Successful Orthotopic Heart Transplantation in Patients with Becker Muscular Dystrophy

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dc.contributor.authorLevent, Ertürk
dc.contributor.authorDoğan, Eser
dc.contributor.authorAydın, Derya
dc.contributor.authorÜlger, Zülal
dc.date.accessioned2023-01-12T20:32:08Z
dc.date.available2023-01-12T20:32:08Z
dc.date.issued2021
dc.departmentN/A/Departmenten_US
dc.description.abstractCardiomyopathy is a major factor contributing to mortality and morbidity in patients with Duchenne and Becker muscular dystrophies (DMD/ BMD), and is therefore among the increasingly important findings. These X-linked recessive disorders involve the deficiency or absence of dystrophin in the skeletal muscle as well as the myocardium. This defect brings about changes in the cardiac muscle in three phases: an initial hypertrophic stage, followed by an arrhythmogenic stage, and finally end-stage dilated cardiomyopathy due to increased loss of myocytes. While cardiac involvement can be observed in carriers of BMD and DMD, the incidence of dilated cardiomyopathy is reported to be higher in BMD patients than DMD patients. The only curative treatment option for medically refractory dystrophinopathic end-stage heart failure is heart transplantation. In this report, we present two patients, 14 and 15 years of age, who presented with dilated cardiomyopathy and were diagnosed with muscular dystrophy. One of the patients remains under follow-up with a left ventricular assist device as a bridge-to-transplantation, while the other underwent successful orthotopic heart transplantation.en_US
dc.identifier.doi10.4274/jpr.galenos.2020.88609
dc.identifier.endpage101en_US
dc.identifier.issn2147-9445
dc.identifier.issn2587-2478
dc.identifier.issue1en_US
dc.identifier.startpage98en_US
dc.identifier.trdizinid507489en_US
dc.identifier.urihttps://doi.org/10.4274/jpr.galenos.2020.88609
dc.identifier.urihttps://search.trdizin.gov.tr/yayin/detay/507489
dc.identifier.urihttps://hdl.handle.net/11454/81036
dc.identifier.volume8en_US
dc.indekslendigikaynakTR-Dizinen_US
dc.language.isoenen_US
dc.relation.ispartofThe Journal of Pediatric Researchen_US
dc.relation.publicationcategoryMakale - Ulusal Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.titleSuccessful Orthotopic Heart Transplantation in Patients with Becker Muscular Dystrophyen_US
dc.typeArticleen_US

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