Left ventricular diastolic function in congenital myotonic dystrophy

OBJECTIVE Examination of left ventricular function and conduction abnormalities in myotonic dystrophy. DESIGN Twelve patients (median age, 13.7 years) with myotonic dystrophy had detailed electrocardiography and echocardiography performed. Echocardiographic parameters were compared with body surface...

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Published in:Archives of disease in childhood 1999-03, Vol.80 (3), p.267-270
Main Authors: Bu’Lock, F A, Sood, M, De Giovanni, J V, Green, S H
Format: Article
Language:English
Subjects:
Adolescent
Adult
Biological and medical sciences
Case-Control Studies
Child
Child, Preschool
Congenital diseases
Control Groups
Correlation
Diastole
diastolic filling
Diseases of striated muscles. Neuromuscular diseases
Doppler echocardiography
Doppler effect
Dystrophy
Echocardiography, Doppler
Electrocardiography
Electrocardiography, Ambulatory
Female
Heart
Heart Disorders
Heart rate
Humans
Male
Medical sciences
Motor Development
Musculoskeletal system
Myotonia Congenita - diagnostic imaging
Myotonia Congenita - physiopathology
myotonic dystrophy
Neurology
Original
Patients
Pregnancy
Regression Analysis
Scientific Concepts
Statistical Analysis
Statistics, Nonparametric
Ventricular Dysfunction, Left - physiopathology
Young adults
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creator Bu’Lock, F A
Sood, M
De Giovanni, J V
Green, S H
description OBJECTIVE Examination of left ventricular function and conduction abnormalities in myotonic dystrophy. DESIGN Twelve patients (median age, 13.7 years) with myotonic dystrophy had detailed electrocardiography and echocardiography performed. Echocardiographic parameters were compared with body surface area (BSA) matched median normal values. RESULTS Fractional shortening was slightly reduced (by 28–29%) in three patients and three patients had mild mitral valve prolapse. Diastolic function was abnormal; isovolumic relaxation time (IVRT) and duration of early filling were prolonged compared with control values (median IVRT, 74v 61 ms). Peak E velocity was increased (median, 0.82 v 0.78 m/s) but atrial phase filling was normal. Heart rate was reduced (median, 68v 81 beats/min). Conduction abnormalities were common but showed no clear relations with diastolic abnormalities. CONCLUSIONS Young patients with myotonic dystrophy have myocardial diastolic dysfunction as well as abnormal electrophysiology. The prognostic implications of such abnormalities require further study.
doi_str_mv 10.1136/adc.80.3.267
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DESIGN Twelve patients (median age, 13.7 years) with myotonic dystrophy had detailed electrocardiography and echocardiography performed. Echocardiographic parameters were compared with body surface area (BSA) matched median normal values. RESULTS Fractional shortening was slightly reduced (by 28–29%) in three patients and three patients had mild mitral valve prolapse. Diastolic function was abnormal; isovolumic relaxation time (IVRT) and duration of early filling were prolonged compared with control values (median IVRT, 74v 61 ms). Peak E velocity was increased (median, 0.82 v 0.78 m/s) but atrial phase filling was normal. Heart rate was reduced (median, 68v 81 beats/min). Conduction abnormalities were common but showed no clear relations with diastolic abnormalities. CONCLUSIONS Young patients with myotonic dystrophy have myocardial diastolic dysfunction as well as abnormal electrophysiology. The prognostic implications of such abnormalities require further study.</description><identifier>ISSN: 0003-9888</identifier><identifier>EISSN: 1468-2044</identifier><identifier>DOI: 10.1136/adc.80.3.267</identifier><identifier>PMID: 10325709</identifier><identifier>CODEN: ADCHAK</identifier><language>eng</language><publisher>London: BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health</publisher><subject>Adolescent ; Adult ; Biological and medical sciences ; Case-Control Studies ; Child ; Child, Preschool ; Congenital diseases ; Control Groups ; Correlation ; Diastole ; diastolic filling ; Diseases of striated muscles. Neuromuscular diseases ; Doppler echocardiography ; Doppler effect ; Dystrophy ; Echocardiography, Doppler ; Electrocardiography ; Electrocardiography, Ambulatory ; Female ; Heart ; Heart Disorders ; Heart rate ; Humans ; Male ; Medical sciences ; Motor Development ; Musculoskeletal system ; Myotonia Congenita - diagnostic imaging ; Myotonia Congenita - physiopathology ; myotonic dystrophy ; Neurology ; Original ; Patients ; Pregnancy ; Regression Analysis ; Scientific Concepts ; Statistical Analysis ; Statistics, Nonparametric ; Ventricular Dysfunction, Left - physiopathology ; Young adults</subject><ispartof>Archives of disease in childhood, 1999-03, Vol.80 (3), p.267-270</ispartof><rights>Royal College of Paediatrics and Child Health</rights><rights>1999 INIST-CNRS</rights><rights>Copyright: 1999 Royal College of Paediatrics and Child Health</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-b507t-ccb73c82ed7658d61262da8d5b1eb35b8e399b97e58a12855b7a28be5632e4533</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.proquest.com/docview/1778083264/fulltextPDF?pq-origsite=primo$$EPDF$$P50$$Gproquest$$H</linktopdf><linktohtml>$$Uhttps://www.proquest.com/docview/1778083264?pq-origsite=primo$$EHTML$$P50$$Gproquest$$H</linktohtml><link.rule.ids>230,314,723,776,780,881,21357,21373,27901,27902,33588,33589,33854,33855,43709,43856,53766,53768,73964,74140</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&amp;idt=1714437$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/10325709$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Bu’Lock, F A</creatorcontrib><creatorcontrib>Sood, M</creatorcontrib><creatorcontrib>De Giovanni, J V</creatorcontrib><creatorcontrib>Green, S H</creatorcontrib><title>Left ventricular diastolic function in congenital myotonic dystrophy</title><title>Archives of disease in childhood</title><addtitle>Arch Dis Child</addtitle><description>OBJECTIVE Examination of left ventricular function and conduction abnormalities in myotonic dystrophy. DESIGN Twelve patients (median age, 13.7 years) with myotonic dystrophy had detailed electrocardiography and echocardiography performed. Echocardiographic parameters were compared with body surface area (BSA) matched median normal values. RESULTS Fractional shortening was slightly reduced (by 28–29%) in three patients and three patients had mild mitral valve prolapse. Diastolic function was abnormal; isovolumic relaxation time (IVRT) and duration of early filling were prolonged compared with control values (median IVRT, 74v 61 ms). Peak E velocity was increased (median, 0.82 v 0.78 m/s) but atrial phase filling was normal. Heart rate was reduced (median, 68v 81 beats/min). Conduction abnormalities were common but showed no clear relations with diastolic abnormalities. CONCLUSIONS Young patients with myotonic dystrophy have myocardial diastolic dysfunction as well as abnormal electrophysiology. 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DESIGN Twelve patients (median age, 13.7 years) with myotonic dystrophy had detailed electrocardiography and echocardiography performed. Echocardiographic parameters were compared with body surface area (BSA) matched median normal values. RESULTS Fractional shortening was slightly reduced (by 28–29%) in three patients and three patients had mild mitral valve prolapse. Diastolic function was abnormal; isovolumic relaxation time (IVRT) and duration of early filling were prolonged compared with control values (median IVRT, 74v 61 ms). Peak E velocity was increased (median, 0.82 v 0.78 m/s) but atrial phase filling was normal. Heart rate was reduced (median, 68v 81 beats/min). Conduction abnormalities were common but showed no clear relations with diastolic abnormalities. CONCLUSIONS Young patients with myotonic dystrophy have myocardial diastolic dysfunction as well as abnormal electrophysiology. The prognostic implications of such abnormalities require further study.</abstract><cop>London</cop><pub>BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health</pub><pmid>10325709</pmid><doi>10.1136/adc.80.3.267</doi><tpages>4</tpages><oa>free_for_read</oa></addata></record>
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ispartof Archives of disease in childhood, 1999-03, Vol.80 (3), p.267-270
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1468-2044
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source Social Science Premium Collection; PubMed Central; Education Collection
subjects Adolescent
Adult
Biological and medical sciences
Case-Control Studies
Child
Child, Preschool
Congenital diseases
Control Groups
Correlation
Diastole
diastolic filling
Diseases of striated muscles. Neuromuscular diseases
Doppler echocardiography
Doppler effect
Dystrophy
Echocardiography, Doppler
Electrocardiography
Electrocardiography, Ambulatory
Female
Heart
Heart Disorders
Heart rate
Humans
Male
Medical sciences
Motor Development
Musculoskeletal system
Myotonia Congenita - diagnostic imaging
Myotonia Congenita - physiopathology
myotonic dystrophy
Neurology
Original
Patients
Pregnancy
Regression Analysis
Scientific Concepts
Statistical Analysis
Statistics, Nonparametric
Ventricular Dysfunction, Left - physiopathology
Young adults
title Left ventricular diastolic function in congenital myotonic dystrophy
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