Hereditary muscular dystrophies and the heart

Hermans, Mieke C. E.; Pinto, Yigal M.; Merkies, Ingemar S. J.; Die-Smulders, C.E.M. de; Crijns, Harry J.G.M.; Faber, Catharina G.

doi:10.1016/j.nmd.2010.04.008

Cited by 170 publications

(157 citation statements)

References 163 publications

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“…Her skal nevnes Emery-Dreifuss' muskeldystrofi, som også er sjelden, men som, for øvrig i likhet med skulder-hofte-muskeldystrofi 1B, innebaerer høy risiko for alvorlig hjerteaffeksjon (11). Emery-Dreifuss' muskeldystrofi arves ofte X-bundet.…”

Section: Andre Muskeldystrofier Med Debut Etter Spedbarnsalderunclassified

Muskelsykdommer med debut i barnealder

Aden¹,

Annexstad²,

Lien³

et al. 2017

Tidsskriftet

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Section: Andre Muskeldystrofier Med Debut Etter Spedbarnsalderunclassified

Muskelsykdommer med debut i barnealder

Aden¹,

Annexstad²,

Lien³

et al. 2017

Tidsskriftet

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“…Kalp kasındaki etkilenme kardiyomiyopati ve atriyoventriküler iletim defektlerine yol açar. 4,5 Musküler distrofili hastalarda miyokard disfonksiyonu, hastaları volatil anesteziklerin miyokardiyal depresan etkilerine daha duyarlı kılar. Anestezi indüksiyonu sırasında süksinilkolin, halotan ya da diğer inhalasyon anesteziklerinin kullanımı sonrasında hiperkalemi, rabdomiyoliz ve kardiyak arrest gibi ciddi perioperatif komplikasyonların geliştiği bildirilmiştir.…”

Section: Limb Girdle Musküler Distrofili Hastadaunclassified

Anesthetic Management in a Patient with Limb Girdle Muscular Dystrophy: Case Report

Sergi̇n¹,

Kocabas²,

Aşkar³

et al. 2015

Turkiye Klinikleri J Anest Reanim

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“…Additionally, 10 of the 16 ECHOs were noted to be technically difficult. There was no difference in mean (SD) age (27 [7] vs. 32 [9] years, p ¼ 0.25) or duration of assisted ventilation (9.8 [6.4] vs. 10.9 [6.9] years, p ¼ 0.32) between 5 patients with normal LV function and 11 patients with reduced LV function. One of the five patients with normal LV function was female.…”

Section: Ecg and Echo Findingsmentioning

confidence: 99%

“…7 The most common abnormalities found on electrocardiography include tachyarrhythmia, tall R waves or R/S ratio >1 in leads V 1 -V 3 , large Q waves in lateral leads, complete or incomplete left bundle branch block (LBBB) and complete right bundle branch block (RBBB). 8,9 Several studies have found that nearly all patients with DMD develop cardiomyopathy, although the age of onset varies considerably. 7,10,11 The structural evidence of cardiomyopathy precedes symptoms by years.…”

Section: Introductionmentioning

confidence: 99%

Cardiac Management of Ventilator-Assisted Individuals with Duchenne Muscular Dystrophy

et al. 2014

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As life expectancy of patients with Duchenne muscular dystrophy (DMD) has increased to the 5th decade, in part due to improved ventilatory support, cardiomyopathy is projected to increase as a cause of death. International guidelines recommend an annual assessment of cardiac function and initiation of appropriate pharmacological treatment. We conducted an audit of the cardiac management in patients with DMD requiring ventilatory support and reported a case series of the collated cardiac investigations. Patients with DMD requiring ventilatory support were included in the study. The date of the last electrocardiogram (ECG), echocardiogram (ECHO), cardiology review and pharmacological management were retrieved from the medical records. If an annual cardiac assessment had not been performed this was requested and the latest ECGs and ECHO reports were collated. A total of 30 patients with DMD (29 males, mean (SD) age of 30 (7) years) met the inclusion criteria. Although there was ECG and ECHO documentation in 24 and 21 individuals, respectively, it was only recent in 10 and 6 individuals. In all, 60% of patients had been assessed by a cardiologist, but only 10% within the last year. Over half of the patients failed to attend their new appointments. From the available results, 18 of the 19 patients had an abnormal ECG, 11 of the 16 patients had left ventricular (LV) impairment and 55% of patients had a change in prescription following cardiac investigations. There is a need for a coordinated cardiorespiratory approach towards adult patients with DMD. Over a third of patients had normal LV function suggesting that cardiomyopathy is not inevitable in this group.

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Hereditary muscular dystrophies and the heart

Cited by 170 publications

References 163 publications

Muskelsykdommer med debut i barnealder

Muskelsykdommer med debut i barnealder

Anesthetic Management in a Patient with Limb Girdle Muscular Dystrophy: Case Report

Cardiac Management of Ventilator-Assisted Individuals with Duchenne Muscular Dystrophy

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