMR) studies in patients with transthyretin (ATTR) cardiac amyloidosis (CA) cardiomyopathy (ATTR-CM).</p><p><strong>Methods: </strong>Patients with confirmed ATTR-CM (N = 56) were divided into two groups: in the larger group A (N = 39; 95% male), treatment with tafamidis 61 mg once daily was initiated after the first CMR study, whereas group B (N = 17; 76% male) comprised ATTR-CM patients who did not receive tafamidis. The observational follow-up period lasted 27 ± 6 months. During this period, patients underwent two multi-parametric CMR studies at our institution as part of a routine clinical observation pipeline.</p><p><strong>Results: </strong>Clinical symptoms assessed by the NYHA class showed a slight, however, significant increase in both groups. NT-proBNP levels substantially increased in both groups at follow-up, however, with a significantly higher increase in the tafamidis-naïve group B (p = 0.014). LV systolic function, defined by LV-EF and 3D global longitudinal peak strain, significantly worsened in both groups at follow-up (54% to 48% p < 0.001 vs 56% to 46%, p < 0.001; -7.4 to -5.3, p < 0.001 vs -8.8 to -4.8, p < 0.001). However, the tafamidis-naïve group B experienced a substantially higher impairment of both parameters when compared to group A (∆p = 0.008 and ∆p = 0.003, respectively). LV wall thickness considerably increased in both groups at follow-up, however, with a significantly higher increase in the tafamidis-naïve group B (from 18.2 mm to 21.1 mm at follow-up, p < 0.001) compared to the tafamidis-treated group A (from 18.5 mm to 19.2 mm, p = 0.012; ∆p < 0.001). Both global native T1 and global ECV values were significantly elevated in both groups-at baseline and at follow-up-with a significant increase in both groups during follow-up. However, a substantially higher increase in global ECV was observed in the tafamidis-naïve group B compared to the tafamidis-treated group A (group A: 51% to 57%, p < 0.001; group B: 50% to 67%, p < 0.001; ∆p < 0.001).</p><p><strong>Conclusion: </strong>Substantial worsening of clinical symptoms, serum biomarkers, and imaging parameters occurred in both tafamidis-treated and tafamidis-naïve ATTR-CM patients within a follow-up period of approximately 2 years. However, the \"extent of worsening\" is significantly lower in the tafamidis-treated compared to the tafamidis-naïve patients.</p>","PeriodicalId":10474,"journal":{"name":"Clinical Research in Cardiology","volume":" ","pages":"785-794"},"PeriodicalIF":3.7,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13083460/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145376282","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Current treatment decisions in cardiac transthyretin amyloidosis: a multicentre analysis.","authors":"Daniel Lavall, Katharina Knoll, Sebastian Spethmann, Katrin Hahn, Gina Barzen, Ephraim B Winzer, Stefanie Jellinghaus, Lisa K Schöner, Monique Tröbs, Dominik Kauffmann, Nora Donhauser, Lars Michel, Julia Vogel, Tienush Rassaf, Maria Papathanasiou, Lara S Schlender, David M Leistner, Birgit Aßmus, Bernhard Unsöld, Larissa Bühner, Fabian Aus dem Siepen, Eva Hofmann, Christian Nagel, Ingrid Kindermann, Angela Zimmer, Roman Pfister, Matthieu Schäfer, Natascha Majunke, Irina Müller-Kozarez, Heribert Schunkert, Patrick Fuchs, Stéphanie K Schwarting, Yuliyan Metodiev, Selen Alieva, Ali Yilmaz, Alexandru Zlibut, Julian Mustroph, Maria Tafelmeier, Thomas Krammer, Stefan Störk, Aikaterini Papagianni, Maximilian J Steinhardt, Vladimir Cejka, Caroline Morbach, Teresa Trenkwalder","doi":"10.1007/s00392-026-02848-z","DOIUrl":"10.1007/s00392-026-02848-z","url":null,"abstract":"<p><strong>Background: </strong>The efficacy of transthyretin stabilisation in cardiac transthyretin amyloidosis (ATTR-CM) has been demonstrated in a clinical trial setting, but little is known about treatment decision-making in the real world. Particularly, initiating or discontinuing specific therapy is challenging in early and advanced disease. We evaluated current decision pathways for tafamidis in ATTR-CM.</p><p><strong>Methods: </strong>This multicentre retrospective study included consecutive patients from 15 tertiary centres in Germany in whom ATTR-CM was newly diagnosed between January and June 2024, as well as patients, in whom tafamidis treatment was discontinued during this period.</p><p><strong>Results: </strong>Out of 516 patients with newly established ATTR-CM included in the present analysis, tafamidis was initiated in 414 (80%). The 99 patients without recommendation for tafamidis were older (p = 0.002), had a higher amyloidosis disease stage (NAC score), worse NYHA class (both p < 0.001), and higher NT-proBNP levels (p = 0.002) compared to those with tafamidis initiation. During the same observation period, tafamidis therapy was discontinued in 28 ATTR-CM patients. Treatment decisions were mainly taken by an interdisciplinary board (73% of centres). The most frequent reasons for not starting or stopping tafamidis were 'frailty' (47%/61%) and 'life expectancy or comorbidity' (38%/43%), respectively.</p><p><strong>Conclusions: </strong>In this multicentre analysis, treatment with tafamidis was initiated in about 80% of patients with newly diagnosed ATTR-CM. In most centres, treatment decisions were made by an interdisciplinary board, and the reasons for treatment decisions were similar across centres. Due to the lack of consensus criteria, our data may help to standardise decision pathways for ATTR-CM.</p>","PeriodicalId":10474,"journal":{"name":"Clinical Research in Cardiology","volume":" ","pages":"862-874"},"PeriodicalIF":3.7,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13083537/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146046167","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Editorial comment regarding article: Incremental prognostic utility of congestion markers in cardiac transthyretin amyloidosis.","authors":"Nicolas Verheyen, Peter P Rainer","doi":"10.1007/s00392-024-02528-w","DOIUrl":"10.1007/s00392-024-02528-w","url":null,"abstract":"","PeriodicalId":10474,"journal":{"name":"Clinical Research in Cardiology","volume":" ","pages":"892-893"},"PeriodicalIF":3.7,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142105068","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}