Cardiomyopathy Myocarditis Petr Lokaj, MD.,Ph.D. [USEMAP] [USEMAP] DEFINITION na myocardial disorder in which the heart muscle is structurally and functionally abnormal n nin the absence of coronary artery disease, hypertension, valvular disease and congenital heart disease n n [USEMAP] CLASSIFICATION n n Dilated CMP – the most common form n Hypertrofic CMP – innapropriate LV hypertrophy n Restrictive CMP – impaired diastolic filling n Arrhytmogenic right ventricular cardiomyopathy n Unclasiffied: Tako Tsubo CMP [USEMAP] 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 [USEMAP] Aetiology of DCMP nFamilial (idiopathic) n nNon familial n * myocarditis – late stage following cardiac infection n * pregnancy – peripartum cardiomyopathy n * cardiotoxic drugs n * alcohol n * tachycardia induced CMP (Atrial fibrilation) n * ischemic CMP n nInflammatory CMP n chronic inflammatory cells in myocardium + n left ventricular dilatation and reduced ejection fraction (< 35 %) [USEMAP] Diagnosis of DCMP n n n nSymptoms + anamnesis n n* left ventricle failure: shortness of breath , hemoptysis, cough n n* right ventricle failure: abdominal swelling or enlargement, n swelling of legs or ankles, absence of appetite, abdominal pain n n* low cardiac output: temporary and brief loss of consciousness, n decreased ability to tolerate physical exertion, palpitations, n dizziness, fatique , low amount of urine during the daytime, n but a need to urinate at night n [USEMAP] Diagnosis of DCMP nPhysical signs: n * left ventricle failure: basilar rales, pulmonary edema, gallop, n pleural effusion, Cheyne – Stokes respiration n * right ventricle failure: peripheral oedema, jugular venous n distention, hepatomegaly, ascites n Diagnostic methods: n * laborathory (BNP) n * ECG n * chest X -ray n * Echocardiography n * MRI n * cardiac catheterization n * heart muscle biopsy [USEMAP] Chest X ray [USEMAP] It is important to understand that the symptoms of systolic and diastolic heart failure are the same. Whether a patient has systolic or diastolic heart failure depends on the ejection fraction. If the EF is less than 40%, it is labeled systolic heart failure. If it is greater than 40%, it is labeled diastolic heart failure. Remember that almost all systolic heart failure has a component of diastolic failure. Patient symptoms must correlate to the physical signs in order for them to be diagnostic of heart failure. The symptoms of left ventricular dysfunction and the physical signs are all resultant of increased left arterial pressure, capillary pressure, and pulmonary congestion. Echo in DCMP [USEMAP] Therapy of DCMP nComplex: nregime nPharmacotherapy (ACEI, BB, diuretic) ncardiostimulation or resynchronization therapy nLVAD (left ventricle assist devices) northotopic heart transplantation n n= therapy of chronic heart failure n [USEMAP] Prognosis of DCMP nPrognosis of patients with CHF is poor. n n50% of patients with CHF die in five years!!! n nYoung patients up to 65 years have i big chance in OTS (orthotopic heart transplantation) [USEMAP] Hypertrophic cardiomyopathy n nthe majority of patients have asymmetrical pattern of hypertrophy, with a predilection for the interventricular septum n nanteroseptal napical n n h9991656_001 [USEMAP] Hypertrophic cardiomyopathy n nconcentric hypertrophy is more frequent in patients with metabolis disorders and hypertension. n [USEMAP] ECG in HCMP [USEMAP] Echo in HCMP [USEMAP] Hypertrophic CMP nTreatment – in symptomatic patients nBetablockers, calcium antagonists antiarrhythmic nDDD stimulations, ICD therapy in high risk patients n - posit. FH n - septum > 30mm n - occur. of VT, syncopy, CPR n - decrease of BP during stress test nAlcoholic septal ablations nSurgical treatment- myectomy [USEMAP] Restrictive Cardiomyopathy nis defined functionally - not morphologically nnormal or reduced diastolic volumes nnormal or reduce systolic volumes nnormal ventricular wall thickness n [USEMAP] Restrictive Cardiomyopathy n nEASY definition: rigid heart walls with diastolic (and systolic) dysfunction due to increase stiffness nhigh pressure rise to a smal increase in volume n nCauses: n * familial: sarcomeric protein mutation, familial amyloidosis, n hemochromatosis, .. n * nonfamilial : amyloidosis n sarcoidosis, scleroderma n carcinoid heart disease, metastatic cancers n drugs (anthracycline toxicity ), radiation n [USEMAP] Restrictive Cardiomyopathy [USEMAP] Restrictive Cardiomyopathy n nSymptoms: exercise intolerance, dyspnea, weakness, n chest pain, peripheral edema, enlarged liver, n ascites, anasarca (symptoms of CHF) nDiagnostic: ECG – low voltage on ECG n ECHO – restrictive filing of LV n MRI n myocardial biopsy nTherapy: only symptomatic n [USEMAP] Tako Tsubo CMP n n n nDefinition: transient regional systolic dysfunction involving the left ventricular apex and or mid ventricle n nin the absence of obstructive coronary disease on coronary angiography n n n n [USEMAP] Tako Tsubo CMP nIn this syndrome, the heart (left ventricle) takes the n shape of an octopus trap n n n 1. • ANd9GcRbkBMnLjFHAyA5txq2jYzpQpsKSHoDc4XIcmU_j7RE7Dwz0O6l apical form mid-ventricular form [USEMAP] Tako Tsubo CMP n nSymptoms: patients present with an abrupt onset of n angina like chest pain - like acute myocardial infarction n ndiffuse T waves inversion, sometimes proceded by ST segment elevation, QT-interval prolongation nmild cardiac enzyme elevation n nDiagnosis: coronarography - negative nIncidence: post - menopausal women (the most often) nsymptoms are often proceded by emotional or physical stress nleft ventricular function usually normalizes over a period of days to weeks n n [USEMAP] Perimyocarditis nMyocarditis - inflammatory disease of the myocardium nInflammatory cardiomyopathy - myocarditis in association with cardiac dysfunction (histological , functional diagnosis) nDilated cardiomyopathy – is a clinical diagnosis characterized by dilation and impaired contraction of the left or both ventricles n [USEMAP] Perimyocarditis nDefinition: inflammatory disease of the myocardium n nCauses: * infectious myocarditis n - viral n - bacterial n - protozoal n - fungal n - parasitic n * immune mediated myocarditis n * toxic myocarditis –drugs, heavy metals, hormones, n radiation, electric shock n [USEMAP] Perimyocarditis npresents in many different ways ranging from mild to life threatening n nclinical presentation: nacute chest pain (starting within 1-4 weeks of a respiratory or gastrointestinal infection) , mimic MI nnew onset or worsening HF (in the absence of CAD) nchronic heart failure nlife threatening arrhytmias and sudden cardiac death ncardiogenic shock n n [USEMAP] Fulminant Perimyocarditis [USEMAP] Perimyocarditis nDiagnosis: nSymptoms + anamnesis nLaborathory: nonspecific; Trop T, BNP, CK, Leu, CRP nECG: nonspecific- inversion of T wave, ST changes, VT, AVB, ... nECHO: physiological, low EF, segmental hypokinesis, n global hypokinesis nMRI: inflammatory changes n nEndomyocardial biopsy: gold standard, invasive L n n [USEMAP] Perimyocarditis [USEMAP] Perimyocarditis nTreatment nConventional medical treatment of heart failure n- rest (3 months) -pharmacotherapy (ACEI, BB) -ICD + CRT -LVADs, OTS nImmunosuppressive therapy (eosinophile, lymes boreliosis, great-cell myocarditis) [USEMAP] Perimyocarditis nPrognosis (acute myocarditis) ndepends on aetiology , clinically presentation and disease stage n50% resolves in the first 2-4 weeks n25% will develop persistent cardiac dysfunction n12-25% deteriorate, die or progress to end stage DCMP with a need for OTS [USEMAP] [USEMAP]