Cardiomyopathy secondary to Duchenne muscular dystrophy in children
Background: Cardiomyopathy (CM) associated with Duchenne muscular dystrophy (DMD) is a commonly recognized appearance of this neuromuscular disease, significantly increased morbidity and mortality, as well as the necessity for cardiological management. CM in DMD is defined by left ventricular (LV)...
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| Format: | Article |
| Language: | English |
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Scientific Medical Association of Moldova
2021-06-01
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| Series: | The Moldovan Medical Journal |
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| Online Access: | http://moldmedjournal.md/wp-content/uploads/2021/05/moldovan-med-j-2021-64-2-rodoman-et-al-full-text.pdf |
| _version_ | 1818765182759862272 |
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| author | Iulia Rodoman Ina Palii Victoria Sacara Sergiu Gladun |
| author_facet | Iulia Rodoman Ina Palii Victoria Sacara Sergiu Gladun |
| author_sort | Iulia Rodoman |
| collection | DOAJ |
| description | Background: Cardiomyopathy (CM) associated with Duchenne muscular dystrophy (DMD) is a commonly recognized appearance of this neuromuscular
disease, significantly increased morbidity and mortality, as well as the necessity for cardiological management. CM in DMD is defined by left ventricular
(LV) systolic dysfunction and both atrial and ventricular dysrhythmias and is associated with higher mortality than other cases of pediatric dilated CMs.
Notwithstanding the high rate of cardiac involvement, patients are usually asymptomatic despite significant LV dysfunction, because of likely poor mobility
that masks the usual heart failure (HF) symptoms. Also, imagistic predictors are provided to be very helpful in defining early LV dysfunction, especially
electrocardiogram and cardiac imaging (transthoracic echocardiography, speckle-tracking, cardiac magnetic resonance) are used to detect the onset and
progression of dilated cardiomyopathy (DCM) in DMD.
Conclusions: As most DMD patients are asymptomatic for a long time of their life, so identifying predictors of HF is crucial to support these patients.
Ventricular dysfunction based on the ejection fraction (EF) measurement helps to choose therapy. In the case of early DCM (LVEF≥50%) the great
purpose is to prevent ventricular dysfunction incipience with first-line HF therapy with Angiotensin-converting-enzyme inhibitors (ACE-I) or angiotensin
receptor blockers (ARBs). Current guidelines recommend the use of conventional HF medication in case of disease progression and DCM with MidRange Reduction of LV EF (40-49%). The therapeutic approach for patients with DCM and severe ventricular dysfunction (<40%) has been studied less
profoundly and contemporary guidelines recommend all drugs used for HF treatment. |
| first_indexed | 2024-12-18T08:14:02Z |
| format | Article |
| id | doaj.art-5230432bd69544e191ad93b08fac5460 |
| institution | Directory Open Access Journal |
| issn | 2537-6373 2537-6381 |
| language | English |
| last_indexed | 2024-12-18T08:14:02Z |
| publishDate | 2021-06-01 |
| publisher | Scientific Medical Association of Moldova |
| record_format | Article |
| series | The Moldovan Medical Journal |
| spelling | doaj.art-5230432bd69544e191ad93b08fac54602022-12-21T21:14:50ZengScientific Medical Association of MoldovaThe Moldovan Medical Journal2537-63732537-63812021-06-016427078https://doi.org/10.52418/moldovan-med-j.64-2.21.13Cardiomyopathy secondary to Duchenne muscular dystrophy in childrenIulia Rodoman0https://orcid.org/0000-0003-0982-2281Ina Palii1https://orcid.org/0000-0002-4320-2951Victoria Sacara2https://orcid.org/0000-0001-9200-0494Sergiu Gladun3https://orcid.org/0000-0001-9200-0494Cardiology Unit, Institute of Mother and Child, Chisinau, the Republic of Moldova Cardiology Unit, Institute of Mother and Child, Chisinau, the Republic of Moldova Department of Pediatrics, Nicolae Testemitanu State University of Medicine and Pharmacy, Chisinau, the Republic of MoldovaCardiology Unit, Institute of Mother and Child, Chisinau, the Republic of Moldova Background: Cardiomyopathy (CM) associated with Duchenne muscular dystrophy (DMD) is a commonly recognized appearance of this neuromuscular disease, significantly increased morbidity and mortality, as well as the necessity for cardiological management. CM in DMD is defined by left ventricular (LV) systolic dysfunction and both atrial and ventricular dysrhythmias and is associated with higher mortality than other cases of pediatric dilated CMs. Notwithstanding the high rate of cardiac involvement, patients are usually asymptomatic despite significant LV dysfunction, because of likely poor mobility that masks the usual heart failure (HF) symptoms. Also, imagistic predictors are provided to be very helpful in defining early LV dysfunction, especially electrocardiogram and cardiac imaging (transthoracic echocardiography, speckle-tracking, cardiac magnetic resonance) are used to detect the onset and progression of dilated cardiomyopathy (DCM) in DMD. Conclusions: As most DMD patients are asymptomatic for a long time of their life, so identifying predictors of HF is crucial to support these patients. Ventricular dysfunction based on the ejection fraction (EF) measurement helps to choose therapy. In the case of early DCM (LVEF≥50%) the great purpose is to prevent ventricular dysfunction incipience with first-line HF therapy with Angiotensin-converting-enzyme inhibitors (ACE-I) or angiotensin receptor blockers (ARBs). Current guidelines recommend the use of conventional HF medication in case of disease progression and DCM with MidRange Reduction of LV EF (40-49%). The therapeutic approach for patients with DCM and severe ventricular dysfunction (<40%) has been studied less profoundly and contemporary guidelines recommend all drugs used for HF treatment.http://moldmedjournal.md/wp-content/uploads/2021/05/moldovan-med-j-2021-64-2-rodoman-et-al-full-text.pdfduchenne muscular dystrophycardiomyopathyheart failureneuromuscular |
| spellingShingle | Iulia Rodoman Ina Palii Victoria Sacara Sergiu Gladun Cardiomyopathy secondary to Duchenne muscular dystrophy in children The Moldovan Medical Journal duchenne muscular dystrophy cardiomyopathy heart failure neuromuscular |
| title | Cardiomyopathy secondary to Duchenne muscular dystrophy in children |
| title_full | Cardiomyopathy secondary to Duchenne muscular dystrophy in children |
| title_fullStr | Cardiomyopathy secondary to Duchenne muscular dystrophy in children |
| title_full_unstemmed | Cardiomyopathy secondary to Duchenne muscular dystrophy in children |
| title_short | Cardiomyopathy secondary to Duchenne muscular dystrophy in children |
| title_sort | cardiomyopathy secondary to duchenne muscular dystrophy in children |
| topic | duchenne muscular dystrophy cardiomyopathy heart failure neuromuscular |
| url | http://moldmedjournal.md/wp-content/uploads/2021/05/moldovan-med-j-2021-64-2-rodoman-et-al-full-text.pdf |
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