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.

Lillo, R., Graziani, F., Panaioli, E., Mencarelli, E., Pieroni, M., Camporeale, A., Manna, R., Sicignano, L. L., Verrecchia, E., Lombardo, A., Lanza, G. A., Crea, F., Right ventricular strain in Anderson-Fabry disease, <<INTERNATIONAL JOURNAL OF CARDIOLOGY>>, 2021; (330): 84-90. [doi:10.1016/j.ijcard.2021.02.038] [http://hdl.handle.net/10807/174501]

Right ventricular strain in Anderson-Fabry disease

Graziani F.;Manna R.;Sicignano L. L.;Verrecchia E.;Lombardo A.;Lanza G. A.;Crea F.
2021

Abstract

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.
Inglese
Lillo, R., Graziani, F., Panaioli, E., Mencarelli, E., Pieroni, M., Camporeale, A., Manna, R., Sicignano, L. L., Verrecchia, E., Lombardo, A., Lanza, G. A., Crea, F., Right ventricular strain in Anderson-Fabry disease, <>, 2021; (330): 84-90. [doi:10.1016/j.ijcard.2021.02.038] [http://hdl.handle.net/10807/174501]
File in questo prodotto:
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/10807/174501
Citazioni
  • ???jsp.display-item.citation.pmc??? 0
  • Scopus 3
  • ???jsp.display-item.citation.isi??? ND
social impact