Exercise does not influence development of phenotype in PLN p.(Arg14del) cardiomyopathy

Background Endurance and frequent exercise are associated with earlier onset of arrhythmogenic right ventricular cardiomyopathy (ARVC) and ventricular arrhythmias (VA) in desmosomal gene variant carriers. Individuals with the pathogenic c.40_42del; p.(Arg14del) variant in the PLN gene are frequently...
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Autor*in:	van Lint, Freyja H. M. [verfasserIn] Hassanzada, Fahima Verstraelen, Tom E. Wang, Weijia Bosman, Laurens P. van der Zwaag, Paul A. Oomen, Toon Calkins, Hugh Murray, Brittney Tichnell, Crystal Beuren, Thais M. A. Asselbergs, Folkert W. Houweling, Arjan van den Berg, Maarten P. Wilde, Arthur A. M. James, Cynthia A. van Tintelen, J. Peter

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2023

Schlagwörter:	Arrhythmogenic right ventricular cardiomyopathy Dilated cardiomyopathy Genetics Exercise Penetrance

Anmerkung:	© The Author(s) 2023

Übergeordnetes Werk:	Enthalten in: Netherlands heart journal - [S.l.] : PubMed Central, 2001, 31(2023), 7-8 vom: 20. Juli, Seite 291-299
Übergeordnetes Werk:	volume:31 ; year:2023 ; number:7-8 ; day:20 ; month:07 ; pages:291-299

Links:	Volltext

DOI / URN:	10.1007/s12471-023-01800-4

Katalog-ID:	SPR052629775

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100	1		\|a van Lint, Freyja H. M. \|e verfasserin \|4 aut
245	1	0	\|a Exercise does not influence development of phenotype in PLN p.(Arg14del) cardiomyopathy
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520			\|a Background Endurance and frequent exercise are associated with earlier onset of arrhythmogenic right ventricular cardiomyopathy (ARVC) and ventricular arrhythmias (VA) in desmosomal gene variant carriers. Individuals with the pathogenic c.40_42del; p.(Arg14del) variant in the PLN gene are frequently diagnosed with ARVC or dilated cardiomyopathy (DCM). The aim of this study was to evaluate the effect of exercise in PLN p.(Arg14del) carriers. Methods In total, 207 adult PLN p.(Arg14del) carriers (39.1% male; mean age 53 ± 15 years) were interviewed on their regular physical activity since the age of 10 years. The association of exercise with diagnosis of ARVC, DCM, sustained VA and hospitalisation for heart failure (HF) was studied. Results Individuals participated in regular physical activities with a median of 1661 metabolic equivalent of task (MET) hours per year (31.9 MET-hours per week) until clinical presentation. The 50% most and least active individuals had a similar frequency of sustained VA (18.3% vs 18.4%; p = 0.974) and hospitalisation for HF (9.6% vs 8.7%; p = 0.827). There was no relationship between exercise and survival free from (incident) sustained VA (p = 0.65), hospitalisation for HF (p = 0.81), diagnosis of ARVC (p = 0.67) or DCM (p = 0.39) during follow-up. In multivariate analyses, exercise was not associated with sustained VA or HF hospitalisation during follow-up in this relatively not-active cohort. Conclusion There was no association between the amount of exercise and the susceptibility to develop ARVC, DCM, VA or HF in PLN p.(Arg14del) carriers. This suggested unaffected PLN p.(Arg14del) carriers can safely perform mild-moderate exercise, in contrast to desmosomal variant carriers and ARVC patients.
650		4	\|a Arrhythmogenic right ventricular cardiomyopathy \|7 (dpeaa)DE-He213
650		4	\|a Dilated cardiomyopathy \|7 (dpeaa)DE-He213
650		4	\|a Genetics \|7 (dpeaa)DE-He213
650		4	\|a Exercise \|7 (dpeaa)DE-He213
650		4	\|a Penetrance \|7 (dpeaa)DE-He213
700	1		\|a Hassanzada, Fahima \|4 aut
700	1		\|a Verstraelen, Tom E. \|4 aut
700	1		\|a Wang, Weijia \|4 aut
700	1		\|a Bosman, Laurens P. \|4 aut
700	1		\|a van der Zwaag, Paul A. \|4 aut
700	1		\|a Oomen, Toon \|4 aut
700	1		\|a Calkins, Hugh \|4 aut
700	1		\|a Murray, Brittney \|4 aut
700	1		\|a Tichnell, Crystal \|4 aut
700	1		\|a Beuren, Thais M. A. \|4 aut
700	1		\|a Asselbergs, Folkert W. \|4 aut
700	1		\|a Houweling, Arjan \|4 aut
700	1		\|a van den Berg, Maarten P. \|4 aut
700	1		\|a Wilde, Arthur A. M. \|4 aut
700	1		\|a James, Cynthia A. \|4 aut
700	1		\|a van Tintelen, J. Peter \|4 aut
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912			\|a GBV_ILN_39
912			\|a GBV_ILN_40
912			\|a GBV_ILN_60
912			\|a GBV_ILN_62
912			\|a GBV_ILN_63
912			\|a GBV_ILN_65
912			\|a GBV_ILN_69
912			\|a GBV_ILN_73
912			\|a GBV_ILN_74
912			\|a GBV_ILN_95
912			\|a GBV_ILN_105
912			\|a GBV_ILN_110
912			\|a GBV_ILN_151
912			\|a GBV_ILN_161
912			\|a GBV_ILN_170
912			\|a GBV_ILN_206
912			\|a GBV_ILN_213
912			\|a GBV_ILN_230
912			\|a GBV_ILN_285
912			\|a GBV_ILN_293
912			\|a GBV_ILN_602
912			\|a GBV_ILN_2014
912			\|a GBV_ILN_4012
912			\|a GBV_ILN_4037
912			\|a GBV_ILN_4112
912			\|a GBV_ILN_4125
912			\|a GBV_ILN_4126
912			\|a GBV_ILN_4249
912			\|a GBV_ILN_4305
912			\|a GBV_ILN_4306
912			\|a GBV_ILN_4307
912			\|a GBV_ILN_4313
912			\|a GBV_ILN_4322
912			\|a GBV_ILN_4323
912			\|a GBV_ILN_4324
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Indexfelder

author_variant	l f h m v lfhm lfhmv f h fh t e v te tev w w ww l p b lp lpb d z p a v dzpa dzpav t o to h c hc b m bm c t ct t m a b tma tmab f w a fw fwa a h ah d b m p v dbmp dbmpv a a m w aam aamw c a j ca caj t j p v tjp tjpv
matchkey_str	article:18766250:2023----::xrieosoifuneeeomnopeoyenlpr
hierarchy_sort_str	2023
publishDate	2023
allfields	10.1007/s12471-023-01800-4 doi (DE-627)SPR052629775 (SPR)s12471-023-01800-4-e DE-627 ger DE-627 rakwb eng van Lint, Freyja H. M. verfasserin aut Exercise does not influence development of phenotype in PLN p.(Arg14del) cardiomyopathy 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2023 Background Endurance and frequent exercise are associated with earlier onset of arrhythmogenic right ventricular cardiomyopathy (ARVC) and ventricular arrhythmias (VA) in desmosomal gene variant carriers. Individuals with the pathogenic c.40_42del; p.(Arg14del) variant in the PLN gene are frequently diagnosed with ARVC or dilated cardiomyopathy (DCM). The aim of this study was to evaluate the effect of exercise in PLN p.(Arg14del) carriers. Methods In total, 207 adult PLN p.(Arg14del) carriers (39.1% male; mean age 53 ± 15 years) were interviewed on their regular physical activity since the age of 10 years. The association of exercise with diagnosis of ARVC, DCM, sustained VA and hospitalisation for heart failure (HF) was studied. Results Individuals participated in regular physical activities with a median of 1661 metabolic equivalent of task (MET) hours per year (31.9 MET-hours per week) until clinical presentation. The 50% most and least active individuals had a similar frequency of sustained VA (18.3% vs 18.4%; p = 0.974) and hospitalisation for HF (9.6% vs 8.7%; p = 0.827). There was no relationship between exercise and survival free from (incident) sustained VA (p = 0.65), hospitalisation for HF (p = 0.81), diagnosis of ARVC (p = 0.67) or DCM (p = 0.39) during follow-up. In multivariate analyses, exercise was not associated with sustained VA or HF hospitalisation during follow-up in this relatively not-active cohort. Conclusion There was no association between the amount of exercise and the susceptibility to develop ARVC, DCM, VA or HF in PLN p.(Arg14del) carriers. This suggested unaffected PLN p.(Arg14del) carriers can safely perform mild-moderate exercise, in contrast to desmosomal variant carriers and ARVC patients. Arrhythmogenic right ventricular cardiomyopathy (dpeaa)DE-He213 Dilated cardiomyopathy (dpeaa)DE-He213 Genetics (dpeaa)DE-He213 Exercise (dpeaa)DE-He213 Penetrance (dpeaa)DE-He213 Hassanzada, Fahima aut Verstraelen, Tom E. aut Wang, Weijia aut Bosman, Laurens P. aut van der Zwaag, Paul A. aut Oomen, Toon aut Calkins, Hugh aut Murray, Brittney aut Tichnell, Crystal aut Beuren, Thais M. A. aut Asselbergs, Folkert W. aut Houweling, Arjan aut van den Berg, Maarten P. aut Wilde, Arthur A. M. aut James, Cynthia A. aut van Tintelen, J. Peter aut Enthalten in Netherlands heart journal [S.l.] : PubMed Central, 2001 31(2023), 7-8 vom: 20. Juli, Seite 291-299 (DE-627)53467299X (DE-600)2365465-X 1876-6250 nnns volume:31 year:2023 number:7-8 day:20 month:07 pages:291-299 https://dx.doi.org/10.1007/s12471-023-01800-4 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 31 2023 7-8 20 07 291-299
spelling	10.1007/s12471-023-01800-4 doi (DE-627)SPR052629775 (SPR)s12471-023-01800-4-e DE-627 ger DE-627 rakwb eng van Lint, Freyja H. M. verfasserin aut Exercise does not influence development of phenotype in PLN p.(Arg14del) cardiomyopathy 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2023 Background Endurance and frequent exercise are associated with earlier onset of arrhythmogenic right ventricular cardiomyopathy (ARVC) and ventricular arrhythmias (VA) in desmosomal gene variant carriers. Individuals with the pathogenic c.40_42del; p.(Arg14del) variant in the PLN gene are frequently diagnosed with ARVC or dilated cardiomyopathy (DCM). The aim of this study was to evaluate the effect of exercise in PLN p.(Arg14del) carriers. Methods In total, 207 adult PLN p.(Arg14del) carriers (39.1% male; mean age 53 ± 15 years) were interviewed on their regular physical activity since the age of 10 years. The association of exercise with diagnosis of ARVC, DCM, sustained VA and hospitalisation for heart failure (HF) was studied. Results Individuals participated in regular physical activities with a median of 1661 metabolic equivalent of task (MET) hours per year (31.9 MET-hours per week) until clinical presentation. The 50% most and least active individuals had a similar frequency of sustained VA (18.3% vs 18.4%; p = 0.974) and hospitalisation for HF (9.6% vs 8.7%; p = 0.827). There was no relationship between exercise and survival free from (incident) sustained VA (p = 0.65), hospitalisation for HF (p = 0.81), diagnosis of ARVC (p = 0.67) or DCM (p = 0.39) during follow-up. In multivariate analyses, exercise was not associated with sustained VA or HF hospitalisation during follow-up in this relatively not-active cohort. Conclusion There was no association between the amount of exercise and the susceptibility to develop ARVC, DCM, VA or HF in PLN p.(Arg14del) carriers. This suggested unaffected PLN p.(Arg14del) carriers can safely perform mild-moderate exercise, in contrast to desmosomal variant carriers and ARVC patients. Arrhythmogenic right ventricular cardiomyopathy (dpeaa)DE-He213 Dilated cardiomyopathy (dpeaa)DE-He213 Genetics (dpeaa)DE-He213 Exercise (dpeaa)DE-He213 Penetrance (dpeaa)DE-He213 Hassanzada, Fahima aut Verstraelen, Tom E. aut Wang, Weijia aut Bosman, Laurens P. aut van der Zwaag, Paul A. aut Oomen, Toon aut Calkins, Hugh aut Murray, Brittney aut Tichnell, Crystal aut Beuren, Thais M. A. aut Asselbergs, Folkert W. aut Houweling, Arjan aut van den Berg, Maarten P. aut Wilde, Arthur A. M. aut James, Cynthia A. aut van Tintelen, J. Peter aut Enthalten in Netherlands heart journal [S.l.] : PubMed Central, 2001 31(2023), 7-8 vom: 20. Juli, Seite 291-299 (DE-627)53467299X (DE-600)2365465-X 1876-6250 nnns volume:31 year:2023 number:7-8 day:20 month:07 pages:291-299 https://dx.doi.org/10.1007/s12471-023-01800-4 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 31 2023 7-8 20 07 291-299
allfields_unstemmed	10.1007/s12471-023-01800-4 doi (DE-627)SPR052629775 (SPR)s12471-023-01800-4-e DE-627 ger DE-627 rakwb eng van Lint, Freyja H. M. verfasserin aut Exercise does not influence development of phenotype in PLN p.(Arg14del) cardiomyopathy 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2023 Background Endurance and frequent exercise are associated with earlier onset of arrhythmogenic right ventricular cardiomyopathy (ARVC) and ventricular arrhythmias (VA) in desmosomal gene variant carriers. Individuals with the pathogenic c.40_42del; p.(Arg14del) variant in the PLN gene are frequently diagnosed with ARVC or dilated cardiomyopathy (DCM). The aim of this study was to evaluate the effect of exercise in PLN p.(Arg14del) carriers. Methods In total, 207 adult PLN p.(Arg14del) carriers (39.1% male; mean age 53 ± 15 years) were interviewed on their regular physical activity since the age of 10 years. The association of exercise with diagnosis of ARVC, DCM, sustained VA and hospitalisation for heart failure (HF) was studied. Results Individuals participated in regular physical activities with a median of 1661 metabolic equivalent of task (MET) hours per year (31.9 MET-hours per week) until clinical presentation. The 50% most and least active individuals had a similar frequency of sustained VA (18.3% vs 18.4%; p = 0.974) and hospitalisation for HF (9.6% vs 8.7%; p = 0.827). There was no relationship between exercise and survival free from (incident) sustained VA (p = 0.65), hospitalisation for HF (p = 0.81), diagnosis of ARVC (p = 0.67) or DCM (p = 0.39) during follow-up. In multivariate analyses, exercise was not associated with sustained VA or HF hospitalisation during follow-up in this relatively not-active cohort. Conclusion There was no association between the amount of exercise and the susceptibility to develop ARVC, DCM, VA or HF in PLN p.(Arg14del) carriers. This suggested unaffected PLN p.(Arg14del) carriers can safely perform mild-moderate exercise, in contrast to desmosomal variant carriers and ARVC patients. Arrhythmogenic right ventricular cardiomyopathy (dpeaa)DE-He213 Dilated cardiomyopathy (dpeaa)DE-He213 Genetics (dpeaa)DE-He213 Exercise (dpeaa)DE-He213 Penetrance (dpeaa)DE-He213 Hassanzada, Fahima aut Verstraelen, Tom E. aut Wang, Weijia aut Bosman, Laurens P. aut van der Zwaag, Paul A. aut Oomen, Toon aut Calkins, Hugh aut Murray, Brittney aut Tichnell, Crystal aut Beuren, Thais M. A. aut Asselbergs, Folkert W. aut Houweling, Arjan aut van den Berg, Maarten P. aut Wilde, Arthur A. M. aut James, Cynthia A. aut van Tintelen, J. Peter aut Enthalten in Netherlands heart journal [S.l.] : PubMed Central, 2001 31(2023), 7-8 vom: 20. Juli, Seite 291-299 (DE-627)53467299X (DE-600)2365465-X 1876-6250 nnns volume:31 year:2023 number:7-8 day:20 month:07 pages:291-299 https://dx.doi.org/10.1007/s12471-023-01800-4 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 31 2023 7-8 20 07 291-299
allfieldsGer	10.1007/s12471-023-01800-4 doi (DE-627)SPR052629775 (SPR)s12471-023-01800-4-e DE-627 ger DE-627 rakwb eng van Lint, Freyja H. M. verfasserin aut Exercise does not influence development of phenotype in PLN p.(Arg14del) cardiomyopathy 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2023 Background Endurance and frequent exercise are associated with earlier onset of arrhythmogenic right ventricular cardiomyopathy (ARVC) and ventricular arrhythmias (VA) in desmosomal gene variant carriers. Individuals with the pathogenic c.40_42del; p.(Arg14del) variant in the PLN gene are frequently diagnosed with ARVC or dilated cardiomyopathy (DCM). The aim of this study was to evaluate the effect of exercise in PLN p.(Arg14del) carriers. Methods In total, 207 adult PLN p.(Arg14del) carriers (39.1% male; mean age 53 ± 15 years) were interviewed on their regular physical activity since the age of 10 years. The association of exercise with diagnosis of ARVC, DCM, sustained VA and hospitalisation for heart failure (HF) was studied. Results Individuals participated in regular physical activities with a median of 1661 metabolic equivalent of task (MET) hours per year (31.9 MET-hours per week) until clinical presentation. The 50% most and least active individuals had a similar frequency of sustained VA (18.3% vs 18.4%; p = 0.974) and hospitalisation for HF (9.6% vs 8.7%; p = 0.827). There was no relationship between exercise and survival free from (incident) sustained VA (p = 0.65), hospitalisation for HF (p = 0.81), diagnosis of ARVC (p = 0.67) or DCM (p = 0.39) during follow-up. In multivariate analyses, exercise was not associated with sustained VA or HF hospitalisation during follow-up in this relatively not-active cohort. Conclusion There was no association between the amount of exercise and the susceptibility to develop ARVC, DCM, VA or HF in PLN p.(Arg14del) carriers. This suggested unaffected PLN p.(Arg14del) carriers can safely perform mild-moderate exercise, in contrast to desmosomal variant carriers and ARVC patients. Arrhythmogenic right ventricular cardiomyopathy (dpeaa)DE-He213 Dilated cardiomyopathy (dpeaa)DE-He213 Genetics (dpeaa)DE-He213 Exercise (dpeaa)DE-He213 Penetrance (dpeaa)DE-He213 Hassanzada, Fahima aut Verstraelen, Tom E. aut Wang, Weijia aut Bosman, Laurens P. aut van der Zwaag, Paul A. aut Oomen, Toon aut Calkins, Hugh aut Murray, Brittney aut Tichnell, Crystal aut Beuren, Thais M. A. aut Asselbergs, Folkert W. aut Houweling, Arjan aut van den Berg, Maarten P. aut Wilde, Arthur A. M. aut James, Cynthia A. aut van Tintelen, J. Peter aut Enthalten in Netherlands heart journal [S.l.] : PubMed Central, 2001 31(2023), 7-8 vom: 20. Juli, Seite 291-299 (DE-627)53467299X (DE-600)2365465-X 1876-6250 nnns volume:31 year:2023 number:7-8 day:20 month:07 pages:291-299 https://dx.doi.org/10.1007/s12471-023-01800-4 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 31 2023 7-8 20 07 291-299
allfieldsSound	10.1007/s12471-023-01800-4 doi (DE-627)SPR052629775 (SPR)s12471-023-01800-4-e DE-627 ger DE-627 rakwb eng van Lint, Freyja H. M. verfasserin aut Exercise does not influence development of phenotype in PLN p.(Arg14del) cardiomyopathy 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2023 Background Endurance and frequent exercise are associated with earlier onset of arrhythmogenic right ventricular cardiomyopathy (ARVC) and ventricular arrhythmias (VA) in desmosomal gene variant carriers. Individuals with the pathogenic c.40_42del; p.(Arg14del) variant in the PLN gene are frequently diagnosed with ARVC or dilated cardiomyopathy (DCM). The aim of this study was to evaluate the effect of exercise in PLN p.(Arg14del) carriers. Methods In total, 207 adult PLN p.(Arg14del) carriers (39.1% male; mean age 53 ± 15 years) were interviewed on their regular physical activity since the age of 10 years. The association of exercise with diagnosis of ARVC, DCM, sustained VA and hospitalisation for heart failure (HF) was studied. Results Individuals participated in regular physical activities with a median of 1661 metabolic equivalent of task (MET) hours per year (31.9 MET-hours per week) until clinical presentation. The 50% most and least active individuals had a similar frequency of sustained VA (18.3% vs 18.4%; p = 0.974) and hospitalisation for HF (9.6% vs 8.7%; p = 0.827). There was no relationship between exercise and survival free from (incident) sustained VA (p = 0.65), hospitalisation for HF (p = 0.81), diagnosis of ARVC (p = 0.67) or DCM (p = 0.39) during follow-up. In multivariate analyses, exercise was not associated with sustained VA or HF hospitalisation during follow-up in this relatively not-active cohort. Conclusion There was no association between the amount of exercise and the susceptibility to develop ARVC, DCM, VA or HF in PLN p.(Arg14del) carriers. This suggested unaffected PLN p.(Arg14del) carriers can safely perform mild-moderate exercise, in contrast to desmosomal variant carriers and ARVC patients. Arrhythmogenic right ventricular cardiomyopathy (dpeaa)DE-He213 Dilated cardiomyopathy (dpeaa)DE-He213 Genetics (dpeaa)DE-He213 Exercise (dpeaa)DE-He213 Penetrance (dpeaa)DE-He213 Hassanzada, Fahima aut Verstraelen, Tom E. aut Wang, Weijia aut Bosman, Laurens P. aut van der Zwaag, Paul A. aut Oomen, Toon aut Calkins, Hugh aut Murray, Brittney aut Tichnell, Crystal aut Beuren, Thais M. A. aut Asselbergs, Folkert W. aut Houweling, Arjan aut van den Berg, Maarten P. aut Wilde, Arthur A. M. aut James, Cynthia A. aut van Tintelen, J. Peter aut Enthalten in Netherlands heart journal [S.l.] : PubMed Central, 2001 31(2023), 7-8 vom: 20. Juli, Seite 291-299 (DE-627)53467299X (DE-600)2365465-X 1876-6250 nnns volume:31 year:2023 number:7-8 day:20 month:07 pages:291-299 https://dx.doi.org/10.1007/s12471-023-01800-4 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 31 2023 7-8 20 07 291-299
language	English
source	Enthalten in Netherlands heart journal 31(2023), 7-8 vom: 20. Juli, Seite 291-299 volume:31 year:2023 number:7-8 day:20 month:07 pages:291-299
sourceStr	Enthalten in Netherlands heart journal 31(2023), 7-8 vom: 20. Juli, Seite 291-299 volume:31 year:2023 number:7-8 day:20 month:07 pages:291-299
format_phy_str_mv	Article
institution	findex.gbv.de
topic_facet	Arrhythmogenic right ventricular cardiomyopathy Dilated cardiomyopathy Genetics Exercise Penetrance
isfreeaccess_bool	true
container_title	Netherlands heart journal
authorswithroles_txt_mv	van Lint, Freyja H. M. @@aut@@ Hassanzada, Fahima @@aut@@ Verstraelen, Tom E. @@aut@@ Wang, Weijia @@aut@@ Bosman, Laurens P. @@aut@@ van der Zwaag, Paul A. @@aut@@ Oomen, Toon @@aut@@ Calkins, Hugh @@aut@@ Murray, Brittney @@aut@@ Tichnell, Crystal @@aut@@ Beuren, Thais M. A. @@aut@@ Asselbergs, Folkert W. @@aut@@ Houweling, Arjan @@aut@@ van den Berg, Maarten P. @@aut@@ Wilde, Arthur A. M. @@aut@@ James, Cynthia A. @@aut@@ van Tintelen, J. Peter @@aut@@
publishDateDaySort_date	2023-07-20T00:00:00Z
hierarchy_top_id	53467299X
id	SPR052629775
language_de	englisch
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M.</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="245" ind1="1" ind2="0"><subfield code="a">Exercise does not influence development of phenotype in PLN p.(Arg14del) cardiomyopathy</subfield></datafield><datafield tag="264" ind1=" " ind2="1"><subfield code="c">2023</subfield></datafield><datafield tag="336" ind1=" " ind2=" "><subfield code="a">Text</subfield><subfield code="b">txt</subfield><subfield code="2">rdacontent</subfield></datafield><datafield tag="337" ind1=" " ind2=" "><subfield code="a">Computermedien</subfield><subfield code="b">c</subfield><subfield code="2">rdamedia</subfield></datafield><datafield tag="338" ind1=" " ind2=" "><subfield code="a">Online-Ressource</subfield><subfield code="b">cr</subfield><subfield code="2">rdacarrier</subfield></datafield><datafield tag="500" ind1=" " ind2=" "><subfield code="a">© The Author(s) 2023</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Background Endurance and frequent exercise are associated with earlier onset of arrhythmogenic right ventricular cardiomyopathy (ARVC) and ventricular arrhythmias (VA) in desmosomal gene variant carriers. Individuals with the pathogenic c.40_42del; p.(Arg14del) variant in the PLN gene are frequently diagnosed with ARVC or dilated cardiomyopathy (DCM). The aim of this study was to evaluate the effect of exercise in PLN p.(Arg14del) carriers. Methods In total, 207 adult PLN p.(Arg14del) carriers (39.1% male; mean age 53 ± 15 years) were interviewed on their regular physical activity since the age of 10 years. The association of exercise with diagnosis of ARVC, DCM, sustained VA and hospitalisation for heart failure (HF) was studied. Results Individuals participated in regular physical activities with a median of 1661 metabolic equivalent of task (MET) hours per year (31.9 MET-hours per week) until clinical presentation. The 50% most and least active individuals had a similar frequency of sustained VA (18.3% vs 18.4%; p = 0.974) and hospitalisation for HF (9.6% vs 8.7%; p = 0.827). There was no relationship between exercise and survival free from (incident) sustained VA (p = 0.65), hospitalisation for HF (p = 0.81), diagnosis of ARVC (p = 0.67) or DCM (p = 0.39) during follow-up. In multivariate analyses, exercise was not associated with sustained VA or HF hospitalisation during follow-up in this relatively not-active cohort. Conclusion There was no association between the amount of exercise and the susceptibility to develop ARVC, DCM, VA or HF in PLN p.(Arg14del) carriers. 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author	van Lint, Freyja H. M.
spellingShingle	van Lint, Freyja H. M. misc Arrhythmogenic right ventricular cardiomyopathy misc Dilated cardiomyopathy misc Genetics misc Exercise misc Penetrance Exercise does not influence development of phenotype in PLN p.(Arg14del) cardiomyopathy
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topic_title	Exercise does not influence development of phenotype in PLN p.(Arg14del) cardiomyopathy Arrhythmogenic right ventricular cardiomyopathy (dpeaa)DE-He213 Dilated cardiomyopathy (dpeaa)DE-He213 Genetics (dpeaa)DE-He213 Exercise (dpeaa)DE-He213 Penetrance (dpeaa)DE-He213
topic	misc Arrhythmogenic right ventricular cardiomyopathy misc Dilated cardiomyopathy misc Genetics misc Exercise misc Penetrance
topic_unstemmed	misc Arrhythmogenic right ventricular cardiomyopathy misc Dilated cardiomyopathy misc Genetics misc Exercise misc Penetrance
topic_browse	misc Arrhythmogenic right ventricular cardiomyopathy misc Dilated cardiomyopathy misc Genetics misc Exercise misc Penetrance
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title	Exercise does not influence development of phenotype in PLN p.(Arg14del) cardiomyopathy
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title_full	Exercise does not influence development of phenotype in PLN p.(Arg14del) cardiomyopathy
author_sort	van Lint, Freyja H. M.
journal	Netherlands heart journal
journalStr	Netherlands heart journal
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author_browse	van Lint, Freyja H. M. Hassanzada, Fahima Verstraelen, Tom E. Wang, Weijia Bosman, Laurens P. van der Zwaag, Paul A. Oomen, Toon Calkins, Hugh Murray, Brittney Tichnell, Crystal Beuren, Thais M. A. Asselbergs, Folkert W. Houweling, Arjan van den Berg, Maarten P. Wilde, Arthur A. M. James, Cynthia A. van Tintelen, J. Peter
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author-letter	van Lint, Freyja H. M.
doi_str_mv	10.1007/s12471-023-01800-4
title_sort	exercise does not influence development of phenotype in pln p.(arg14del) cardiomyopathy
title_auth	Exercise does not influence development of phenotype in PLN p.(Arg14del) cardiomyopathy
abstract	Background Endurance and frequent exercise are associated with earlier onset of arrhythmogenic right ventricular cardiomyopathy (ARVC) and ventricular arrhythmias (VA) in desmosomal gene variant carriers. Individuals with the pathogenic c.40_42del; p.(Arg14del) variant in the PLN gene are frequently diagnosed with ARVC or dilated cardiomyopathy (DCM). The aim of this study was to evaluate the effect of exercise in PLN p.(Arg14del) carriers. Methods In total, 207 adult PLN p.(Arg14del) carriers (39.1% male; mean age 53 ± 15 years) were interviewed on their regular physical activity since the age of 10 years. The association of exercise with diagnosis of ARVC, DCM, sustained VA and hospitalisation for heart failure (HF) was studied. Results Individuals participated in regular physical activities with a median of 1661 metabolic equivalent of task (MET) hours per year (31.9 MET-hours per week) until clinical presentation. The 50% most and least active individuals had a similar frequency of sustained VA (18.3% vs 18.4%; p = 0.974) and hospitalisation for HF (9.6% vs 8.7%; p = 0.827). There was no relationship between exercise and survival free from (incident) sustained VA (p = 0.65), hospitalisation for HF (p = 0.81), diagnosis of ARVC (p = 0.67) or DCM (p = 0.39) during follow-up. In multivariate analyses, exercise was not associated with sustained VA or HF hospitalisation during follow-up in this relatively not-active cohort. Conclusion There was no association between the amount of exercise and the susceptibility to develop ARVC, DCM, VA or HF in PLN p.(Arg14del) carriers. This suggested unaffected PLN p.(Arg14del) carriers can safely perform mild-moderate exercise, in contrast to desmosomal variant carriers and ARVC patients. © The Author(s) 2023
abstractGer	Background Endurance and frequent exercise are associated with earlier onset of arrhythmogenic right ventricular cardiomyopathy (ARVC) and ventricular arrhythmias (VA) in desmosomal gene variant carriers. Individuals with the pathogenic c.40_42del; p.(Arg14del) variant in the PLN gene are frequently diagnosed with ARVC or dilated cardiomyopathy (DCM). The aim of this study was to evaluate the effect of exercise in PLN p.(Arg14del) carriers. Methods In total, 207 adult PLN p.(Arg14del) carriers (39.1% male; mean age 53 ± 15 years) were interviewed on their regular physical activity since the age of 10 years. The association of exercise with diagnosis of ARVC, DCM, sustained VA and hospitalisation for heart failure (HF) was studied. Results Individuals participated in regular physical activities with a median of 1661 metabolic equivalent of task (MET) hours per year (31.9 MET-hours per week) until clinical presentation. The 50% most and least active individuals had a similar frequency of sustained VA (18.3% vs 18.4%; p = 0.974) and hospitalisation for HF (9.6% vs 8.7%; p = 0.827). There was no relationship between exercise and survival free from (incident) sustained VA (p = 0.65), hospitalisation for HF (p = 0.81), diagnosis of ARVC (p = 0.67) or DCM (p = 0.39) during follow-up. In multivariate analyses, exercise was not associated with sustained VA or HF hospitalisation during follow-up in this relatively not-active cohort. Conclusion There was no association between the amount of exercise and the susceptibility to develop ARVC, DCM, VA or HF in PLN p.(Arg14del) carriers. This suggested unaffected PLN p.(Arg14del) carriers can safely perform mild-moderate exercise, in contrast to desmosomal variant carriers and ARVC patients. © The Author(s) 2023
abstract_unstemmed	Background Endurance and frequent exercise are associated with earlier onset of arrhythmogenic right ventricular cardiomyopathy (ARVC) and ventricular arrhythmias (VA) in desmosomal gene variant carriers. Individuals with the pathogenic c.40_42del; p.(Arg14del) variant in the PLN gene are frequently diagnosed with ARVC or dilated cardiomyopathy (DCM). The aim of this study was to evaluate the effect of exercise in PLN p.(Arg14del) carriers. Methods In total, 207 adult PLN p.(Arg14del) carriers (39.1% male; mean age 53 ± 15 years) were interviewed on their regular physical activity since the age of 10 years. The association of exercise with diagnosis of ARVC, DCM, sustained VA and hospitalisation for heart failure (HF) was studied. Results Individuals participated in regular physical activities with a median of 1661 metabolic equivalent of task (MET) hours per year (31.9 MET-hours per week) until clinical presentation. The 50% most and least active individuals had a similar frequency of sustained VA (18.3% vs 18.4%; p = 0.974) and hospitalisation for HF (9.6% vs 8.7%; p = 0.827). There was no relationship between exercise and survival free from (incident) sustained VA (p = 0.65), hospitalisation for HF (p = 0.81), diagnosis of ARVC (p = 0.67) or DCM (p = 0.39) during follow-up. In multivariate analyses, exercise was not associated with sustained VA or HF hospitalisation during follow-up in this relatively not-active cohort. Conclusion There was no association between the amount of exercise and the susceptibility to develop ARVC, DCM, VA or HF in PLN p.(Arg14del) carriers. This suggested unaffected PLN p.(Arg14del) carriers can safely perform mild-moderate exercise, in contrast to desmosomal variant carriers and ARVC patients. © The Author(s) 2023
collection_details	GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700
container_issue	7-8
title_short	Exercise does not influence development of phenotype in PLN p.(Arg14del) cardiomyopathy
url	https://dx.doi.org/10.1007/s12471-023-01800-4
remote_bool	true
author2	Hassanzada, Fahima Verstraelen, Tom E. Wang, Weijia Bosman, Laurens P. van der Zwaag, Paul A. Oomen, Toon Calkins, Hugh Murray, Brittney Tichnell, Crystal Beuren, Thais M. A. Asselbergs, Folkert W. Houweling, Arjan van den Berg, Maarten P. Wilde, Arthur A. M. James, Cynthia A. van Tintelen, J. Peter
author2Str	Hassanzada, Fahima Verstraelen, Tom E. Wang, Weijia Bosman, Laurens P. van der Zwaag, Paul A. Oomen, Toon Calkins, Hugh Murray, Brittney Tichnell, Crystal Beuren, Thais M. A. Asselbergs, Folkert W. Houweling, Arjan van den Berg, Maarten P. Wilde, Arthur A. M. James, Cynthia A. van Tintelen, J. Peter
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doi_str	10.1007/s12471-023-01800-4
up_date	2024-07-03T13:38:48.733Z
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Exercise does not influence development of phenotype in PLN p.(Arg14del) cardiomyopathy

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