myotonic dystrophy and the heart...• presyncope/ syncope • dysautonomia or autonomic...
TRANSCRIPT
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Myotonic Dystrophy and The Heart
HOWARD M. ROSENFELD, MD FACC
Chief Division of Pediatric Cardiology
Children’s Hospital and Research Center Oakland
Pediatric Cardiology Medical Group-East Bay, Inc
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Overview
• Cardiac Anatomy/ Function
• Cardiac testing
• Cardiac Manifestations/ Symptoms
• Rhythm Disturbances
• & Management
• “level playing field”
• Myotonic Dystrophy I and II
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Leonardo da vinci 1510
Egyptian god of the dead, Anubis weighing
a heart/soul
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Heart as a pump
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Cardiac Electrical System
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Department of Musculoskeletal Biology, Institute of Ageing & Chronic Disease, University of Liverpool
Cardiac Electrical System
Iodine absorbtion micro CT scanner
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ECG/EKG
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EKG Elements“Sinus beat”
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Rhythm• Sinus rhythm requires 1) P wave preceding every QRS
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Rhythm• Sinus rhythm requires 2) QRS following each P wave
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Axes
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Intervals
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FORCES
• Ventricular hypertrophyleft sided
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Holter monitoring
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Event monitoring
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Echocardiogram
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Echocardiography:Physics• Sound travels in waves
of compression and decompression through a transmitting medium (water, tissue)
• These high frequency sound waves travel in straight lines and are either reflected or transmitted at changes in medium
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Echo “Planes”• The plane of the transducer
determines the “cut” of heart imaged
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Valve Pathology
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Echocardiography funciton
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Echocardiography funciton
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Free breathing cine imaging LV short axis stack
MRI for cardiac funciton/morphology
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Catheterization
Werner Forssman 1929
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Catheterization
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Electrophysiology testing
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MD: Cardiac Manifestations
• Pump related: Dilated Cardiomyophathy- progressive muscle weakness leading to cardiac enlargement and congestive heart failure (pump failure) or arrhythmia
• Electrical related: Conduction systemic disease- atrial arrhythmias, complete conduction block or ventricular fibrillation/ tachycardia leading to sudden cardiac death
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Symptoms of arrhythmia
• Palpitations
• shortness of breath/exercise intolerance
• dizziness/lightheadedness
• presyncope/ syncope
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• Dysautonomia or Autonomic dysfunction: Included in the differential with hypotension, postural orthostatic tachycadia (POTS), or vasovagal dizziness/syncope
Symptoms of arrhythmia
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Single Extra-beats
PAC-premature atrial complex: conducted on non conducted
PVC-premature ventricular complex
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• Premature atrial beats with block
Slow Cardiac rhythms
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• first degree AV block- PR prolongation
Atrioventricular block-types:
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• second degree AVB- dropped beats
Mobitz type 1 Wenckebach
Mobitz type 2
Atrioventricular block-types:
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• Third degree AV block-complete heart block
Atrioventricular block-types:
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• Atrial flutter
Fast Cardiac Rhythms
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• Atrial flutter with 1:1 conduction and aberrancy
Fast Cardiac Rhythms
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Fast Cardiac Rhythms• Atrial flutter- 2:1 block
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• Atrial fibrillation
Fast Cardiac Rhythms
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• Atrial fibrillation
Fast Cardiac Rhythms
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• atrial flutter: cardioversion
Fast Cardiac Rhythms
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• ventricular tachycardia
Fast Cardiac Rhythms
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• perfusing ventricular rythm
Fast Cardiac Rhythms
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Fast Cardiac RhythmsNonperfusiong ventricurlar ryhthm
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Rhythm Disturbances• Prolongation of the corrected QT interval
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Cardiac Evaluation• Annual Cardiology visit
• Annual EKG
• Holter monitoring as needed
• Echocardiography (every 2-5 years or as needed based on functional concerns)
• Catheterization
• Electrophysiology Testing
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Arrhythmia and Sudden death in MD
• Normal EKG = low risk for sudden cardiac death over a 5 year period
• Arrhythmias in younger patients more frequently tachyarrhythmias than conduction block
• Endpoints of Sudden cardiac death or pacemaker implantation:
• associated with prolonged baseline PR interval & QTc
• Looser association- advanced age/muscle imapirment.
• No association with number of CTG repeats
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Pacemaker & AICD
Pacemaker-slow rhythms and block
Defibrillator- slow rhythms/block & fast rhythms
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• 406 adult MD1 patients
• 5.7 year follow-up 27 sudden deaths
• SD Associated with
• Severe EKG abnormality: non-sinus rhythm, PR greater than 240msec, QRS interval greater than 120msec, 2nd or 3rd degree AVB
• Atrial tachyarrhythmias
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Indication for pacemaker therapy• 2nd and 3rd degree AV block
Class I indication: Condition in which permanent pacing is definitely beneficial, useful, and effective. Implantation of a cardiac pacemaker is acceptable and necessary.
• 1st degree AV block Class IIB indication: Condition in which the usefulness, efficacy of permanent pacing is less well established by evidence/opinion.
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• Is an invasive strategy with electrophysiolgic studies and prophylactic permanent pacing in MD1 patients with infranodal conduction delays superior to a noninvisive stragety?
• Conclusion: Among patients with MD1, an invasive strategy was associated with a higher rate of 9 year survival when compared with a noninvasive strategy.
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Defibrillator Therapy
• Secondary prevention: preventing sudden cardiac death following the survival of an initial event
• Primary prevention: Preventing sudden cardiac death before the occurance of an initial event
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• Study: Assessment in MD1 of implant rates, indications, and outcomes for patients receiving pacemakers or implantable cardioverter-defibrillators
• Conclusion: MD1 patients commonly receive antiarrhythmic devices. The risk of VT/VF and sudden death suggests that AICDs rather than pacemakers should be considered for these patients.
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What can you do??
• Be aware of symptoms of heart disease: fatigue, SOB, CP, palpitations, dizziness and syncope
• Regular EKG and cardiologist involvement
• Be knowledgable and a good self advocate regarding cardiac disease
• Research therapeutic options carefully
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Flecainide in MD??
Type 1C - sodium channel blocker
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• Ultrasound = sound waves like audible sound
• Audible sound 15-20 kilohertz (15-20,000 cycles/second)
• Medical ultrasound 1-12 megahertz (1-12,000,000 cycles/second)
Physics of echocardiography
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• “Planes” of sound cut through the heart to provide slices of anatomy
• Wavelengths, less than a millimeter, are capable of resolving fine anatomic structures
Echo Imaging
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Basic Principles:EKG
• EKG elements• Rate• Axes• Rhythm• Intervals• forces
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Rhythm• Sinus rhythm requires 3) Appropriate P wave axis
Sinus axis
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Rate
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• atrial flutter with 2:1 block
Repeat after emesis
Fast Cardiac Rhythms
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• Complete heart block
Slow Cardiac rhythms