Download 5-Cardiomyopathy and Myocarditis

Dr. Talaat Ali Sabeeh
Al-Jarrah
Pediatric Cardiologist
Cardiomyopathy
Classification (2 systems):
Based on Suspected Etiology:
1-Primary. 2-Secondary
Based on echocardiography:
1-Dilated – usually mostly Systolic dysfunction
2-Restrictive – usually mostly Diastolic dysfunction
3-Hypertrophic – usually mostly Diastolic dysfunction
I-Dilated Cardiomyopathy
Dilation of all four chambers (ventricles > atria); increased muscle mass;
systolic failure
Usually presents as unexplained heart failure (prior to echo)
Signs & symptoms:
Dyspnea on exertion, fatigue, PND, dependent edema, orthopnea,
palpitations
Manifestations of embolizaiton: neurologic deficits, flank pain,
hematuria, pulseless cyanotic extremity
Rales, S3 & S4 gallops, narrow pulse pressure, mitral or tricuspid
regurgitation.
Causes:
80% are usually classified as idiopathic
Ischemic is the most common identified cause.
Toxins: Alcohol, cocaine, amphetamines, lead, lithium
Infectious: CMV, HIV, Varicella, Hepatitis
Endocrine: Thyroid/Growth hormone excess or deficiency
Peripartum
Diagnosis
 ECG: LVH, Lt. or biatrial hypertrophy, poor R wave progression,
new BBB, atrial fibrillation.
 Chest Xray: nonspecific; cardiomegaly & pulmonary vascular
congestion (globular appearance)
 Echocardiograpy:
 ventricles & atria enlargement,
 increased systolic/diastolic volumes,
 EF <45%, abnormal ventricle contraction,
 valvular regurgitation: MR,AR.
Treatment
Treat CHF: diuretics, digitalis, vasodilators, ACE inhibitors
Anticoagulants: all with mural thrombi; evidence of pulmonary or
systemic emboli; atrial fibrillation
II-Restrictive Cardiomyopathy
Characterized by diastolic restriction of ventricular filling
Low end diastolic volume
Decreased cardiac output
Causes
1-Infiltrative: Amyloidosis (most common), Sarcoidosis, Scleroderma
2-Storage diseases: Hemochromotosis
3-Endomyocardial diseases: radiation, hypereosinophlic syndrome,
carcinoid
Presentation:Similar to dilated cardiomyopathy, but more
pronounced right sided heart failure & dyspnea on exertion.
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Physical Exam:
Rales, peripheral edema, JVD, gallops, tachycardia,
regurgitation murmurs, KUSSMAUL’s Sign?
Diagnostic Studies:
ECG: chamber enlargement (atria > ventricles); low voltage,
atrial fibrillation.
Chest X ray: normal to enlarged heart with pulmonary
vascular congestion.
Echocardiogram: Thickened walls, markedly dilated atria,
normal systolic function, mitral/tricuspid regurgitation
Catheterization
Treatment
Usually diuretics and digitalis
Vasodilators can cause pronounced hypotension and may
need to be avoided.
III-Hypertrophic Cardiomyopathy
Hypertrophy of the left ventricle, and occasionally of the right ventricle
Interventricular septum is typically more prominently involved than the
left ventricular free wall
Left ventricular volume is normal or reduced; Diastolic dysfunction
usually present
50% inherited - autosomal dominant
Presentation
Dyspnea on exertion, ischemic chest pain
Palpitations, arrhythmias, syncope, pre-syncope
Sudden Death
Physical Exam
S4 gallop
Prominent systolic ejection murmur at left sternal border that increases with
valsalva maneuver, sudden standing, or exercise.
Diagnosis
Chest Xray: usually normal
Echocardiogram: LVH with disproportionate septal hypertrophy; small
ventricular volume
ECG: LVH & LAH, atrial fibrillation.
NB. think hypertrophic cardiomyopathy in any young person whose ECG shows
large septal Q waves
Treatment
Can become unstable in atrial fibrillation.
1-Long term care with Beta blockers
2-Amiodarone is treatment of choice for ventricular
dysrhhythmias
3-Diuretics in the face of pulmonary congestion
4-Avoid agents that reduce ventricular volume (nitrates) or
increase myocardial contractility (digoxin)
5-Antibiotic prophylaxis for dental procedures
6-Avoidance of competitive athletics
7-Anticoagulation if in atrial fibrillation 
Myocarditis
Definition: inflammation of the heart muscle
Causes
1- Infection: Viruses are the most frequent pathogens, but , bacteria,
fungi, protozoa, and helminths have been implicated ,most frequently
identified viruses were enteroviruses (including coxsackievirus) until
the 1990s, but now parvovirus B19 and Human Herpes Virus 6 are more
common.
Parasites: Chagas’s, Toxoplasmosis, Trichinosis
2-Autoimmune disorders: such as SLE, Wegener's granulomatosis,
giant cell arteritis, Kawasaki’s, and Takayasu arteritis
3-Drugs: Penicillin, sulfonamides
4-Toxins: Cocaine, toluene
Presentation
Think myocarditis in a young male with recent viral prodrome & now
presents with unexplained CHF, new dysarrythmias/conduction defect,
or symptoms of pericarditis with heart failure!
 Spectrum of presentation of myocarditis:
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Excessive fatigue
Chest pain
Unexplained sinus tachycardia
Acute pericarditis
S3, S4, or summation gallop
Abnormal electrocardiogram
Abnormal echocardiogram
New cardiomegaly on chest x-ray
Atrial or ventricular arrhythmia
Partial or complete heart block
New onset congestive heart failure
Atypical myocardial infarction
Cardiogenic shock
Diagnosis
Combination of clinical presentation and laboratory/imaging studies
ESR: elevated.
Cardiac Enzymes: rise over several days as opposed to hours in AMI.
Endomyocardial Biopsy: definitive diagnosis.
CBC: Mild leukocytosis Echocardiography: Dilated chambers with focal
wall motion defects.
ECG: Low Voltage, non specific ST/T wave changes, any dysrhhythmia,
sinus tachycadia most common.
Treatment
Admit to ICU
Administer Antibiotics if suspected bacterial infection
Aspirin & IVIG if suspected Kawasaki’s
Bed Rest
CHF treated , but caution with digoxin
Dysarrythmias managed per normal protocol.
PERICARDITIS
Follows a viral URI
Sharp chest pain, retrosternal, difficulty in deep inspiration
Patient resists lying down
Pain worsened by pressure over the sternum
Friction rub, pulsus paradoxus
ECG is diagnostic: diffuse ST elevation.
TREATMENT:
Reassurance
NSAIDS
Occasional pericardial tap
Post-pericardiotomy Syndrome: 2 weeks after surgery.