Duchenne/Becker muscular dystrophy Cardiac care in Czech
republic: a cardiovascular magnetic resonance study with T1
mapping

p 2018

Duchenne/Becker muscular dystrophy Cardiac care in Czech republic: a cardiovascular magnetic resonance study with T1 mapping

PEŠL, Martin, Roman PANOVSKÝ, Tomáš HOLEČEK, Jan MÁCHAL, Vera FEITOVÁ et. al.

Základní údaje

Originální název

Duchenne/Becker muscular dystrophy Cardiac care in Czech republic: a cardiovascular magnetic resonance study with T1 mapping

Autoři

PEŠL, Martin, Roman PANOVSKÝ, Tomáš HOLEČEK, Jan MÁCHAL, Vera FEITOVÁ, Lenka MRÁZOVÁ, Jana HABERLOVÁ, Alžběta SLABÁ, Pavel VÍT, Veronika STARÁ a Vladimír KINCL

Vydání

2018

Další údaje

Jazyk

angličtina

Typ výsledku

Vyžádané přednášky

Obor

30201 Cardiac and Cardiovascular systems

Stát vydavatele

Česká republika

Utajení

není předmětem státního či obchodního tajemství

Organizační jednotka

Lékařská fakulta

Klíčová slova česky

DMD, BMD, kardiomyopatie, megnetická resonance srdce

Klíčová slova anglicky

DMD, BMD, cardiomyopathy, cardiac magnetic resonance imaging

Příznaky

Mezinárodní význam

Změněno: 28. 9. 2018 13:22, MUDr. Martin Pešl, Ph.D.

Anotace

V originále

The progressive cardiomyopathy that develops in Duchenne and Becker muscular dystrophy (DMD/BMD) boys during their teenage is presumed to be a secondary consequence of the fibrosis within the myocardium. This is hard to follow by standard echocardiography, due to narrow intercostal spaces, skeletal abnormities and uneasy positioning of the patients. The same time there are only limited data on using parametric imaging in these patients. Our study aimed to assess native T1 and extracellular volume (ECV) values in DMD/BMD patients. The Czech population of males with DMD/BMD was screened with help of registry of muscular dystrophies, REaDY. 49 teenage males underwent cardiac magnetic resonance examination including T1 native and postcontrast mapping measurements. Eligible for evaluation were 48 of them and those were compared with Control group of gender matched controls (n=13). DMD/BMD patients without late gadolinium enhancement (LGE) (n=27) were set as group D1, those DMD/BMD patients with LGE (n=21) were set as group D2. Results: Both DMD/BMD groups had higher T1 native relaxation time compared to controls. These results are concordant in all 6 segments as well as in global values. Group D2 had significantly increased global ECV 0.277 ± 0.046 vs 0.243 ± 0.013, (p < 0.05) and segmental ECV in inferolateral and anterolateral segments in comparison with controls. The results were significant also after the adjustment for subjects’ age. Conclusion: DMD/BMD males had increased native T1 relaxation time independently on the presence or absence of myocardial fibrosis. Elevated ECV was found only in patients with LGE. These results supports timely prescription of ACE inhibitors as prevention and slowing of heart muscle remodeling, the same time authors have to stress importance of cardiac magnetic resonance, giving more detailed and repeatable outcomes compare to standard echocardiography.

Citovat

PEŠL, Martin, Roman PANOVSKÝ, Tomáš HOLEČEK, Jan MÁCHAL, Vera FEITOVÁ, Lenka MRÁZOVÁ, Jana HABERLOVÁ, Alžběta SLABÁ, Pavel VÍT, Veronika STARÁ a Vladimír KINCL. Duchenne/Becker muscular dystrophy Cardiac care in Czech republic: a cardiovascular magnetic resonance study with T1 mapping. 2018.

@misc{1442156,
   author = {Pešl, Martin and Panovský, Roman and Holeček, Tomáš and Máchal, Jan and Feitová, Vera and Mrázová, Lenka and Haberlová, Jana and Slabá, Alžběta and Vít, Pavel and Stará, Veronika and Kincl, Vladimír},
   keywords = {DMD, BMD, cardiomyopathy, cardiac magnetic resonance imaging},
   language = {eng},
   title = {Duchenne/Becker muscular dystrophy Cardiac care in Czech republic: a cardiovascular magnetic resonance study with T1 mapping},
   year = {2018}
}

TY  - SLIDE
ID  - 1442156
AU  - Pešl, Martin - Panovský, Roman - Holeček, Tomáš - Máchal, Jan - Feitová, Vera - Mrázová, Lenka - Haberlová, Jana - Slabá, Alžběta - Vít, Pavel - Stará, Veronika - Kincl, Vladimír
PY  - 2018
TI  - Duchenne/Becker muscular dystrophy Cardiac care in Czech republic: a cardiovascular magnetic resonance study with T1 mapping
KW  - DMD, BMD, cardiomyopathy, cardiac magnetic resonance imaging
N2  - The progressive cardiomyopathy that develops in Duchenne and Becker muscular dystrophy (DMD/BMD) boys during their teenage is presumed to be a secondary consequence of the fibrosis within the myocardium. This is hard to follow by standard echocardiography, due to narrow intercostal spaces, skeletal abnormities and uneasy positioning of the patients. The same time there are only limited data on using parametric imaging in these patients. Our study aimed to assess native T1 and extracellular volume (ECV) values in DMD/BMD patients. The Czech population of males with DMD/BMD was screened with help of registry of muscular dystrophies, REaDY. 49 teenage males underwent cardiac magnetic resonance examination including T1 native and postcontrast mapping measurements. Eligible for evaluation were 48 of them and those were compared with Control group of gender matched controls (n=13). DMD/BMD patients without late gadolinium enhancement (LGE) (n=27) were set as group D1, those DMD/BMD patients with LGE (n=21) were set as group D2. Results: Both DMD/BMD groups had higher T1 native relaxation time compared to controls. These results are concordant in all 6 segments as well as in global values. Group D2 had significantly increased global ECV 0.277 ± 0.046 vs 0.243 ± 0.013, (p < 0.05) and segmental ECV in inferolateral and anterolateral segments in comparison with controls. The results were significant also after the adjustment for subjects’ age. Conclusion: DMD/BMD males had increased native T1 relaxation time independently on the presence or absence of myocardial fibrosis. Elevated ECV was found only in patients with LGE. These results supports timely prescription of ACE inhibitors as prevention and slowing of heart muscle remodeling, the same time authors have to stress importance of cardiac magnetic resonance, giving more detailed and repeatable outcomes compare to standard echocardiography.
ER  -

PEŠL, Martin, Roman PANOVSKÝ, Tomáš HOLEČEK, Jan MÁCHAL, Vera FEITOVÁ, Lenka MRÁZOVÁ, Jana HABERLOVÁ, Alžběta SLABÁ, Pavel VÍT, Veronika STARÁ a Vladimír KINCL. \textit{Duchenne/Becker muscular dystrophy Cardiac care in Czech republic: a cardiovascular magnetic resonance study with T1 mapping}. 2018.

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