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  • Damy, ThibaudReferral Center for Cardiac Amyloidosis, Department of Cardiology, Mondor Amyloidosis Network, GRC Amyloid Res. Institute, Clin. Invest. Center, 006, DHU A-TVB INSERM U955 All at CHU Henri Mondor, UPEC, Créteil, France (författare)

Transthyretin cardiac amyloidosis in continental Western Europe : An insight through the Transthyretin Amyloidosis Outcomes Survey (THAOS)

  • Artikel/kapitelEngelska2019

Förlag, utgivningsår, omfång ...

  • 2019-04-01
  • Oxford University Press,2019
  • electronicrdacarrier

Nummerbeteckningar

  • LIBRIS-ID:oai:DiVA.org:umu-203485
  • https://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-203485URI
  • https://doi.org/10.1093/eurheartj/ehz173DOI

Kompletterande språkuppgifter

  • Språk:engelska
  • Sammanfattning på:engelska

Ingår i deldatabas

Klassifikation

  • Ämneskategori:ref swepub-contenttype
  • Ämneskategori:art swepub-publicationtype

Anmärkningar

  • Aims: Transthyretin amyloidosis (ATTR amyloidosis) is a heterogeneous disorder with cardiac, neurologic, and mixed phenotypes. We describe the phenotypic and genotypic profiles of this disease in continental Western Europe as it appears from the Transthyretin Amyloidosis Survey (THAOS).Methods and results: THAOS is an ongoing, worldwide, longitudinal, observational survey established to study differences in presentation, diagnosis, and natural history in ATTR amyloidosis subjects. At data cut-off, 1411 symptomatic subjects from nine continental Western European countries were enrolled in THAOS [1286 hereditary (ATTRm) amyloidosis; 125 wild-type ATTR (ATTRwt) amyloidosis]. Genotypes and phenotypes varied notably by country. Four mutations (Val122Ile, Leu111Met, Thr60Ala, and Ile68Leu), and ATTRwt, were associated with a mainly cardiac phenotype showing symmetric left ventricular (LV) hypertrophy, normal diastolic LV dimensions and volume, and mildly depressed LV ejection fraction (LVEF). Morphologic and functional abnormalities on echocardiogram were significantly more severe in subjects with cardiac (n = 210), compared with a mixed (n = 298), phenotype: higher median (Q1-Q3) interventricular septal thickness [18 (16-21) vs. 16 (13-20) mm; P = 0.0006]; and more frequent incidence of LVEF <50% (38.1 vs. 17.5%; P = 0.0008). Subjects with cardiac mutations or ATTRwt (or cardiac or mixed phenotype) had a lower survival rate than subjects in other genotype (or the neurologic phenotype) categories (P < 0.0001, for both).Conclusion: ATTR amyloidosis genotypes and phenotypes are highly heterogeneous in continental Western Europe. A geographic map of the different disease profiles and awareness that a subset of subjects have a dominant cardiac phenotype, mimicking hypertrophic cardiomyopathy, at presentation can facilitate the clinical recognition of this underdiagnosed disease.Trial registration: ClinicalTrials.gov: NCT00628745.

Ämnesord och genrebeteckningar

Biuppslag (personer, institutioner, konferenser, titlar ...)

  • Kristen, Arnt VDepartment of Cardiology, Amyloidosis Center, Heidelberg University, Heidelberg, Germany (författare)
  • Suhr, Ole B.Umeå universitet,Institutionen för folkhälsa och klinisk medicin(Swepub:umu)olsu0002 (författare)
  • Maurer, Mathew SColumbia University College of Physicians and Surgeons, NY, New York, United States (författare)
  • Planté-Bordeneuve, ViolaineDepartment of Neurology, Mondor Amyloid Network, Inserm U955-Team10, East Paris University Hospital Henri-Mondor, France (författare)
  • Yu, Ching-RayPfizer Inc, NY, New York, United States (författare)
  • Ong, Moh-LimPfizer Inc, NY, New York, United States (författare)
  • Coelho, TeresaDepartment of Neurosciences, Hospital de Santo António, Centro Hospitalar Do Porto, Porto, Portugal (författare)
  • Rapezzi, ClaudioDepartment of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy (författare)
  • Referral Center for Cardiac Amyloidosis, Department of Cardiology, Mondor Amyloidosis Network, GRC Amyloid Res. Institute, Clin. Invest. Center, 006, DHU A-TVB INSERM U955 All at CHU Henri Mondor, UPEC, Créteil, FranceDepartment of Cardiology, Amyloidosis Center, Heidelberg University, Heidelberg, Germany (creator_code:org_t)

Sammanhörande titlar

  • Ingår i:European Heart Journal: Oxford University Press43:5, s. 391-4000195-668X1522-9645

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