Risk stratification in transthyretin-related cardiac amyloidosis
Transthyretin related cardiac amyloidosis (TTR-CA) is an infiltrative cardiomyopathy that cause heart failure with preserved ejection fraction, mainly in aging people. Due to the introduction of a non invasive diagnostic algorithm, this disease, previously considered to be rare, is increasingly reco...
| Main Authors: | , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Frontiers Media S.A.
2023-03-01
|
| Series: | Frontiers in Cardiovascular Medicine |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fcvm.2023.1151803/full |
| _version_ | 1797866787491020800 |
|---|---|
| author | Riccardo Scirpa Edoardo Cittadini Lorenzo Mazzocchi Giacomo Tini Giacomo Tini Matteo Sclafani Domitilla Russo Andrea Imperatrice Alessandro Tropea Camillo Autore Beatrice Musumeci |
| author_facet | Riccardo Scirpa Edoardo Cittadini Lorenzo Mazzocchi Giacomo Tini Giacomo Tini Matteo Sclafani Domitilla Russo Andrea Imperatrice Alessandro Tropea Camillo Autore Beatrice Musumeci |
| author_sort | Riccardo Scirpa |
| collection | DOAJ |
| description | Transthyretin related cardiac amyloidosis (TTR-CA) is an infiltrative cardiomyopathy that cause heart failure with preserved ejection fraction, mainly in aging people. Due to the introduction of a non invasive diagnostic algorithm, this disease, previously considered to be rare, is increasingly recognized. The natural history of TTR-CA includes two different stages: a presymptomatic and a symptomatic stage. Due to the availability of new disease-modifying therapies, the need to reach a diagnosis in the first stage has become impelling. While in variant TTR-CA an early identification of the disease may be obtained with a genetic screening in proband's relatives, in the wild-type form it represents a challenging issue. Once the diagnosis has been made, in order to identifying patients with a higher risk of cardiovascular events and death it is necessary to focus on risk stratification. Two prognostic scores have been proposed both based on biomarkers and laboratory findings. However, a multiparametric approach combining information from electrocardiogram, echocardiogram, cardiopulmonary exercise test and cardiac magnetic resonance may be warranted for a more comprehensive risk prediction. In this review, we aim at evaluating a step by step risk stratification, providing a clinical diagnostic and prognostic approach for the management of patients with TTR-CA. |
| first_indexed | 2024-04-09T23:29:50Z |
| format | Article |
| id | doaj.art-8e6e05ce9ff7456ab636bbc2560fc53e |
| institution | Directory Open Access Journal |
| issn | 2297-055X |
| language | English |
| last_indexed | 2024-04-09T23:29:50Z |
| publishDate | 2023-03-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Cardiovascular Medicine |
| spelling | doaj.art-8e6e05ce9ff7456ab636bbc2560fc53e2023-03-21T06:03:30ZengFrontiers Media S.A.Frontiers in Cardiovascular Medicine2297-055X2023-03-011010.3389/fcvm.2023.11518031151803Risk stratification in transthyretin-related cardiac amyloidosisRiccardo Scirpa0Edoardo Cittadini1Lorenzo Mazzocchi2Giacomo Tini3Giacomo Tini4Matteo Sclafani5Domitilla Russo6Andrea Imperatrice7Alessandro Tropea8Camillo Autore9Beatrice Musumeci10Division of Cardiology, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, ItalyDivision of Cardiology, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, ItalyDivision of Cardiology, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, ItalyDivision of Cardiology, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, ItalyDepartment of Cardiology, IRCCS San Raffaele Pisana, Rome, ItalyDivision of Cardiology, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, ItalyDivision of Cardiology, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, ItalyDivision of Cardiology, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, ItalyDivision of Cardiology, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, ItalySan Raffaele Cassino (FR), Cassino, ItalyDivision of Cardiology, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, ItalyTransthyretin related cardiac amyloidosis (TTR-CA) is an infiltrative cardiomyopathy that cause heart failure with preserved ejection fraction, mainly in aging people. Due to the introduction of a non invasive diagnostic algorithm, this disease, previously considered to be rare, is increasingly recognized. The natural history of TTR-CA includes two different stages: a presymptomatic and a symptomatic stage. Due to the availability of new disease-modifying therapies, the need to reach a diagnosis in the first stage has become impelling. While in variant TTR-CA an early identification of the disease may be obtained with a genetic screening in proband's relatives, in the wild-type form it represents a challenging issue. Once the diagnosis has been made, in order to identifying patients with a higher risk of cardiovascular events and death it is necessary to focus on risk stratification. Two prognostic scores have been proposed both based on biomarkers and laboratory findings. However, a multiparametric approach combining information from electrocardiogram, echocardiogram, cardiopulmonary exercise test and cardiac magnetic resonance may be warranted for a more comprehensive risk prediction. In this review, we aim at evaluating a step by step risk stratification, providing a clinical diagnostic and prognostic approach for the management of patients with TTR-CA.https://www.frontiersin.org/articles/10.3389/fcvm.2023.1151803/fullrisk stratificationcardiac amyloidosisnatural historytransthyretinheart failurearrhythmias |
| spellingShingle | Riccardo Scirpa Edoardo Cittadini Lorenzo Mazzocchi Giacomo Tini Giacomo Tini Matteo Sclafani Domitilla Russo Andrea Imperatrice Alessandro Tropea Camillo Autore Beatrice Musumeci Risk stratification in transthyretin-related cardiac amyloidosis Frontiers in Cardiovascular Medicine risk stratification cardiac amyloidosis natural history transthyretin heart failure arrhythmias |
| title | Risk stratification in transthyretin-related cardiac amyloidosis |
| title_full | Risk stratification in transthyretin-related cardiac amyloidosis |
| title_fullStr | Risk stratification in transthyretin-related cardiac amyloidosis |
| title_full_unstemmed | Risk stratification in transthyretin-related cardiac amyloidosis |
| title_short | Risk stratification in transthyretin-related cardiac amyloidosis |
| title_sort | risk stratification in transthyretin related cardiac amyloidosis |
| topic | risk stratification cardiac amyloidosis natural history transthyretin heart failure arrhythmias |
| url | https://www.frontiersin.org/articles/10.3389/fcvm.2023.1151803/full |
| work_keys_str_mv | AT riccardoscirpa riskstratificationintransthyretinrelatedcardiacamyloidosis AT edoardocittadini riskstratificationintransthyretinrelatedcardiacamyloidosis AT lorenzomazzocchi riskstratificationintransthyretinrelatedcardiacamyloidosis AT giacomotini riskstratificationintransthyretinrelatedcardiacamyloidosis AT giacomotini riskstratificationintransthyretinrelatedcardiacamyloidosis AT matteosclafani riskstratificationintransthyretinrelatedcardiacamyloidosis AT domitillarusso riskstratificationintransthyretinrelatedcardiacamyloidosis AT andreaimperatrice riskstratificationintransthyretinrelatedcardiacamyloidosis AT alessandrotropea riskstratificationintransthyretinrelatedcardiacamyloidosis AT camilloautore riskstratificationintransthyretinrelatedcardiacamyloidosis AT beatricemusumeci riskstratificationintransthyretinrelatedcardiacamyloidosis |