Cardiomyopathies

Definition :

- A Group of disorders that :

 Primarily involve the myocardium

Are not the result of : Congenital , valvular , pericardial , hypertensive or ischemic diseases

BY : Mahmoud Reda Sewilam

Dilated cardiomyopathy Hypertrophic cardiomyopathy Restrictive cardiomyopathy

Etiology 1- Primary : idiopathic , familial 1- Primary : idiopathic , familial 1- Primary :
2- Secondary : 2- secondary : pheochromocytoma  endomyocardial fibrosis , endomyocardial eosinophilic disease
 Infective : especially coxsakie B virus ( Loffler’s endocarditis )
 Infiltration & granulomas : amyloidosis , hemochromatosis , sarcoidosis 2- secondary :
 Immunologic : SLE , scleroderma  Infiltration & granulomas : amyloidosis , hemochromatosis , sarcoidosis
 Iatrogenic & toxic : drugs ( doxorubicin ) , toxins ( alcohol )  Immunologic : scleroderma
 Endocrinal : DM , acromegaly , myxedema
 Peripartum cardiomyopathy
 Neurological : Duchenne myopathy , Friedreich’s ataxia
Pathophysiology  Impaired systolic function ( ↓ contractility ) Asymmetric LV hypertrophy ( SWT > FWT ) , leading to : Myocardial fibrosis or infiltration :
 Biventricular dilatation .  Impaired diastolic function ( ↓ compliance ) → pulmonary congestion  The RV is involved more that the LV
- In approximately 30 % of patients with DCM :  Obstruction of the LV outflow tract , causing low CO  Impaired diastolic function ( ↓ compliance ) → venous congestion
There is an abnormality in heart’s electrical conducting system  The septum may bulge into the RV cavity , causing giant a wave  Mildly impaired systolic function ( ↓ contractility ) → low co
( called an “ intraventricular conduction delay “ or bundle branch block  The condition functionally resembles : constrictive pericarditis
Clinical picture Manifestation of : Biventricular failure  Symptoms : pulmonary congestion , low cop Manifestation of systemic congestion and low COP
Manifestation of : complications :  General signs : giant a wave , jerky pulse Manifestation of : complications :
 Embolic manifestation : systemic & pulmonary  Cardiac signs : LVH , systolic ejection murmur along the sternal border  Embolic manifestation : systemic & pulmonary
 Arrhythmias e.g. AF , VT  Complications : arrhythmias eg.: ( AF,VT ) , sudden death  Arrhythmias e.g. AF , VT
 Sudden death
Investigations - ECG : intraventricular conduction delay : BBB - Echocardiography : - Cardiac catheterization & angiocardiography : Square root sign
- Echocardiography : * size : Asymmetric LV hypertrophy ( SWT > FWT ) - Investigation of the cause : endomyocardial biopsy eg : for infiltration ,
* size : Biventricular dilatation * contractility : ↑ contractile function of the LV granuloma , fibrosis
* contractility : impaired global contractile function
-other investigations for the cause : e.g. serology for SLE
DD DD : constrictive pericarditis :
RCM CP
Pericardial knock : absent may be present
Pericardial calcification : absent may be present
CT & MRI : --------- pericardial thickening
Endomyocardial biopsy : diagnostic -------------------
Exploratory thoracotomy : diagnostic diagnostic
TTT 1- TTT of HF : eg. : ACEIs , BB , Diuretics , CRT 1- TTT of HF but avoid positive inotropics 1- TTT of HF : eg. : ACEIs, Diuretics
2- TTT of complications eg. : anti-arrhythmic drugs for arrhythmias 2- TTT of complications eg. : anti-arrhythmic drugs for arrhythmias 2- TTT of complications eg. : anti-arrhythmic drugs for arrhythmias
3- TTT of cause e.g immunosuppressive drugs in SLE 3- CCBs (verapamil ) or BB ( propranolol ) : to relax the septum 3- TTT of cause e.g immunosuppressive drugs in scleroderma
4- Cardiac transplantation in terminal refractory cases . 4- Surgical TTT : resection of the hypertrophied septum 4- Cardiac transplantation in terminal refractory cases .
 Supraventricular tachycardia Atrial Flutter Ventricular Tachycardia
Def. It is an tachy-arrhythmia originating from above the ventricle - A tachycardia in which the atria discharge at a regular rapid rate : 240-440 / min -It is an arrhythmia originating from the ventricle that presents with :
- a physiological block occurs in the AVN ( 2:1 or 3:1 or 4:1 block ) Rapid regular tachycardia at rate of 120 – 250 / min.
- therefore only ½ or 1/3 or ¼ of the atrial impulses will pass to the ventricles -Since there is no retrograde conduction in the AVN ,
- the average ventricular rate will be ½ or 1/3 or ¼ the atrial rate there will be AV dissociation :
- the block may be : *The ventricles will be controlled by the ventricular focus ( VR 120-250/mi
* fixed e.g. 2:1 * the atria will be controlled by the SAN : ( AR : 60 – 100 / min )
* variable e.g. changing from : 2:1 or 3:1 or 4:1 … etc …
3- Special types : see later
Types 1- Atrial tachycardia : the arrhythmia originates from the atria 1- Type I ( common , typical ) : the atrial rate is 240 – 340 / min = 300/min 1- Sustained : persists more than 30 sec.
2- Nodal tachycardia : the arrhythmia originates from the AVN 2- Type II ( rare ) : : the atrial rate is 340 – 440 / min = 400/min or causes hemodynamic instability
2- Non sustained : persists less that 3o sec.
With no hemodynamic instablity
Etiology It is usually in a normal heart & therefore it’s presence does not always an organic heart disease It usually occurs in a patient with organic heart disease, It’s v v v rarely occurs in a normal heart , & therefore it’s presence almost
1- Physiological : occurs in normal heart ( no organic heart disease ) however , it may occur in a normal heart. denotes an organic heart diseas
2- Pathological : organic heart disease 1- Physiological : may occurs in normal heart ( no organic heart disease )
 CAD especially AMI 2- Pathological : organic heart disease … = OHD NOOO physiological
 Cardiomyopathy and myocarditis 3- Pharmacological : …
 Congenital heart disease 1- Pathological : organic heart disease …
 Rheumatic heart disease 2- Pharmacological : …
 Hypertension
 Hyperthyroidism
 Pre-excitation syndromes : WPW syndrome
3- Pharmacological :
 Digitalis
 Sympathomimetic drugs
 Some anti-arrhythmic drugs e.g. class I C
BY : Mahmoud Reda Sewilam

AF = atrial fibrillation ES = Premature beats Nodal ( Junctional ) rhythm

D -A form of tachycardia in which the atria discharge at rate : 400 – 600 / min -They are ectopic cardiac impulses occurring before the expected - abnormal heart rhythm , where the AVN initiates electrical activity of the heart
- an irregular block occurs in the AVN allowing only some impulses to pass to Sinus impulse causing premature beats . -the impulses spread up & down to activate the atria & the ventricles simultaneously
the ventricles in an irregular manner -when the normal sinus impulse arises , the heart will not respond -there are 2 possibilities :
- therefore, the ventricular beats will be : it will be in the refractory period 1-Nodal tachycardia ( discussed before )
* Rhythm : markedly irregular -these ectopic cardiac impulses may arise : -abnormal automaticity in the AVN overtakes the normal SAN
* Rate : 100 – 160 / min *supraventricular ( from the atria or AVN ) OR 2-Nodal rhythm ( discussed below )
* force : variable *ventricular ( from the ventricles ) -An escape rhythm in which the AVN becomes the pace-maker of the heart discharging at a rate of 40 – 60 /min
Types : AHA / ACC / ESC : see later In cases of :
E 1 - Primary ( idiopathic ) “ LONE AF “ ( not secondary to any disease 1- physiological : : may occurs in normal heart ( no OHD )  Severe bradycardia : when the SAN discharges at rate slower than the intrinsic AVN pacemaker
-it’s more common in old age and may be slow .  Emotions  Heart block : conduction problem between the SAN & the AVN
2-pathological ( secondary to different diseases ) :  Excessive : smoking , coffee , tea E : It usually occurs in a patient with organic heart disease,
 Chest disease , especially COPD 2- Pathological : organic heart disease … however , it may occur in a normal heart.
 Constrictive pericarditis  Hypokalemia 1- Physiological : may occurs in normal heart ( increase vagal tone during sleep )
 Congenital heart disease especially ASD Nooo Pre-excitation syndromes : WPW syndrome 2- Pathological : organic heart disease
 Rheumatic heart disease especially MS  CAD especially AMI , inferior .
 … 3- Pharmacological : …  Cardiomyopathy and myocarditis
3- Pharmacological : …  Sick sinus syndrome
3- Pharmacological : * Digitalis . ** B – Blockers
 Sinus tachycardia Sinus bradycardia
Definition The SAN discharges at rapid rate > 100 / min The SAN discharges at slow rate < 60 / min
Etiology 1- Physiological : 1- Physiological :
 Exercise , Emotions , pregnancy , infancy .  Athletes , Asleep .

2- Pathological : 2- Pathological :
 Anemia , hypotension , hypovolemia  Hypothermia , Hypothyroidism
 Pulmonary embolism , fever , shock .  ↑ vagal stimulation eg. Vasovagal syncope
 Heart failure , myocarditis  ↑ intracranial tension eg. Brain tumours
 Hyperdynamic circulation , hyperthyroidism  Cholestasis : obstructive jaundice

3- Pharmacological : 3- Pharmacological :
 Sympathomimetic drugs : eg adrenaline  Parasympathomimetics drugs: eg neostigmine
 Parasympatholytic drugs : eg. Atropine  Sympatholytic drugs : eg B-blockers
 CCBs : eg. : nifedipine  CCBs : eg. : verapamil & diltiazem
 Alcohol , nicotine , caffeine , thyroid hormones .  Digitalis .

Symptoms 1- Asymptomatic 1- Asymptomatic

2- Palpitation : rapid , regular , gradual onset , gradual offset 2- Palpitation : slow , regular , gradual onset , gradual offset
3- symptoms of : Low COP 3- symptoms of : Low COP
4- precipitation of angina & HF in susceptible patients 4- precipitation of angina & HF in susceptible patients
Signs : PULSE Rhythm : regular Rhythm : regular
Rate : 100 - 180 / min Rate : < 60 / min ( 50 – 60 / min , or less )
Response to carotid massage : Response to carotid exercise :
 Gradual slowing of the rate .  Gradual acceleration of the rate .
 Gradual acceleration : to the original rate on stopping massage  Gradual slowing : to the original rate on stopping exercise
NECK VEINS Normal rapid waves with the same rate of pulse Normal slow waves with the same rate of pulse
AUSCULTATION Accentuated S1 Normal or weak S1
ECG : QRS Rhythm : regular Rhythm : regular
Rate : 100-180 / min Rate : 50 – 60 / min , ( or less )
Duration : normal Duration : normal
BY : Mahmoud P wave normal Normal
TTT 1- TTT of the cause , eg. Antithyroid drugs for hyperthyroidism 1- TTT of the cause , eg. L-thyroxin for hypothyroidism
Reda 2- Sedative : may be needed 2- Atropine : may be needed
3- B-Blockers eg. Propranolol , in severe cases
Sewilam

Special types of ventricular tachycardia AF = atrial fibrillation , Types : AHA / ACC / ESC :
1- Torsade de points : The AHA , ACC & ESC recommended in their guidelines the following classification system based on : Timing & Termination
 QRS complexes changes continuously & rapidly & irregularly from an upright to an inverted position ( twisting of points ) AF category : defining characteristics :
 Causes include AMI , ↓ K , ↓ Ca  First detected ( acute ) : only one diagnosed episode ( < 48 hours in duration )
 It is serious & may cause VF & sudden death  Paroxysmal : recurrent episodes that self –terminate in < 7 days ( without need for cardioversion )
 Persistent : : recurrent episodes that self –terminate in > 7 days ( not self-terminating; with need for cardioversion ,
chemical or electrical cardioversion
 Permanent : long term AF > 7 days , in which cardioversion either failed or was not attempted .
2- Accelerated Idio – Ventricular rhythm The AHA , ACC & ESC guidelines also describe AF categories in terms of : Etiological considerations :
 An ectopic ventricular pacemaker discharges at a rate 60 – 120 / min , and  Long atrial fibrillation ( LAF ) : absence of cardiac or any other disease
controls the ventricles only resulting in slowing VT  Secondary AF : secondary to different diseases
 Causes include : AMI , post-coronary thrombolysis ( reperfusion arrhythmias )  Valvular AF : presence of RHD
 It is transient & rarely causes hemodynamic disturbances .  Non – valvular AF : absence of RHD
NB : ACC = American College of Cardiology , AHA = American Heart Association , ESC = European Society of Cardiology
Clinical picture Supraventricular tachycardia Atrial Flutter Ventricular Tachycardia
Symptoms -Palpitation : rapid , regular , sudden onset , sudden offset -Palpitation : rapid , regular ( or irregular ) , sudden onset , sudden offset ( or → AF ) -Palpitation : rapid , regular , sudden onset , sudden offset
-symptoms of : Low COP -symptoms of : Low COP -symptoms of : Low COP & shock are more common
- precipitation of angina & HF in susceptible patients - precipitation of angina & HF in susceptible patients - precipitation of angina & HF in susceptible patients
-sudden death : if converted to ventricular fibrillation ( VF )
Signs : PULSE Rhythm : regular Rhythm : regular ( fixed block ) , irregular ( variable block ) Rhythm : regular
Rate : 120 – 250 / min Rate : 150 or 100 or 75 / min ( according to AV block ) Rate : 120 – 250 / min
Response to carotid massage : sudden disappearance of the arrhythmia may occur Response to carotid massage : ( ↑ AV block from 2:1 → 3:1 → 4:1) : Response to carotid massage : no response
*stepwise slowing : of the rate ( 150 → 100 → 75 / min ) No vagal supply to ventricles
*stepwise acceleration : to the original rate on stopping massage
NECK VEINS -in atrial tachycardia : normal rapid waves with the same rate of pulse Multiple a waves : double or triple or quadriple the rate of pulse - a waves : normal rate ( 60 – 100 / min ) & less than the pulse rate
-in nodal tachycardia : regular cannon waves with the same rate of pulse - occasional cannon waves
AUSCULTATION -in atrial tachycardia : accentuated S1 accentuated S1 - occasional cannon sounds
-in nodal tachycardia : regular cannon sounds - variable intensity of S1
ECG : QRS Rhythm : regular Rhythm : regular ( fixed block ) , irregular ( variable block ) Rhythm : regular
Rate : 120 – 250 / min Rate : 150 or 100 or 75 / min ( according to AV block ) Rate : 120 – 250 / min
Duration : normal Duration : normal Duration : wide
P wave -in atrial tachycardia : deformed - replaced by : multiple flutter waves at a rate of 240 – 340 / min - May not appear
-in nodal tachycardia : absent or inverted Typically : saw – tooth appearance - normal in rate ( 60 – 100 / min ) & shape
- comes before , after or hidden by the QRS ( AV dissociation )
BY : Mahmoud Reda Sewilam , mr.sewilam@gmail.com ‫ال تنسوني من صالح دعائكم‬

Clinical picture AF = atrial fibrillation ES = Premature beats Nodal ( Junctional ) rhythm

Symptoms -Palpitation : rapid , irregular , sudden onset , sudden offset - Asymptomatic - Asymptomatic
-symptoms of : Low COP - palpitation : irregular -Palpitation : regular
- precipitation of angina & HF in susceptible patients -symptoms of : Low COP
Complications : atrial thrombosis & embolization - precipitation of angina & HF in susceptible patients
Signs : PULSE - Rhythm : - Rhythm : Rhythm : regular
* marked irregularity ( in rhythm & volume ) * occasional irregularity Rate : 40 – 60 / min
*pulse deficit : > 10 beats / min *pulse deficit : < 10 beats / min Response to exercise :
- Rate : 100 – 160 / min - Rate : variable according to the sinus rhythm ( ↑,↓ or normal) - Reversion to sinus rhythm may occur in some cases
Causes of slow AF : -Response to exercise : variable
Drugs : digitalis or B- blockers ↓ irregularity : due to shortened diastolic period
Lone AF ↑ irregularity : due to sympathetic stimulation
Associated heart block NECK VEINS: Normal waves with occasional irregularity
-Response to carotid massage : AUSCULTATION : Normal sounds with occasional irregularity
Gradual slowing : due to ↓ AV conduction ECG
-Response to exercise : :- Rhythm : irregular (occasional irregularity ) , but : sinus .
↑ irregularity : due to ↑ AV conduction -the premature beat comes early & is followed by a compensatory pause :
NECK VEINS a waves : absent *1- supraventricular ( from the atria or AVN ) : Regular cannon waves with the same rate of pulse
systolic expansion Atrial beats : deformed p wave followed by a normal QRS
Nodal beats : absent or inverted p wave , a normal QRS
AUSCULTATION - variable intensity of S1 Regular cannon sounds
ECG : QRS Rhythm : marked irregular *2-ventricular ( from the ventricles ) : Rhythm : regular
Rate : 100 – 160 / min Ventricular beats : absent p wave , wide QRS Rate : 40 - 60 / min
Duration : normal Duration : normal
P wave Absent p wave , Replaced by : fibrillation waves ( irregular vibration ) Absent or inverted
Treatment : Supraventricular tachycardia Atrial Flutter Ventricular Tachycardia
I- During the attack : The same : but add : I- During the attack :
A- if the patient is hemodynamically stable : A- if the patient is hemodynamically stable :
1- Vagal stimulation : carotid sinus massage Digitalis, by increasing atrial automaticity , 1- Lidocaine ( first drug of choice ) : initial bolus of 2 mg / kg IV , followed by maintenance infusion of 1 – 4 mg / min
2- Slow the ventricular rate : May convert atrial flutter into AF, 2- Amiodarone or Procainamid IV ( second drug of choice )
- Adenosine 6mg IV If the drug is then stopped , 3- Bretylium IV ( in resistant cases )
- B-blocker ( propranolol ) 5 mg IV Sinus rhythm may be restored . B- A- if the patient is hemodynamically unstable :
- CCB ( verapamil & diltiazem) 5mg IV - DC cardioversion immediately followed by IV lidocaine .
- Digitalis 1 mg IV
3- Restore sinus rhythm ( cardioversion ) : after ↓ the ventricular rate
- Chemical cardioversion : class IA , IC , or class III drugs
- Electrical cardioversion ( DC ) : if chemical cardioversion fails
B- A- if the patient is hemodynamically unstable :
1- DC cardioversion
2- Overdrive pacing :
The atria are paced at a faster rate than the tachycardia rate .
Sudden cessation of pacing is usually followed by
“ restoration of sinus rhythm “
II- Prevention of a future attack : II- Prevention of a future attack :
A- Maintenance therapy : A- Maintenance therapy :
Drugs : oral , class IA , IC or class III anti-arrhythmic drugs Drugs : oral , class I or class III anti-arrhythmic drugs
B- Intervention : B- Intervention :
Ablation of focus : catheter ( radiofrequency energy ) or surgery - Ablation of focus : catheter ( radiofrequency energy ) or surgery
C- TTT of the cause - Implantable Cardivertor Defibrillator ( ICD ) “ anti – tachycardia pacing “
C- TTT of the cause
BY : Mahmoud Reda Sewilam

AF = atrial fibrillation ES = Premature beats Nodal ( Junctional ) rhythm

I- During the attack : , II- Prevention of a future attack : as SVT 1- TTT of the cause 1- TTT of the cause eg. : permanent pacemaker for sick sinus syndrome
A- if the patient is hemodynamically stable : , unstable as SVT 2- Sedatives : may be needed 2- Atropine in symptomatic cases
1- Restore sinus rhythm = rhythm control = cardioversion : 3- TTT of ES :
- Indication : A- Supraventricular beats : NB : INDICATIOS OF ANTICOAGULANT IN AF :
* recent onset AF <12 month duration - they usually need no ttt ( asymptomatic ) 1- Cardioversion ( before & after )
* absence of : embolization or history of embolization -they may need B-blockers ( symptomatic : causing palpitation ) 2- Embolization or presence of atrial thrombus ( INR : 2.5 – 3.5 )
* absence of : atrial thrombus or significant AE B- Ventricular beats : 3- Valvular AF , especially MS ( INR 2- 3 )
- contraindication : - They usually need no ttt ( asymptomatic ) 4- Non valvular AF with associated risk factors for thromboembolism :
* long standing AF > 12 month duration - The may need anti-arrhythmic drugs , if they are :  Age > 75 years
* presence of : embolization or history of embolization  Symptomatic causing palpitation  Presence of CAD , HF , HTN . ( INR : 2- 3 )
* presence of : atrial thrombus or significant AE  Multiple  Associated DM
- methods :  Multifocal
* chemical cardiversion : class IA , IC or class III drugs  Falling on the preceding T-wave ( R on T phenomenon )
* electrical cardioversion ( DC ) : if chemical cardioversion fails .  Occurring in association with AMI 2- Slow ventricular rate : rate control : Indications & drugs :
- precautions : - Anti-arrhythmic drugs are :
* anticoagulation should be given : indications : >>> * In emergency conditions : eg : AMI , Digitalis toxicity * indication : when cardioversion fails or contraindicated
i. before cardiversion ( 3- 4 weeks ) -IV lidocaine is the drug of choice * drugs : B-blockers ( propranolol ) , CCBs ( verapamil ) , Digitalis
ii. after cardioversion ( 3-4 weeks ) *in stable conditions :
* Digitalis should be stopped before electrical cardiversion ( DC ( - oral therapy with class , I , II , or III drugs .
2- Slow ventricular rate : rate control : Indications & drugs >>>