Right ventricular strain in Anderson-Fabry disease.

Echocardiography Fabry Disease / diagnostic imaging Heart Ventricles / diagnostic imaging Humans Ventricular Dysfunction, Right / diagnostic imaging Ventricular Function, Right
Anderson-Fabry disease Cardiomyopathy Right ventricle Speckle tracking

Journal

International journal of cardiology
ISSN: 1874-1754
Titre abrégé: Int J Cardiol
Pays: Netherlands
ID NLM: 8200291

Informations de publication

Date de publication:
01 05 2021
Historique:
received: 14 09 2020
revised: 08 02 2021
accepted: 11 02 2021
pubmed: 19 2 2021
medline: 29 5 2021
entrez: 18 2 2021
Statut: ppublish

Résumé

2D speckle tracking echocardiography (2DSTE) is superior to standard echocardiography in the assessment of subtle right ventricle (RV) systolic dysfunction. In this study we aimed to: 1) test the hypothesis that 2DSTE may unveil subtle RV systolic dysfunction in patients with Fabry disease; 2) investigate whether the physiologic difference between the 3-segment (RV-FWS) and the 6-segment (RV-GLS) RV strain (∆RV strain) is preserved in Fabry patients. Standard echocardiography and 2DSTE were performed in 49 Fabry patients and 49 age- and sex-matched healthy controls. Fabry patients were divided in two groups according to the presence/absence of left ventricular hypertrophy (LVH+: left ventricular wall thickness > 12 mm, 49% of total Fabry patients). RV systolic function assessed by standard echocardiography was normal in the majority of Fabry patients (92%) while RV-GLS and RV-FWS were impaired in about 40%. RV-GLS and RV-FWS were significantly worse in patients LVH+ vs LVH- and vs controls (RV-GLS: LVH+ vs LVH-: -18.4 ± -4.3% vs -23.8 ± -3.1% p<0.001; LVH+ vs controls: -18.4 ± -4.3% vs -23.9 ± -2.8% p<0.001; RV-FWS: LVH+ vs LVH-: -21.8 ± -5.3% vs -26.7 ± -3.8% p = 0.002, LVH+ vs controls -21.8 ± -5.3% vs -26.8 ± -3.9% p<0.001). No difference was found between LVH- patients and controls in both RV-GLS (p = 0.65) and RV-FWS (p = 0.79). ∆RV strain was similar among the groups. In Fabry cardiomyopathy impaired RV-GLS and RV-FWS is a common finding, while RV strain is preserved in Fabry patients without overt cardiac involvement. The physiologic difference between RV-FWS and RV-GLS is maintained in Fabry patients, regardless of the presence of cardiomyopathy.

Sections du résumé

BACKGROUND
2D speckle tracking echocardiography (2DSTE) is superior to standard echocardiography in the assessment of subtle right ventricle (RV) systolic dysfunction. In this study we aimed to: 1) test the hypothesis that 2DSTE may unveil subtle RV systolic dysfunction in patients with Fabry disease; 2) investigate whether the physiologic difference between the 3-segment (RV-FWS) and the 6-segment (RV-GLS) RV strain (∆RV strain) is preserved in Fabry patients.
METHODS AND RESULTS
Standard echocardiography and 2DSTE were performed in 49 Fabry patients and 49 age- and sex-matched healthy controls. Fabry patients were divided in two groups according to the presence/absence of left ventricular hypertrophy (LVH+: left ventricular wall thickness > 12 mm, 49% of total Fabry patients). RV systolic function assessed by standard echocardiography was normal in the majority of Fabry patients (92%) while RV-GLS and RV-FWS were impaired in about 40%. RV-GLS and RV-FWS were significantly worse in patients LVH+ vs LVH- and vs controls (RV-GLS: LVH+ vs LVH-: -18.4 ± -4.3% vs -23.8 ± -3.1% p<0.001; LVH+ vs controls: -18.4 ± -4.3% vs -23.9 ± -2.8% p<0.001; RV-FWS: LVH+ vs LVH-: -21.8 ± -5.3% vs -26.7 ± -3.8% p = 0.002, LVH+ vs controls -21.8 ± -5.3% vs -26.8 ± -3.9% p<0.001). No difference was found between LVH- patients and controls in both RV-GLS (p = 0.65) and RV-FWS (p = 0.79). ∆RV strain was similar among the groups.
CONCLUSIONS
In Fabry cardiomyopathy impaired RV-GLS and RV-FWS is a common finding, while RV strain is preserved in Fabry patients without overt cardiac involvement. The physiologic difference between RV-FWS and RV-GLS is maintained in Fabry patients, regardless of the presence of cardiomyopathy.

Identifiants

DOI: 10.1016/j.ijcard.2021.02.038 PMID: 33600844
pubmed: 33600844
pii: S0167-5273(21)00291-6
doi: 10.1016/j.ijcard.2021.02.038
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

84-90

Informations de copyright

Copyright © 2021 Elsevier B.V. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of Competing Interest The authors report no relationships that could be construed as a conflict of interest.

Auteurs

Rosa Lillo (R)

Department of Cardiovascular and Thoracic Sciences, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; Catholic University of the Sacred Heart, Rome, Italy.

Francesca Graziani (F)

Department of Cardiovascular and Thoracic Sciences, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy. Electronic address: francesca.graziani@policlinicogemelli.it.

Elena Panaioli (E)

Department of Cardiovascular and Thoracic Sciences, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; Catholic University of the Sacred Heart, Rome, Italy.

Erica Mencarelli (E)

Department of Cardiovascular and Thoracic Sciences, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; Catholic University of the Sacred Heart, Rome, Italy.

Maurizio Pieroni (M)

Cardiovascular Department, San Donato Hospital, Arezzo, Italy.

Antonia Camporeale (A)

Multimodality Cardiac Imaging Section, IRCCS Policlinico San Donato, San Donato Milanese, Milan, Italy.

Raffaele Manna (R)

Catholic University of the Sacred Heart, Rome, Italy; Rare diseases and Periodic Fever Research Center, Department of Internal Medicine, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.

Ludovico Luca Sicignano (LL)

Rare diseases and Periodic Fever Research Center, Department of Internal Medicine, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.

Elena Verrecchia (E)

Rare diseases and Periodic Fever Research Center, Department of Internal Medicine, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.

Antonella Lombardo (A)

Department of Cardiovascular and Thoracic Sciences, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; Catholic University of the Sacred Heart, Rome, Italy.

Gaetano Antonio Lanza (GA)

Department of Cardiovascular and Thoracic Sciences, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; Catholic University of the Sacred Heart, Rome, Italy.

Filippo Crea (F)

Department of Cardiovascular and Thoracic Sciences, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; Catholic University of the Sacred Heart, Rome, Italy.

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Classifications MeSH

questionsmedicales.fr › Diagnostic › Techniques et procédures diagnostiques › Techniques de diagnostic cardiovasculaire › Tests de la fonction cardiaque › Échocardiographie › Right ventricular strain in Anderson-Fabry disease.
questionsmedicales.fr › Maladies métaboliques et nutritionnelles › Maladies métaboliques › Erreurs innées du métabolisme › Maladies lysosomiales › Maladies neurologiques de surcharge lysosomiale › Sphingolipidoses › Maladie de Fabry › Right ventricular strain in Anderson-Fabry disease.
questionsmedicales.fr › Système cardiovasculaire › Coeur › Ventricules cardiaques › Right ventricular strain in Anderson-Fabry disease.
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Right ventricular strain in Anderson-Fabry disease.
questionsmedicales.fr › Maladies cardiovasculaires › Cardiopathies › Dysfonction ventriculaire › Dysfonction ventriculaire droite › Right ventricular strain in Anderson-Fabry disease.
questionsmedicales.fr › Phénomènes physiologiques respiratoires et circulatoires › Phénomènes physiologiques cardiovasculaires › Fonction ventriculaire › Fonction ventriculaire droite › Right ventricular strain in Anderson-Fabry disease.