Late profound muscle weakness following heart transplantation due to danon disease

2012 ◽  
Vol 47 (1) ◽  
pp. 135-137 ◽  
Author(s):  
Pieter Van Der Starre ◽  
Tobias Deuse ◽  
Chad Pritts ◽  
Carlos Brun ◽  
Hannes Vogel ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Muscle Weakness ◽  
Danon Disease

NovelLamp-2 gene mutation and successful treatment with heart transplantation in a large family with Danon disease

2006 ◽  
Vol 33 (3) ◽  
pp. 393-397 ◽  
Author(s):  
Andoni Echaniz-Laguna ◽  
Michel Mohr ◽  
Eric Epailly ◽  
Ichizo Nishino ◽  
Philippe Charron ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Gene Mutation ◽  
Successful Treatment ◽  
Large Family ◽  
Danon Disease

A Case of Female Danon Disease with Rapidly-deteriorated Heart Failure Heading for Heart Transplantation

2012 ◽  
Vol 18 (10) ◽  
pp. S184
Author(s):  
Yuichiro Iida ◽  
Hisahito Shinagawa ◽  
Takayuki Inomata ◽  
Toyoji Kaida ◽  
Toshimi Koitabashi ◽  
...  
Keyword(s):  
Heart Failure ◽  
Heart Transplantation ◽  
Danon Disease

Heart transplantation in two adolescents with Danon disease

2018 ◽  
pp. e13335 ◽  
Author(s):  
Daniel Oren ◽  
Peter Chau ◽  
Melanie Manning ◽  
Joann Kwong ◽  
Beth D. Kaufman ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Danon Disease

Heart transplantation in Danon disease: Long term single centre experience and review of the literature

2020 ◽  
Vol 63 (2) ◽  
pp. 103645 ◽  
Author(s):  
Concetta Di Nora ◽  
Daniela Miani ◽  
Angela Valentina D'Elia ◽  
Stefano Poli ◽  
Maria Iascone ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Review Of The Literature ◽  
Single Centre ◽  
Danon Disease ◽  
Single Centre Experience

Evolution of muscle strength and physical activity 1 year after heart transplantation: a prospective observational study

2021 ◽  
Vol 28 (Supplement_1) ◽  
Author(s):  
M Hornikx ◽  
L Van Aelst ◽  
W Droogne ◽  
S Janssens ◽  
J Van Cleemput
Keyword(s):  
Physical Activity ◽  
Muscle Strength ◽  
Heart Transplantation ◽  
Cardiac Rehabilitation ◽  
Exercise Capacity ◽  
Muscle Weakness ◽  
Peripheral Muscle ◽  
Functional Exercise ◽  
Functional Exercise Capacity

Abstract Funding Acknowledgements Type of funding sources: None. Background Heart transplantation (HTX) is a therapeutic option in a selected group of patients with end-stage heart failure. Although cardiac function normalizes after surgery, maximal exercise capacity of HTX-patients after 1 year is only half that of age- and gender matched healthy subjects. Data on the evolution of muscle strength and physical activity after HTX are scant. Having this knowledge might help to optimize rehabilitation programs. Purpose To describe changes in muscle strength and physical activity following HTX. Methods 58 HTX-patients were addressed, of whom 52 (90%) patients participated in the study. Study visits were planned every 3 months from hospital discharge until 1 year of follow-up. 43 HTX-patients (67% male; age: 48 ± 14 years; BMI: 24 ± 4 kg/m²) fulfilled the study protocol. Outcome measures included functional exercise capacity (6MWD), peripheral strength (QF), respiratory muscle strength (MIP) and objectively measured physical activity (PAwalk, walking intensity (WI), PAsteps). All patients received physiotherapy at home during the first 6 weeks, as standard of care after thoracic surgery. After that, cardiac rehabilitation in a specialized center was started. Data were analyzed using repeated measures ANOVA, with Bonferroni test as post-hoc test. Results 6MWD (+178 ± 17 meter), QF (+26 ± 4 Nm) and MIP (-32 ± 3 cmH2O) significantly improved over time (p < 0.0001). Despite improvements in QF, peripheral muscle weakness was still present in 32% of patients 1 year post-HTX. A significant time effect in PA (PAwalk (+33 ± 7 minutes/day), WI (+0.036 ± 0.007 g) and PAsteps (+3711 ± 640 steps/day)) could be noticed (p < 0.0001). Sedentary time did not significantly change during follow-up (p = 0.14). (Figure 1) Conclusion Functional exercise capacity and muscle strength gradually improve during 1 year after HTX. A considerable number of patients still present with peripheral muscle weakness. Despite improvements in PA, results remain below recommended health levels. Enrollment in a cardiac rehabilitation program, offering a combined exercise and PA intervention seems warranted to further enhance health outcomes in this patient population. Abstract Figure 1

scholarly journals Inspiratory Muscle Weakness is Related to Poor Short-Term Outcomes for Heart Transplantation

2021 ◽  
Author(s):  
Isis Begot ◽  
Walter J. Gomes ◽  
Isadora S. Rocco ◽  
Caroline Bublitz ◽  
Laion R. A. Gonzaga ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Muscle Weakness ◽  
Inspiratory Muscle ◽  
Short Term

Heart transplantation in Danon disease: A single family displaying diverse phenotypes

2018 ◽  
Vol 37 (2) ◽  
pp. 312-313 ◽  
Author(s):  
Simon Poignant ◽  
Thierry Bourguignon ◽  
Fabien Espitalier
Keyword(s):  
Heart Transplantation ◽  
Single Family ◽  
Danon Disease

scholarly journals Severe Myopathy Following Heart Transplantation in a Patient with Danon Disease

2021 ◽  
Vol 40 (4) ◽  
pp. S489
Author(s):  
J. Guzman Bofarull ◽  
L. Castrillo ◽  
J. Milisenda ◽  
A. García Álvarez ◽  
M. Castel ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Danon Disease
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