Evidence map›Paper›PMID 40452225›Full record

Observational studyEuropean heart journal2025

Cardiac transthyretin amyloidosis treatment improves outcomes after aortic valve replacement for severe stenosis.

Christian Nitsche, Stephan Dobner, Hannah R Rosenblum, Kush P Patel, Simone Longhi, Ali Yilmaz, Marco Merlo, Maria Papathanasiou, Jan Griffin, Marish I F J Oerlemans and 23 more

Registry-linked trialAbstract readMulticenter StudyObservational Study
In one paragraph

Observational study in European heart journal, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT06129331 (Aortic Stenosis and Cardiac Amyloidosis), which is not on this map. Cited by 13 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
13citing papers in PubMed, 1 pooled it
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT06129331 unknown statusnot on this map

Aortic Stenosis and Cardiac Amyloidosis: A Pragmatic, Streamlined International

TypeobservationalSponsorMedical University of ViennaRan2023 to 2024Enrolled300ConditionsCardiac Amyloidosis, Aortic StenosisArmsAmyloid-specific treatment, No amyloid-specific treatment
3 · Its place in the literature

Who cites it

13 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
  2. Cardiac amyloidosis: what every interventional cardiologist should know.Cardiovascular intervention and therapeutics · 2026
    Review
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

33 authors.

Christian NitscheDivision of Cardiology, Department of Internal Medicine II, Medical University of Vienna, Waehringerguertel 18-20, 1090 Vienna, Austria.ORCID 0000-0002-0141-7639
Stephan DobnerDepartment of Cardiology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
Hannah R RosenblumDivision of Cardiology, Columbia University Medical Center, New York Presbyterian Hospital, New York City, New York, USA.
Kush P PatelBarts Heart Centre, KGV Building, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK.
Simone LonghiCardiology Unit, Cardiac Thoracic and Vascular Department, IRCCS Azienda Ospedaliero, Universitaria di Bologna, Bologna, Italy.ORCID 0000-0002-7512-1179
Ali YilmazDivision of Cardiovascular Imaging, Department of Cardiology I, University Hospital Münster, Münster, Germany.
Marco MerloEuropean Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart.ORCID 0000-0002-1022-3131
Maria PapathanasiouDepartment of Cardiology and Vascular Medicine, West German Heart and Vascular Center, University Hospital Essen, Essen, Germany.
Jan GriffinDivision of Cardiology, Department of Medicine, Medical University of South Carolina, Charleston, SC, USA.
Marish I F J OerlemansEuropean Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart.
Francisco GamaCardiology Department, Hospital de Santa Cruz, Unidade Local de Saúde Lisboa Ocidental, Lisbon, Portugal.
Ashraf HamdanDepartment of Cardiology, Rabin Medical Center, Petah Tikva, and Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel.ORCID 0000-0002-3347-825X
Andrew D KelionDepartment of Cardiology, John Radcliffe Hospital, Oxford, UK.
Andreas SchusterDepartment of Cardiology and Pneumology, University Medical Center Göttingen, Georg-August University, Göttingen, Germany.
Sigita GlaveckaitéClinic of Cardiovascular Diseases, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, Vilnius, Lithuania.
Nuriye AkyolDivision of Cardiovascular Imaging, Department of Cardiology I, University Hospital Münster, Münster, Germany.
Aldostefano PorcariEuropean Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart.
Lara SchlenderDepartment of Cardiology, University Hospital Frankfurt, Goethe University, Frankfurt, Germany.
Teresa CapovillaDepartment of Cardiothoracovascular, Azienda Sanitaria Universitaria Integrata Giuliana Isontina (ASUGI), University of Trieste, Trieste, Italy.
Maximilian AutherithDivision of Cardiology, Department of Internal Medicine II, Medical University of Vienna, Waehringerguertel 18-20, 1090 Vienna, Austria.
Laurenz HauptmannDivision of Cardiology, Department of Internal Medicine II, Medical University of Vienna, Waehringerguertel 18-20, 1090 Vienna, Austria.
Kseniya HalavinaDivision of Cardiology, Department of Internal Medicine II, Medical University of Vienna, Waehringerguertel 18-20, 1090 Vienna, Austria.
João L CavalcanteCardiovascular Imaging Research Center and Core Lab, Minneapolis Heart Institute Foundation, Minneapolis, MN, USA.
Marianna FontanaNational Amyloidosis Centre, Division of Medicine, University College London, Royal Free Hospital, London, UK.
Paul R ScullyBarts Heart Centre, KGV Building, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK.
James C MoonBarts Heart Centre, KGV Building, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK.
Julia MascherbauerDivision of Cardiology, Department of Internal Medicine II, Medical University of Vienna, Waehringerguertel 18-20, 1090 Vienna, Austria.
Robin RistlCenter for Medical Data Science, Medical University of Vienna, Vienna, Austria.
Elena BiaginiCardiology Unit, Cardiac Thoracic and Vascular Department, IRCCS Azienda Ospedaliero, Universitaria di Bologna, Bologna, Italy.ORCID 0000-0003-0295-0440
Stefan StorteckyDepartment of Cardiology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.ORCID 0000-0002-5076-0177
Matthew S MaurerDivision of Cardiology, Columbia University Medical Center, New York Presbyterian Hospital, New York City, New York, USA.
Thomas A TreibelBarts Heart Centre, KGV Building, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK.
AS-Amyloidosis Consortium

Funding

Pfizer Global ID#87865391
6 · The paper itself

Abstract

BACKGROUND AND

aimsConcomitant aortic stenosis (AS) and transthyretin-associated cardiac amyloidosis (ATTR-CA) is an increasingly recognized cause of structural heart failure. Aortic valve replacement (AVR) improves prognosis in this population, but the efficacy of ATTR-specific medication remains unclear. This study aimed to investigate the prognostic implications of ATTR-specific medication in patients with dual AS-CA.

methodsThis is a multicenter, international, transatlantic registry of patients with a concomitant pathology of significant AS (moderate/severe) and ATTR-CA (ClinicalTrials.gov identifier: NCT06129331). AS severity was diagnosed by transthoracic echocardiography and ATTR-CA by myocardial uptake on bone scintigraphy and/or positive endomyocardial biopsy in the absence of monoclonal proteins. Mortality [all-cause and cardiovascular (CV)] and hospitalisation for heart failure (HHF) served as clinical endpoints. Outcomes were compared with a control cohort of confirmed lone AS receiving AVR matched for EuroSCORE II.

resultsOf 226 patients with dual pathology (85 ± 6 years, 80.4% male) identified in 16 centres across 10 countries, AS was severe in 196 (86.7%), and moderate in 30 (13.3%). Valve treatment strategies were transcatheter/surgical AVR in 71.7%/3.5%, balloon angioplasty in 1.3%, and conservative management in 23.5%. Seventy-three patients (32.3%) were prescribed, and 69 patients (30.5%) eventually received ATTR-specific medication (99% tafamidis) and were younger, with lower EuroSCORE II, a higher portion of moderate AS, but higher interventricular septum thickness and more severely impaired left ventricular function compared with patients without ATTR medication. After 3.6 ± 1.7 years, 112 (49.6%) had died [CV death: 89 (79.5%)] and 58 (25.7%) experienced HHF. ATTR-specific medication was independently associated with lower all-cause [weighted hazard ratio (HR) 0.40, 95% confidence interval (CI) 0.24-0.68] and CV mortality (weighted HR 0.47, 95% CI 0.27-0.83) but not HHF. AVR improved survival in the overall (HR 0.60, 95% CI 0.39-0.93) and severe AS cohort (HR 0.42, 95% CI 0.26-0.70). Patients who received both ATTR-specific medication and AVR had the most favourable prognosis, comparable to a control cohort with lone AS undergoing AVR.

conclusionsATTR-specific treatment and AVR both result in significant survival benefit in dual pathology AS and ATTR-CA. Results should be interpreted in the context of the non-randomized study setting and differences in patient characteristics.

Indexed as

Amyloid Neuropathies, FamilialAortic Valve StenosisCardiomyopathiesHeart Valve Prosthesis ImplantationAgedAged, 80 and overEchocardiographyFemaleHeart FailureHumansMalePrognosisRegistriesTreatment OutcomeAmyloidSAVRTafamidisTAVR

Identifiers

PMID40452225
PMCPMC12634099

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Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.