Effects of cardiac medications on ventricular function in patients with Duchenne muscular dystrophy-related cardiomyopathy.
Adolescent
Angiotensin-Converting Enzyme Inhibitors
/ administration & dosage
Cardiomyopathies
/ diagnosis
Child
Humans
Male
Muscular Dystrophy, Duchenne
/ diagnosis
Retrospective Studies
Stroke Volume
/ drug effects
Ventricular Dysfunction, Left
/ drug therapy
Ventricular Function
/ drug effects
Ventricular Function, Left
/ drug effects
Young Adult
ACE inhibitors
beta-blockers
dystrophinopathy
heart failure
prognosis
Journal
Muscle & nerve
ISSN: 1097-4598
Titre abrégé: Muscle Nerve
Pays: United States
ID NLM: 7803146
Informations de publication
Date de publication:
08 2021
08 2021
Historique:
revised:
22
05
2021
received:
06
09
2020
accepted:
26
05
2021
pubmed:
30
5
2021
medline:
7
9
2021
entrez:
29
5
2021
Statut:
ppublish
Résumé
The DMD Care Considerations Working Group Guidelines 2010 recommended treating cardiac dystrophinopathy with angiotensin-converting enzyme-inhibitor (ACEi) and beta-blocker (BB) therapy to prevent the progressive decline in left ventricular function expected from earlier, natural history studies. The aim of this research was to audit change in measures of left ventricular function over 8 years to 4 years before and 4 years after deploying an ACEi/BB combination systematically at a dedicated "cardiology-muscle" clinic. This is an institutionally registered, retrospective, case-file-based audit of serial echocardiographic measures of left ventricular fractional shortening accumulated over the period 1995 to 2015. Data from 104 genetically confirmed Duchenne muscular dystrophy (DMD) patients, aged 22.2 ± 5.3 years at data censure, were included. Mean age at first detection of left ventricular dysfunction was 15.1 ± 4.2 years, but older in those on maintenance steroid therapy (16.8 ± 4.2 vs 14.5 ± 4.1 years; P = .04). Group mean fractional shortening fell by 1.5%/year over the 4 years before therapy, but this decreased to 0.9%/year over the first 4 years after starting therapy. Analysis of limited left ventricular ejection fraction measures showed similar but nonsignificant changes. Neither age at detection of left ventricular dysfunction nor fractional shortening percent at time of therapy initiation affected the beneficial response. The results support the international DMD recommendations of the time. This combination of cardiac medications helps stabilize heart function. For the best long-term effects, therapy needs to be initiated no later than on first detection left ventricular impairment.
Substances chimiques
Angiotensin-Converting Enzyme Inhibitors
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
163-171Subventions
Organisme : British Heart Foundation
ID : SP/05/001/18616
Pays : United Kingdom
Informations de copyright
© 2021 Wiley Periodicals LLC.
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