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Article: Effects and Optimal Dose of Exercise on Endothelial Function in Patients with Heart Failure: A Systematic Review and Meta-Analysis.

Fuertes-Kenneally, Laura / Manresa-Rocamora, Agustín / Blasco-Peris, Carles / Ribeiro, Fernando / Sempere-Ruiz, Noemí / Sarabia, José Manuel / Climent-Paya, Vicente

Sports medicine - open

2023 Volume 9, Issue 1, Page(s) 8

Abstract: Background: Exercise-based cardiac rehabilitation (CR) is considered an effective treatment for enhancing endothelial function in patients with heart failure (HF). However, recent studies have been published and the optimal "dose" of exercise required ... ...

Abstract	Background: Exercise-based cardiac rehabilitation (CR) is considered an effective treatment for enhancing endothelial function in patients with heart failure (HF). However, recent studies have been published and the optimal "dose" of exercise required to increase the benefits of exercise-based CR programmes on endothelial function is still unknown. Objectives: (a) To estimate the effect of exercise-based CR on endothelial function, assessed by flow-mediated dilation (FMD), in patients with HF; (b) to determine whether high-intensity interval training (HIIT) is better than moderate-intensity training (MIT) for improving FMD; and (c) to investigate the influence of exercise modality (i.e. resistance exercise vs. aerobic exercise and combined exercise vs. aerobic exercise) on the improvement of endothelial function. Methods: Electronic searches were carried out in PubMed, Embase, and Scopus up to February 2022. Random-effects models of between-group mean differences were estimated. Heterogeneity analyses were performed by means of the chi-square test and I Results: We found a FMD increase of 3.09% (95% confidence interval [CI] = 2.01, 4.17) in favour of aerobic-based CR programmes compared with control groups in patients with HF and reduced ejection fraction (HFrEF). However, the results of included studies were inconsistent (p < .001; I Conclusion: Aerobic-based CR is a non-pharmacological treatment for enhancing endothelial function in patients with HFrEF. However, higher training frequency and HIIT induce greater adaptation of endothelial function in these patients, which should betaken into consideration when designing exercise-based CR programmes. Trial registration The protocol was prospectively registered on the PROSPERO database (CRD42022304687).
Language	English
Publishing date	2023-02-04
Publishing country	Switzerland
Document type	Systematic Review
ZDB-ID	2809942-4
ISSN	2198-9761 ; 2199-1170
ISSN (online)	2198-9761
ISSN	2199-1170
DOI	10.1186/s40798-023-00553-z
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Article ; Online: Is the Verification Phase a Suitable Criterion for the Determination of Maximum Oxygen Uptake in Patients with Heart Failure and Reduced Ejection Fraction? A Validation Study.

Manresa-Rocamora, Agustín / Fuertes-Kenneally, Laura / Blasco-Peris, Carles / Sempere-Ruiz, Noemí / Sarabia, José Manuel / Climent-Paya, Vicente

International journal of environmental research and public health

2023 Volume 20, Issue 4

Abstract: The verification phase (VP) has been proposed as an alternative to the traditional criteria used for the determination of the maximum oxygen uptake ( ... ...

Abstract	The verification phase (VP) has been proposed as an alternative to the traditional criteria used for the determination of the maximum oxygen uptake (VO
MeSH term(s)	Adult ; Humans ; Male ; Female ; Heart Failure ; Stroke Volume ; Oxygen Consumption ; Oxygen ; Exercise ; Exercise Test/methods
Chemical Substances	Oxygen (S88TT14065)
Language	English
Publishing date	2023-02-04
Publishing country	Switzerland
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2175195-X
ISSN	1660-4601 ; 1661-7827
ISSN (online)	1660-4601
ISSN	1661-7827
DOI	10.3390/ijerph20042764
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Article ; Online: Effects of Exergaming in Patients with Cardiovascular Disease Compared to Conventional Cardiac Rehabilitation: A Systematic Review and Meta-Analysis.

Blasco-Peris, Carles / Fuertes-Kenneally, Laura / Vetrovsky, Tomas / Sarabia, José Manuel / Climent-Paya, Vicente / Manresa-Rocamora, Agustín

International journal of environmental research and public health

2022 Volume 19, Issue 6

Abstract: Background: Exercise-based cardiac rehabilitation (CR) programs are used for improving prognosis and quality of life in patients with cardiovascular disease (CVD). Nonetheless, adherence to these programs is low, and exercise-based CR programs based on ... ...

Abstract	Background: Exercise-based cardiac rehabilitation (CR) programs are used for improving prognosis and quality of life in patients with cardiovascular disease (CVD). Nonetheless, adherence to these programs is low, and exercise-based CR programs based on virtual reality (i.e., exergaming) have been proposed as an alternative to conventional CR programs. However, whether exergaming programs are superior to conventional CR programs in patients with CVD is not known. Objective: This systematic review with meta-analysis was conducted to explore whether exergaming enhances exercise capacity, quality of life, mental health, motivation, and exercise adherence to a greater extent than conventional CR programs in patients with CVD. Method: Electronic searches were carried out in PubMed, Embase, Web of Science, and Cumulative Index to Nursing and Allied Health Literature databases up to June 2021. Meta-analyses were performed using robust variance estimation with small-sample corrections. The effect sizes were calculated as the mean differences (MD) or standardized mean differences (SMD) as appropriate. The SMD magnitude was classified as trivial (<0.20), small (0.20−0.49), medium (0.50−0.79), or large (≥0.80). Heterogeneity was interpreted based on the I2 statistics as low (25%), moderate (50%), or high (75%). Results: Pooled analyses showed no differences between exergaming and conventional CR programs for enhancing exercise capacity (i.e., distance covered in the six-minute walk test) (MD+ = 14.07 m (95% confidence interval (CI) −38.18 to 66.32 m); p = 0.426) and mental health (SMD+ = 0.17 (95% CI −0.36 to 0.70); p = 0.358). The results showed a small, statistically nonsignificant improvement in quality of life in favor of exergaming (SMD+ = 0.22 (95% CI = −0.37 to 0.81); p = 0.294). Moderate heterogeneity was found for exercise capacity (I2 = 53.7%), while no heterogeneity was found for quality of life (I2 = 3.3%) and mental health (I2 = 0.0%). Conclusions: Exergaming seems not to be superior to conventional CR programs for improving exercise capacity, quality of life, or mental health in patients with CVD.
MeSH term(s)	Cardiac Rehabilitation/methods ; Cardiovascular Diseases/etiology ; Exercise Therapy ; Exergaming ; Humans ; Quality of Life
Language	English
Publishing date	2022-03-15
Publishing country	Switzerland
Document type	Journal Article ; Meta-Analysis ; Research Support, Non-U.S. Gov't ; Review ; Systematic Review
ZDB-ID	2175195-X
ISSN	1660-4601 ; 1661-7827
ISSN (online)	1660-4601
ISSN	1661-7827
DOI	10.3390/ijerph19063492
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Article ; Online: Intermediate-effect size p.Arg637Gln in

Piqueras-Flores, Jesús / Villacorta-Argüelles, Eduardo / Galvin, Joseph / Climent-Payá, Vicente / Escobar-López, Luis Enrique / Amor-Salamanca, Almudena / Garcia-Hernandez, Soledad / Esmonde, Sean / Martínez-Del Río, Jorge / Soto-Pérez, Maeve / Garcia-Pavia, Pablo / Ochoa, Juan Pablo

Journal of medical genetics

2024 Volume 61, Issue 5, Page(s) 423–427

Abstract: Formin homology 2 domain-containing 3 ( ...

Abstract	Formin homology 2 domain-containing 3 (
MeSH term(s)	Humans ; Cardiomyopathy, Hypertrophic/genetics ; Phenotype ; Homozygote ; Penetrance ; Heterozygote ; Formins/genetics
Chemical Substances	FHOD3 protein, human ; Formins
Language	English
Publishing date	2024-04-19
Publishing country	England
Document type	Journal Article
ZDB-ID	220881-7
ISSN	1468-6244 ; 0022-2593
ISSN (online)	1468-6244
ISSN	0022-2593
DOI	10.1136/jmg-2023-109413
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Article ; Online: Left atrial appendage closure with 2 different devices.

Ruiz-Nodar, Juan M / Ibáñez Criado, José L / Climent Payá, Vicente

Revista espanola de cardiologia (English ed.)

2019 Volume 73, Issue 6, Page(s) 498

MeSH term(s)	Atrial Appendage/diagnostic imaging ; Atrial Appendage/surgery ; Atrial Fibrillation/surgery ; Cardiac Surgical Procedures ; Humans ; Stroke ; Treatment Outcome
Language	Spanish
Publishing date	2019-09-24
Publishing country	Spain
Document type	Journal Article
ZDB-ID	2592481-3
ISSN	1885-5857 ; 1885-5857
ISSN (online)	1885-5857
ISSN	1885-5857
DOI	10.1016/j.rec.2019.06.012
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Article ; Online: International Physical Activity Questionnaire Short Form and accelerometer-assessed physical activity: concurrent validity using six cut-points in HF patients.

Blasco-Peris, Carles / Climent-Paya, Vicente / Vetrovsky, Tomas / García-Álvarez, María I / Manresa-Rocamora, Agustín / Beltrán-Carrillo, Vicente J / Sarabia, José Manuel

ESC heart failure

2023 Volume 11, Issue 1, Page(s) 126–135

Abstract: Aims: Physical activity (PA) is an important target for improving clinical outcomes in heart failure (HF) patients. Nonetheless, assessing the daily PA profile in this population is a challenging task, traditionally performed using self-report ... ...

Abstract	Aims: Physical activity (PA) is an important target for improving clinical outcomes in heart failure (HF) patients. Nonetheless, assessing the daily PA profile in this population is a challenging task, traditionally performed using self-report questionnaires such as the International PA Questionnaire Short Form (IPAQ-SF). This study aimed to evaluate the concurrent validity of the IPAQ-SF and accelerometer-assessed PA using six published cut-points in patients with HF and reduced or mildly reduced ejection fraction. Methods and results: The concordance between the IPAQ-SF and a hip-worn accelerometer regarding daily time spent performing moderate to vigorous PA in bouts of at least 10 min was assessed in 53 participants for seven consecutive days using six different cut-points (Barnett, Dibben, Mark, Sanders, Troiano, and Vaha-Ypya). Spearman's correlation and Bland-Altman plots were used to evaluate concurrent validity between methods. Regressions were used to study the association between patient variables, wear protocol (waking hour or 24 h), and absolute bias. The kappa index was used to evaluate the concordance between IPAQ-SF and accelerometry for classifying patients as active or non-active. All analyses were re-run using non-bouted metrics to investigate the effect of bouted versus non-bouted analysis. The IPAQ-SF and accelerometry showed low to negligible correlation (ρ = 0.12 to 0.37), depending on the cut-point used. The regression analysis showed that the absolute bias was higher in participants following the waking-hour protocol at all cut-points except Dibben's (P ≤ 0.007). The concordance between the two methods to classify patients as active and non-active was low when using Mark (κ = 0.23) and Barnett (κ = 0.34) cut-points and poor for the remaining cut-points (κ = 0.03 to 0.18). The results of the sensitivity analysis showed negligible to low correlation using non-bouted metrics (ρ = 0.27 to 0.33). Conclusions: Moderate to vigorous PA measures using IPAQ-SF and accelerometers are not equivalent, and we do not encourage researchers to use IPAQ-SF alone when assessing PA in HF patients. Moreover, applying personalized collection and processing criteria is important when assessing PA in HF patients. We recommend following the 24 h protocol and selecting cut-points calibrated in patients with cardiovascular diseases. Finally, it is necessary to develop a new tailored questionnaire that considers walking intensity and is adjusted to the current World Health Organisation recommendations, which use non-bouted metrics.
MeSH term(s)	Humans ; Exercise ; Surveys and Questionnaires ; Self Report ; Accelerometry ; Heart Failure/diagnosis
Language	English
Publishing date	2023-10-16
Publishing country	England
Document type	Journal Article
ZDB-ID	2814355-3
ISSN	2055-5822 ; 2055-5822
ISSN (online)	2055-5822
ISSN	2055-5822
DOI	10.1002/ehf2.14514
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Article ; Online: Infective endocarditis in elderly and very elderly patients.

Menchi-Elanzi, Marouane / Ramos-Rincón, José M / Merino-Lucas, Esperanza / Reus-Bañuls, Sergio / Torrús-Tendero, Diego / Clíment-Paya, Vicente / Boix, Vicente / Portilla-Sogorb, Joaquín

Aging clinical and experimental research

2019 Volume 32, Issue 7, Page(s) 1383–1388

Abstract: Aims: To compare the clinical and epidemiological characteristics and the evolution of infective endocarditis in adults aged under 65 years, 65-79 years, and 80 years or older.: Methods: An observational retrospective cohort study in patients with ... ...

Abstract	Aims: To compare the clinical and epidemiological characteristics and the evolution of infective endocarditis in adults aged under 65 years, 65-79 years, and 80 years or older. Methods: An observational retrospective cohort study in patients with infective endocarditis was performed in a public hospital in Spain from January 2013 to December 2017. Results: Seventy-two patients were treated: 26 (36.1%) were under 65 years old, 28 (38.9%) were 65-79 years old, and 18 (25%) were aged 80 or older. Prosthetic valve endocarditis was less common in patients aged 65-79 years (3.6%) than in younger (23.1%; p = 0.047) or older (38.9%; p = 0.004) patients. In contrast, degenerative heart disease was more prevalent in the 65-79 year age group [64.3% compared to 15.4% (p < 0.001) in the youngest group, and 33.3% (p = 0.04) in the oldest]. Surgical interventions were similar in patients aged 65-79 (50%) and under 65 years (42.3%), but less common in people over 80 years (16.7%; p = 0.022). Conclusions: The characteristics of infective endocarditis are different in patients aged 65-79 years and in those over 80 years.
MeSH term(s)	Age Distribution ; Aged ; Aged, 80 and over ; Echocardiography ; Endocarditis/epidemiology ; Humans ; Middle Aged ; Retrospective Studies ; Spain/epidemiology
Language	English
Publishing date	2019-08-19
Publishing country	Germany
Document type	Journal Article
ZDB-ID	2104785-6
ISSN	1720-8319 ; 1594-0667
ISSN (online)	1720-8319
ISSN	1594-0667
DOI	10.1007/s40520-019-01314-3
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Article ; Online: Penetrance of Dilated Cardiomyopathy in Genotype-Positive Relatives.

Cabrera-Romero, Eva / Ochoa, Juan Pablo / Barriales-Villa, Roberto / Bermúdez-Jiménez, Francisco José / Climent-Payá, Vicente / Zorio, Esther / Espinosa, María Angeles / Gallego-Delgado, María / Navarro-Peñalver, Marina / Arana-Achaga, Xabier / Piqueras-Flores, Jesús / Espejo-Bares, Victoria / Rodríguez-Palomares, José F / Lacuey-Lecumberri, Gemma / López, Javier / Tiron, Coloma / Peña-Peña, María Luisa / García-Pinilla, Jose M / Lorca, Rebeca /

Ripoll-Vera, Tomas / Díez-López, Carles / Mogollon, María Victoria / García-Álvarez, Ana / Martínez-Dolz, Luis / Brion, María / Larrañaga-Moreira, Jose María / Jiménez-Jáimez, Juan / García-Álvarez, María Isabel / Vilches, Silvia / Villacorta, Eduardo / Sabater-Molina, María / Solla-Ruiz, Itziar / Royuela, Ana / Domínguez, Fernando / Mirelis, Jesús G / Garcia-Pavia, Pablo

Journal of the American College of Cardiology

2024 Volume 83, Issue 17, Page(s) 1640–1651

Abstract: Background: Disease penetrance in genotype-positive (G+) relatives of families with dilated cardiomyopathy (DCM) and the characteristics associated with DCM onset in these individuals are unknown.: Objectives: This study sought to determine the ... ...

Abstract	Background: Disease penetrance in genotype-positive (G+) relatives of families with dilated cardiomyopathy (DCM) and the characteristics associated with DCM onset in these individuals are unknown. Objectives: This study sought to determine the penetrance of new DCM diagnosis in G+ relatives and to identify factors associated with DCM development. Methods: The authors evaluated 779 G+ patients (age 35.8 ± 17.3 years; 459 [59%] females; 367 [47%] with variants in TTN) without DCM followed at 25 Spanish centers. Results: After a median follow-up of 37.1 months (Q1-Q3: 16.3-63.8 months), 85 individuals (10.9%) developed DCM (incidence rate of 2.9 per 100 person-years; 95% CI: 2.3-3.5 per 100 person-years). DCM penetrance and age at DCM onset was different according to underlying gene group (log-rank P = 0.015 and P <0.01, respectively). In a multivariable model excluding CMR parameters, independent predictors of DCM development were: older age (HR per 1-year increase: 1.02; 95% CI: 1.0-1.04), an abnormal electrocardiogram (HR: 2.13; 95% CI: 1.38-3.29); presence of variants in motor sarcomeric genes (HR: 1.92; 95% CI: 1.05-3.50); lower left ventricular ejection fraction (HR per 1% increase: 0.86; 95% CI: 0.82-0.90) and larger left ventricular end-diastolic diameter (HR per 1-mm increase: 1.10; 95% CI: 1.06-1.13). Multivariable analysis in individuals with cardiac magnetic resonance and late gadolinium enhancement assessment (n = 360, 45%) identified late gadolinium enhancement as an additional independent predictor of DCM development (HR: 2.52; 95% CI: 1.43-4.45). Conclusions: Following a first negative screening, approximately 11% of G+ relatives developed DCM during a median follow-up of 3 years. Older age, an abnormal electrocardiogram, lower left ventricular ejection fraction, increased left ventricular end-diastolic diameter, motor sarcomeric genetic variants, and late gadolinium enhancement are associated with a higher risk of developing DCM.
MeSH term(s)	Adult ; Female ; Humans ; Male ; Middle Aged ; Young Adult ; Cardiomyopathy, Dilated/genetics ; Cardiomyopathy, Dilated/physiopathology ; Connectin/genetics ; Electrocardiography ; Follow-Up Studies ; Genotype ; Penetrance ; Spain/epidemiology ; Retrospective Studies
Chemical Substances	Connectin
Language	English
Publishing date	2024-04-24
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	605507-2
ISSN	1558-3597 ; 0735-1097
ISSN (online)	1558-3597
ISSN	0735-1097
DOI	10.1016/j.jacc.2024.02.036
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Article ; Online: Clinical Findings and Prognosis of Danon Disease. An Analysis of the Spanish Multicenter Danon Registry.

López-Sainz, Ángela / Salazar-Mendiguchía, Joel / García-Álvarez, Ana / Campuzano Larrea, Oscar / López-Garrido, Miguel Ángel / García-Guereta, Luis / Fuentes Cañamero, María Eugenia / Climent Payá, Vicente / Peña-Peña, María Luisa / Zorio-Grima, Esther / Jordá-Burgos, Paloma / Díez-López, Carles / Brugada, Ramón / García-Pinilla, José Manuel / García-Pavía, Pablo

Revista espanola de cardiologia (English ed.)

2018 Volume 72, Issue 6, Page(s) 479–486

Abstract: Introduction and objectives: Danon disease (DD) is caused by mutations in the LAMP2 gene. It is considered a multisystemic disease characterized by hypertrophic cardiomyopathy with pre-excitation and extreme hypertrophy, intellectual disability, ... ...

Abstract	Introduction and objectives: Danon disease (DD) is caused by mutations in the LAMP2 gene. It is considered a multisystemic disease characterized by hypertrophic cardiomyopathy with pre-excitation and extreme hypertrophy, intellectual disability, myopathy, childhood presentation, and worse prognosis in men. There are scarce data on the clinical characteristics and prognosis of DD. Methods: We analyzed the clinical records of patients with DD from 10 Spanish hospitals. Results: Twenty-seven patients were included (mean age, 31 ± 19 years; 78% women). Male patients showed a high prevalence of extracardiac manifestations: myopathy (80%), learning disorders (83%), and visual alterations (60%), which were uncommon findings in women (5%, 0%, and 27%, respectively). Although hypertrophic cardiomyopathy was the most common form of heart disease (61%), the mean maximum wall thickness was 15 ± 7 mm and dilated cardiomyopathy was present in 12 patients (10 women). Pre-excitation was found in only 11 patients (49%). Age at presentation was older than 20 years in 16 patients (65%). After a median follow-up of 4 years (interquartile range, 2-9), 4 men (67%) and 9 women (43%) died or required a transplant. Cardiac disease and adverse events occurred later in women (37 ± 9 vs 23 ± 16 and 36 ± 20 vs 20 ± 11 years, respectively). Conclusions: The clinical characteristics of DD differ substantially from traditional descriptions: age at presentation of DD is older, the disease is not multisystemic in women, and pre-excitation is infrequent.
MeSH term(s)	Adolescent ; Adult ; Cardiomyopathy, Hypertrophic/diagnosis ; Cardiomyopathy, Hypertrophic/epidemiology ; Cardiomyopathy, Hypertrophic/etiology ; Electrocardiography ; Female ; Glycogen Storage Disease Type IIb/complications ; Glycogen Storage Disease Type IIb/diagnosis ; Glycogen Storage Disease Type IIb/genetics ; Humans ; Incidence ; Lysosomal-Associated Membrane Protein 2/genetics ; Lysosomal-Associated Membrane Protein 2/metabolism ; Male ; Mutation ; Phenotype ; Prognosis ; Registries ; Retrospective Studies ; Spain/epidemiology ; Wolff-Parkinson-White Syndrome/diagnosis ; Wolff-Parkinson-White Syndrome/epidemiology ; Wolff-Parkinson-White Syndrome/etiology ; Young Adult
Chemical Substances	LAMP2 protein, human ; Lysosomal-Associated Membrane Protein 2
Language	Spanish
Publishing date	2018-08-11
Publishing country	Spain
Document type	Journal Article ; Multicenter Study
ISSN	1885-5857
ISSN (online)	1885-5857
DOI	10.1016/j.rec.2018.04.035
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Article ; Online: Prevalence and clinical outcomes of dystrophin-associated dilated cardiomyopathy without severe skeletal myopathy.

Restrepo-Cordoba, Maria A / Wahbi, Karim / Florian, Anca R / Jiménez-Jáimez, Juan / Politano, Luisa / Arad, Michael / Climent-Paya, Vicente / Garcia-Alvarez, Ana / Hansen, Rasmus B / Larrañaga-Moreira, José M / Kubanek, Milos / Lopes, Luis R / Ros, Andrea / Jurcut, Ruxandra / Rasmussen, Torsten B / Ruiz-Guerrero, Luis / Pribe-Wolferts, Regina / Palomino-Doza, Julian / Bilinska, Zofia /

Rodríguez-Palomares, José F / Van Loon, Rosa L E / Basurte Elorz, María Teresa / Quarta, Giovanni / Robledo Iñarritu, Maria / Verdonschot, Job A J / Stojkovic, Tanya / Shomanova, Zornitsa / Bermudez-Jimenez, Francisco / Palladino, Alberto / Freimark, Dov / García-Álvarez, Maria I / Jorda, Paloma / Dominguez, Fernando / Ochoa, Juan Pablo / Girolami, Francesca / Brugada, Ramon / Meder, Benjamin / Barriales-Villa, Roberto / Mogensen, Jens / Laforêt, Pascal / Yilmaz, Ali / Elliott, Perry / Garcia-Pavia, Pablo

European journal of heart failure

2021 Volume 23, Issue 8, Page(s) 1276–1286

Abstract: Aims: Dilated cardiomyopathy (DCM) associated with dystrophin gene (DMD) mutations in individuals with mild or absent skeletal myopathy is often indistinguishable from other DCM forms. We sought to describe the phenotype and prognosis of DMD associated ... ...

Abstract	Aims: Dilated cardiomyopathy (DCM) associated with dystrophin gene (DMD) mutations in individuals with mild or absent skeletal myopathy is often indistinguishable from other DCM forms. We sought to describe the phenotype and prognosis of DMD associated DCM in DMD mutation carriers without severe skeletal myopathy. Methods and results: At 26 European centres, we retrospectively collected clinical characteristics and outcomes of 223 DMD mutation carriers (83% male, 33 ± 15 years). A total of 112 individuals (52%) had DCM at first evaluation [n = 85; left ventricular ejection fraction (LVEF) 34 ± 11.2%] or developed DCM (n = 27; LVEF 41.3 ± 7.5%) after a median follow-up of 96 months (interquartile range 5-311 months). DCM penetrance was 45% in carriers older than 40 years. DCM appeared earlier in males and was independent of the type of mutation, presence of skeletal myopathy, or elevated serum creatine kinase levels. Major adverse cardiac events (MACE) occurred in 22% individuals with DCM, 18% developed end-stage heart failure and 9% sudden cardiac death or equivalent. Skeletal myopathy was not associated with survival free of MACE in patients with DCM. Decreased LVEF and increased left ventricular end-diastolic diameter at baseline were associated with MACE. Individuals without DCM had favourable prognosis without MACE or death during follow-up. Conclusions: DMD-associated DCM without severe skeletal myopathy is characterized by incomplete penetrance but high risk of MACE, including progression to end-stage heart failure and ventricular arrhythmias. DCM onset is the major determinant of prognosis with similar survival regardless of the presence of skeletal myopathy.
MeSH term(s)	Adolescent ; Adult ; Cardiomyopathy, Dilated/epidemiology ; Cardiomyopathy, Dilated/genetics ; Dystrophin/genetics ; Female ; Heart Failure/epidemiology ; Humans ; Male ; Middle Aged ; Muscular Diseases ; Prevalence ; Retrospective Studies ; Stroke Volume ; Ventricular Function, Left ; Young Adult
Chemical Substances	Dystrophin
Language	English
Publishing date	2021-06-09
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1483672-5
ISSN	1879-0844 ; 1388-9842
ISSN (online)	1879-0844
ISSN	1388-9842
DOI	10.1002/ejhf.2250
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