Prevalence and Prognostic Significance of Restriction Versus Systolic Dysfunction in Patients With Transthyretin and Light Chain Cardiac Amyloidosis
Restriction was the most common presenting phenotype, while preserved LV function represented approximately one-third. The rate of progression from preserved LV function towards restriction was high, whereas it was limited towards systolic dysfunction. Although patients with preserved LV function presented the best event-free survival, considering all-cause mortality and heart transplantation, compared with restriction or systolic dysfunction, these phenotypes are not independent predictors of this composite end point.
Authors
- Simone Longhi (ORCID: https://orcid.org/0000-0002-7512-1179)
- Beatrice Musumeci (ORCID: https://orcid.org/0000-0003-4872-7981)
- Camillo Autore (ORCID: https://orcid.org/0000-0002-6542-7342)
- Carlotta Mazzoni (ORCID: https://orcid.org/0000-0003-1330-7223)
- Marianna Eleonora Labate
- Federico Perfetto (ORCID: https://orcid.org/0000-0003-0276-8331)
- Alberto Ponziani
- Mattia Zampieri (ORCID: https://orcid.org/0000-0002-9339-068X)
- Giulia Biagioni (ORCID: https://orcid.org/0000-0002-9005-7945)
- Elena Biagini (ORCID: https://orcid.org/0000-0003-0295-0440)
- Giulia Saturi (ORCID: https://orcid.org/0000-0002-8154-3538)
- Luca Bordignon
- Margherita Zanoletti
- Annamaria Del Franco
- Marco Canepa
- Giacomo Tini
- E B Emanuele Barbato
- C Fumagalli
- Italo Porto
- Alessia Argirò
- Giuseppe Sena
- Iacopo Olivotto
- Marco Merlo
- Aldostefano Porcari
- Gianfranco Sinagra
- Vera Fico
- Francesco Cappelli
Institutions
- Università degli studi di Cassino e del Lazio Meridionale (IT)
- Amyloidosis Foundation (US)
- Meyer Children's Hospital (IT)
- Azienda Ospedaliero-Universitaria Careggi (IT)
- Azienda Ospedaliera Universitaria Senese (IT)
- ERN GUARD-Heart (NL)
- Ospedale Policlinico San Martino (IT)
- Azienda Sanitaria Universitaria Integrata di Trieste (IT)
- University College London (GB)
- University of Genoa (IT)
- Sapienza University of Rome (IT)
Publication Details
- Journal
- Circulation Heart Failure
- Published
- 2026-02-24
- DOI
- https://doi.org/10.1161/circheartfailure.125.012337
- Primary Topic
- Amyloidosis: Diagnosis, Treatment, Outcomes
- Type
- article
- Field-Weighted Citation Impact
- 0.00