Cardiomyopathy
Myocarditis
Petr Lokaj, MD.,Ph.D.
DEFINITION
 a myocardial disorder in which the heart muscle
is structurally and functionally abnormal
 in the absence of coronary artery disease,
hypertension, valvular disease and congenital
heart disease
CLASSIFICATION
 Dilated CMP – the most common form
 Hypertrofic CMP – innapropriate LV hypertrophy
 Restrictive CMP – impaired diastolic filling
 Arrhytmogenic right ventricular cardiomyopathy
 Unclasiffied: Tako Tsubo CMP
DCMP
defined by the presence of left ventricular dilatation and left ventricular systolic dysfunction
right ventricular dilatation and dysfunction may be present but are not necessary for the diagnosis
Aetiology of DCMP
 Familial (idiopathic)
 Non familial
* myocarditis – late stage following cardiac infection
* pregnancy – peripartum cardiomyopathy
* cardiotoxic drugs
* alcohol
* tachycardia induced CMP (Atrial fibrilation)
* ischemic CMP
 Inflammatory CMP
chronic inflammatory cells in myocardium +
left ventricular dilatation and reduced ejection fraction (< 35 %)
Diagnosis of DCMP
 Symptoms + anamnesis
* left ventricle failure: shortness of breath , hemoptysis, cough
* right ventricle failure: abdominal swelling or enlargement,
swelling of legs or ankles, absence of appetite, abdominal pain
* low cardiac output: temporary and brief loss of consciousness,
decreased ability to tolerate physical exertion, palpitations,
dizziness, fatique , low amount of urine during the daytime,
but a need to urinate at night
Diagnosis of DCMP
 Physical signs:
* left ventricle failure: basilar rales, pulmonary edema, gallop,
pleural effusion, Cheyne – Stokes respiration
* right ventricle failure: peripheral oedema, jugular venous
distention, hepatomegaly, ascites
 Diagnostic methods:
* laborathory (BNP)
* ECG
* chest X -ray
* Echocardiography
* MRI
* cardiac catheterization
* heart muscle biopsy
Chest X ray
Echo in DCMP
Therapy of DCMP
Complex:
 regime
 Pharmacotherapy (ACEI, BB, diuretic)
 cardiostimulation or resynchronization therapy
 LVAD (left ventricle assist devices)
 orthotopic heart transplantation
 = therapy of chronic heart failure
Prognosis of DCMP
 Prognosis of patients with CHF is poor.
 50% of patients with CHF die in five years!!!
 Young patients up to 65 years have i big chance
in OTS (orthotopic heart transplantation)
Hypertrophic cardiomyopathy
 the majority of patients have asymmetrical pattern of
hypertrophy, with a predilection for the interventricular
septum
 anteroseptal
 apical
Hypertrophic cardiomyopathy
 concentric hypertrophy is more frequent in patients
with metabolis disorders and hypertension.
ECG in HCMP
Echo in HCMP
Hypertrophic CMP
Treatment – in symptomatic patients
 Betablockers, calcium antagonists antiarrhythmic
 DDD stimulations, ICD therapy in high risk patients
- posit. FH
- septum > 30mm
- occur. of VT, syncopy, CPR
- decrease of BP during stress test
 Alcoholic septal ablations
 Surgical treatment- myectomy
Restrictive Cardiomyopathy
 is defined functionally - not morphologically
 normal or reduced diastolic volumes
 normal or reduce systolic volumes
 normal ventricular wall thickness
Restrictive Cardiomyopathy
 EASY definition: rigid heart walls with diastolic (and systolic)
dysfunction due to increase stiffness
 high pressure rise to a smal increase in volume
 Causes:
* familial: sarcomeric protein mutation, familial amyloidosis,
hemochromatosis, ..
* nonfamilial : amyloidosis
sarcoidosis, scleroderma
carcinoid heart disease, metastatic cancers
drugs (anthracycline toxicity ), radiation
Restrictive Cardiomyopathy
Restrictive Cardiomyopathy
 Symptoms: exercise intolerance, dyspnea, weakness,
chest pain, peripheral edema, enlarged liver,
ascites, anasarca (symptoms of CHF)
 Diagnostic: ECG – low voltage on ECG
ECHO – restrictive filing of LV
MRI
myocardial biopsy
 Therapy: only symptomatic
Tako Tsubo CMP
 Definition: transient regional systolic
dysfunction involving the left ventricular apex
and or mid ventricle
 in the absence of obstructive coronary
disease on coronary angiography
Tako Tsubo CMP
 In this syndrome, the heart (left ventricle) takes the
shape of an octopus trap
apical formmid-ventricular form
Tako Tsubo CMP
 Symptoms: patients present with an abrupt onset of
angina like chest pain - like acute myocardial infarction
 diffuse T waves inversion, sometimes proceded by ST
segment elevation, QT-interval prolongation
 mild cardiac enzyme elevation
 Diagnosis: coronarography - negative
 Incidence: post - menopausal women (the most often)
 symptoms are often proceded by emotional or physical
stress
 left ventricular function usually normalizes over a period of
days to weeks
Perimyocarditis
 Myocarditis - inflammatory disease of the
myocardium
 Inflammatory cardiomyopathy - myocarditis in
association with cardiac dysfunction (histological ,
functional diagnosis)
 Dilated cardiomyopathy – is a clinical diagnosis
characterized by dilation and impaired contraction of
the left or both ventricles
Perimyocarditis
 Definition: inflammatory disease of the myocardium
 Causes: * infectious myocarditis
- viral
- bacterial
- protozoal
- fungal
- parasitic
* immune mediated myocarditis
* toxic myocarditis –drugs, heavy metals, hormones,
radiation, electric shock
Perimyocarditis
presents in many different ways ranging from mild to life
threatening
 clinical presentation:
 acute chest pain (starting within 1-4 weeks of a
respiratory or gastrointestinal infection) , mimic MI
 new onset or worsening HF (in the absence of CAD)
 chronic heart failure
 life threatening arrhytmias and sudden cardiac death
 cardiogenic shock
Fulminant Perimyocarditis
Perimyocarditis
 Diagnosis:
 Symptoms + anamnesis
 Laborathory: nonspecific; Trop T, BNP, CK, Leu,
CRP
 ECG: nonspecific- inversion of T wave, ST changes,
VT, AVB, ...
 ECHO: physiological, low EF, segmental hypokinesis,
global hypokinesis
 MRI: inflammatory changes
 Endomyocardial biopsy: gold standard, invasive 
Perimyocarditis
Perimyocarditis
Treatment
 Conventional medical treatment of heart failure
- rest (3 months)
- pharmacotherapy (ACEI, BB)
- ICD + CRT
- LVADs, OTS
 Immunosuppressive therapy (eosinophile, lymes
boreliosis, great-cell myocarditis)
Perimyocarditis
Prognosis (acute myocarditis)
 depends on aetiology , clinically presentation
and disease stage
 50% resolves in the first 2-4 weeks
 25% will develop persistent cardiac dysfunction
 12-25% deteriorate, die or progress to end stage
DCMP with a need for OTS