Cardiomyopathy

Welcome to this self-paced web based training on cardiomyopathy. Echocardiography A definitive tool for establishing presence and type of cardiomyopathy Provides information about specific etiology Tracks physiologic abnormalities associate with the cardiomyopathy A Class I diagnostic test This course is designed to give you a better understanding of dilated,  hypertophic, restrictive, and arrhythmogenic cardiomyopathies.     Cardiomyopathy is an abnormal heart condition that causes the heart to become: dilated (poor pumping power) restrictive (impaired ability of the heart to fill) hypertrophic (obstructive or non-obstructive) A fourth cardiomyopathy, arrhythmogenic RV, is a genetic condition in which the right ventricular mayocardium is replaced by fat and fibrosis. You will find more information on each type of cardiomyopathy on the next pages. The information in this course review is an overview of the course material that will assist you in passing the assessment. Characterized by a dilated LV cavity and decreased global systolic function 50% of the cases are idiopathic & classified as ischemic and non-ischemic  Regional wall motion abnormalities can be present As a consequence of dilation and systolic dysfunction, the LV takes on a more spherical geometry          Idiopathic: a disease of unknown etiology that primarily affects the myocardium Familial: a cardiomyopathy that is genetic in etiology Peripartum: a rare disorder in which a weakened heart is diagnosed within the last month of pregnancy or within 5 months after delivery, without other identifiable causes for dysfunction of the heart Ischemic: left ventricular dysfunction that is caused by diffused coronary artery disease and the effects of chronic ischemia Toxic: left ventricular dysfunction that caused by the effects of alcohol, cocaine, carbon monoxide poisoning, Adriamycin, and other chemotherapy Infectious: left ventricular dysfunction that is caused by the effects of HIV, Chaga’s disease, post-viral myocarditis Bacterial: left ventricular dysfunction that is caused by Legionnaires disease, mycobacterial, sepsis Poorly controlled hypertension Long standing renal disease Metabolic - left ventricular dysfunction that is caused by: Nutritional Obesity Scurvy (Vitamin C deficiency) Beriberi (thiamine deficiency) Pellagra (niacin deficiency) Hypervitaminosis D Kwashiorkor (protein deficiency) Endocrine Diabetes Mellitus Cushing’s Syndrome Myxedema Acromegaly Connective tissue such as rheumatoid arthritis End stage valvular disease: response from terminal valvular conditions because the heart is unable to compensate for pressure or volume overloads any longer Cocaine: increases oxygen demand by increasing heart rate and blood pressure; affects the heart's muscular architecture Cocaine abuse causes increased left ventricular mass index with a higher tendency toward increased posterior wall thickness Alcohol: ethanol creates architectural damage. Decreases cells responsible for holding all heart cells together and keeping the normal structure/shape of the heart. Heart function depends on tight connections of heart cells to work synchronously and efficiently Dyspnea Fatigue & weakness Orthopnea Paroxysmal noctural dyspnea Pulmonary Edema Chest Pain Syncope Ventricular Arrhythmias Atrial Fibrillation Hemoptysis Nocturia Swelling of the ankles Palpitations Goal: to manage signs and symptoms, prevent the condition from worsening, and reduce the risk of complications Surgically implanted devices Biventricular Pacemaker: coordinates the contraction between the left and right ventricle ICD: protects patients who are at serious risk from arrhythmias or drug therapy Balloon heart pump (aortic counter-pulsation balloon) Left ventricular assist device (LVAD) Heart transplantation Angiotensin:  converting enzyme (ACE) inhibitors to improve the heart's pumping capability, such as enalapril (Vasotec), lisinopril (Zestril, Prinivil), ramipril (Altace) or captopril (Capoten) Angiotensin receptor blockers (ARBs): for those who can't take ACE inhibitors, such as losartan (Cozaar) and valsartan (Diovan) Beta blockers: improve heart function, such as carvedilol (Coreg) and metoprolol (Lopressor, Toprol XL) Diuretics: remove excess fluid Digitalis: strengthen the heart’s pumping ability 2D Left ventricular dilation Increasing sphericity of the LV geometry Apical and lateral displacement of papillary muscles Left ventricular thrombus (laminar, pedunculated, or mobile) Reduced Ejection Fraction Left atrial dilation Left atrial thrombus/stasis of blood (“smoke”) Increased left atrial volume Right ventricular dilation       Double diamond appearance of mitral valve Increased End Point Septal Separation (EPSS) Defined as the distance from the anterior septal endocardium to the maximal early opening point (E-point) of the mitral valve B-bump mitral valve closure Associated with elevated left atrial pressure, typically exceeding 20mmHG Rounding of the Aortic Valve closure Normally the aortic valve depicts a crisp box on M-Mode. Due to the reduced forward flow in systole, there is rounding of the box at end systole.      EPSS is increased significantly Mitral Valve Tricuspid Valve Due to the apical and lateral displacement of the papillary muscles, the mitral valve coaptation length is shortened, resulting in mitral regurgitation                       Due to the dilatation of the right ventricle, the tricuspid valve annulus dilates resulting in tricuspid regurgitation Pulmonary Hypertension Mitral valve regurgitation   Tricuspid valve regurgitation Cardiomyopathy Represents a diverse group of diseases intrinsic to the myocardium This abnormal heart condition causes the heart to become: dilated (poor pumping power) restrictive (impaired ability of the heart to fill) hypertrophic (obstructive or non-obstructive) Many occur for no known reason and are called idiopathic Secondary cardiomyopathy: a problem linked to another form of heart disease, an illness, substance abuse, or other conditions Impairment of diastolic function from a stiff ventricle with abnormal thickness of the ventricular muscle; may present with or without obstruction Obstructive variant of hypertrophic obstructive cardiomyopathy (HOCM) is also known as idiopathic hypertrophic subaortic stenosis (IHSS) Stiffness in the LV occurs when heart muscle thickens, causing cellular changes Thickened septum may cause a narrowing that can block or reduce blood flow from LV to aorta LVOT narrowing disrupts proper function of MV. Obstruction causes MV to strike septum     Primary Idiopathic: unknown cause Autosomal dominal trait: a single, abnormal gene is passed on from one of the first 22 “non sex” chromosomes from either parent Secondary Hypertension: Usually concentric because all areas of the myocardium react to the increased afterload as a response to increased systemic blood pressure Aortic Stenosis: increase in wall thickness due to the increased ventricular pressure as blood flow is obstructed as it crosses the left ventricular outflow tract     Using tissue doppler can help determine the magnitude of the motion of the mitral valve annulus movement towards the apex from ventricular contraction There is a direct relationship between annular velocity and the left ventricular ejection fraction. The lower the ejection fraction, the lower the systolic velocity (S-wave)        Orthopnea Dyspnea Paroxysmal nocturnal dyspnea Syncope Chest Pain Fatigue Pulmonary Edema Palpitations Asymptomatic (non-obstructive) Surgical procedures Septal myotomy-myectomy: remove a portion of the thickened muscle wall that blocks normal blood flow Non-surgical procedure Alcohol ablation: uses injected alcohol to destroy extra heart muscle, may reduce muscle thickening and improve blood flow Surgically Implanted Devices ICD: protects patients that are at serious risk from arrhythmias or drug therapy Pacemaker Calcium channels blockers: to decrease the force of contraction of the myocardium Verapamil (Calin, Isoptin) Beta blockers: to relax the heart, slow its pumping action, and stabilize the rhythm Lopressor 2D Near cavity obliteration Asymmetrical Septal Hypertrophy (ASH): most common morphological pattern concentric, apical, or free wall LV hypertrophy can be present Symptom of dynamic obstruction: basal septum is hypertrophied and bulges in LVOT Most frequent morphologic varieties of hypertrophic cardiomyopathy diffuse hypertrophy of the ventricular septum and anterolateral free wall (70-75%) basal septal hypertrophy (10-15%) concentric hypertrophy (5%) hypertrophy of the lateral wall (1-2%) apical hypertrophy (<0.5%)   Near cavity obliteration Note SAM Dynamic LVOT obstruction due to the bulging of the septum into the LVOT The MV apparatus comes in contact with the septum Obstruction is likely to be present if SAM persists for 40% of systolic cycle and makes direct contact with IVS The velocity of blood flow across the narrowed LVOT increases and produces a Venturi efffect Mitral leaflets and support apparatus are drawn toward septum            If obstruction worsens with provocation (Valsalva maneuver or a pharmalogical intervention [i.e. amyl nitrate inhalation]), the SAM will also worsen. SAM is graded as follows: 0 = absent 1 = present, with a minimum distance between the mitral valve and ventricular septum during systole  <10 mm 2 = without mitral-septal contact, but with a distance of >10 mm between the mitral valve and septum 3 = brief mitral-septal contact (<40 percent of echocardiographic systole) 4 = prolonged apposition of the mitral valve leaflet with the septum (>40 percent of echocardiographic systole) Asymmetrical Septal Hypertrophy Occurs when the septal to posterior wall thickness ratio is at least 1.3:1 Systolic Anterior Motion (SAM) of the Mitral Valve Occurs because of the abnormal geometric relationship of papillary muscle and the mitral supporting apparatus combined with hyperdynamic left ventricular function so there is an anterior displacement of varying portions of the mitral valve in systole                     Mid-systolic closure of the aortic valve Obstruction can occur mid-to-late systole concurrent with late-phase left ventricular contraction Ejection diminishes briefly Reduction in flow volume causes partial closure of the aortic valve Often, secondary opening appears as final ejection occurs LV end-diastolic cavity dimension less than 45 mm Myocardial relaxation is impaired due to the hypertrophic myocardium Image:​ Initial opening of the aortic valve: normal Shows a prominent midsystolic closure or notch Coincides with onset of subaortic obstruction induced by the systolic contact of the MV with the septum (SAM) Aortic valve often reopens for the second half of systole                     Has a late peak dagger-shaped appearance The late peaking of the outflow tract gradient is evidence of the dynamic nature of the gradient which develops toward mid-late systole rather than being a fixed obstruction which the gradient occurs early in systole This is not truly obstructive with respect to volume of flow because the majority of the left ventricular stroke volume has been ejected ​Small stroke volume due to small diastolic volumes Increased LVOT flow with a late peak dagger-shaped appearance Patients with at least 3/6 murmurs could have subaortic gradient exceeding 30 mmHg The severity of LVOT obstruction can documented with continuous wave Doppler during the Valsalva maneuver or amyl nitrate inhalation Mitral regurgitation might be present in obstructive hypertrophic cardiomyopathy                  Early rapid filling (E-wave) is reduced because the myocardial relaxation is impaired Deceleration Time (DT) is prolonged because it takes the atrio-ventricular pressure longer to equilibrate Atrial filling (A-wave) is increased because at the time of atrial systole, the atrium is relatively full so there is a compensatory increase in flow during atrial contraction Infiltration of the myocardium resulting in rigid ventricular walls which may impede diastolic filling Systolic function of the ventricle might be normal in early stages, while systolic dysfunction can present in later stages.  Rarest form of cardiomyopathy and most difficult to treat There is no cure, only treatment Medical treatment is for relief of systemic and pulmonary venous congestion Can be confused with constrictive pericarditis Condition in which the layers of the pericardium becomes thickened, calcified, and stiff              Primary Idiopathic: unknown cause of the stiffened ventricle Secondary                                                                                                                                               Endocardial Fibroelastosis: a rare disorder that causes a thickened layer of fibrous tissue within the left ventricle Endomyocardial Fibrosis: cardiac dysfunction occurs because of fibrous lesions that affect the inflow of the right and/or left ventricles and that may also involve the atrioventricular valves,  thereby producing regurgitant lesions Loffler Endocarditis: hypereosinophilic syndrome Amyloidosis:  occurs due to a build-up of amyloid proteins (abnormal proteins usually produced by bone marrow cells that can be deposited in any tissue) in organs. Hemachromatosis: this inherited disorder, the most common form of iron overload disease, causes the body to absorb and store too much iron. Excess iron stored throughout the body in organs and tissues, including the pancreas, heart, liver, and skin, can damage these organs if left untreated.  ​Sarcoidosis: an inflammatory disease that can affect almost any organ in the body. It causes heightened immunity resulting in damage to the body’s own tissues. Sarcoidosis can cause the heart to pump weakly.  The classic feature of sarcoidosis is the formation of granulomas. Pompe disease: is caused by increased storage of glycogen and fat in tissues of the body. The build-up of glycogen causes progressive muscle weakness throughout the body and affects various body tissues, particularly in the heart. Radiation: the heart muscle is gradually replaced by scar tissue. Scarring results from injury due to radiation therapy for cancer Cardiac neoplasms: primary or secondary cardiac tumors. Cardiac neoplasms may involve only the endocardium, only the myocardium, only the epicardium, or any combination of these. By far the most common location of metastatic cardiac neoplasm is the epicardium.  Intramyocardial masses are commonly found on the left ventricular free wall and the ventricular septum. Examples of cardiac neoplasms include myxomas, rhabdomyomas, and rhabomyosarcomas.  Carcinoid: metastatic tumors originating in the GI tract.  They are associated with deposition of fibrous tissue on the endocardial surfaces Chest pain or pressure Dyspnea (on exertion, at night, or supine) Weakness Fatigue Edema Palpitations Distended neck veins Congestive heart disease Diuretics: remove fluid and help improve breathing Corticosteroids: suppress the immune system to prevent further heart injury.  Corticosteroids reduces the inflammation caused by sarcoidosis Heart Transplantation: consider when the heart function is poor Autologous stem cell transplant current Tx Amyloidosis Balloon valvuloplasty of stenotic tricuspid and pulmonic valves Carcinoid Syndrome Iron chelation with desferrioxamine Hemochromatosis  2D Speckled or grain-glass appearance of the myocardium, endocardium, and subendocardium due to the infiltration of amyloid into the intracellular spaces. Normal or slightly thickened mitral valve Atria size may be increased due to elevated diastolic pressure   Granular or sparkling appearance of the myocardium This finding alone is relatively non-specific but the combination of reflective echoes and atrial septum thickening is highly specific for cardiac amyloid Increased symmetrical LV and/or RV wall thickness Small ventricular chambers Mitral regurgitation Tricuspid regurgitation Aortic regurgitation Restrictive filling pattern A result of a reduction in ventricular compliance and increase in ventricular filling pressures Increase in left atrial pressure causes the mitral valve to open early Tall E-wave (due to the increase in transmitral gradient in early diastole) Short to absent A-wave due to little to no filling during atrial contraction because of high left ventricular end-diastolic pressure (LVEDP) Shortened isovolumic relaxation time (IVRT) due to the early opening of the mitral valve (<70msec) Shortened deceleration time (<160 msecs) due to the early diastolic flow into the non-compliant ventricle results in a rapid increase in early ventricular diastolic pressure with rapid equalization of ventricular and atrial pressures                                                e-Dominant Restrictive Inflow Pattern The transmitral continuous wave Doppler in severe mitral regurgitation shows an E wave dominant, restrictive inflow pattern. There is a high-velocity E wave, which in this example is 1.7m/sec. The A wave is small and the E/A ratio is, therefore, increased.                                                                                    Decrease A duration Pulmonary and hepatic vein flow Systolic velocity much smaller diastolic velocity Increased diastolic flow reversal in the hepatic vein during inspiration Increased atrial flow reversal velocity and duration in pulmonary vein Arrhythmogenic Right Ventricular Dysplasia (ARVD) A rare genetic progressive heart condition where the myocardium is replaced by fat and fibrosis ARVC usually affects the right side but can affect both sides Also called Arrhythmogenic RV Dysplasia Is characterized by four phases A concealed phase in which the patient is asymptomatic A phase that is characterized by overt clinical signs of an electrical system disturbance Progression to signs and symptoms of right ventricular failure Biventricular congestive heart failure     Two pathological patterns seen in Arrhythmic RV Cardiomyopathy: Fatty infiltration Confined to the right ventricle Involves a partial or near-complete substitution of myocardium with fatty tissue without wall thinning Fibro-fatty infiltration Involves replacement of myocytes (myocardial cells) with fibro-fatty tissue Leads to thinning of the RV free wall (to < 3 mm thickness)   Familial: a cardiomyopathy that is genetic in etiology Syncope Palpitations Ventricular Tachycardia Lower extremity edema Sudden death Beta Blocker Sotalol Metoprolol Antiarrhythmic Agents Amiodarone  Anticoagulant (patients with a decreased EF) i.e. Warfarin Catheter Ablation To treat ventricular tachycardia Implantable Medical Devices Implantable Cardioverter-Defibrillator (ICD) implications Preventing sudden death Symptomatic ventricular tachycardia (VT) Failed drug therapy Severe RV involvement with poor tolerance of VT Surgical Heart Transplantation When an ICD controls the arrhythmias, more individuals with heart failure live longer therefore possibly requiring a transplant in their future 2D Hypokinetic right ventricle with a paper-thin RV free wall RV dilatation (can be segmental) Paradoxical septal motion may also be present Possible localized RV aneurysms Decreased RV ejection fraction Little to no left ventricular impairment Thin RV free wall Decreased RV function Dilated RV Possible paradoxical septal motion           Tricuspid Regurgitation Caused by dilatation of the tricuspid valve annulus RVSP Is typically in the low to mid-range due to right ventricular failure Cardiomyopathy can be described as an abnormal heart condition in which the heart is dilated (poor pumping power), restrictive (impaired ability of the heart to fill), and hypertrophic (obstructive or non-obstructive) We discussed the four cardiomyopathies: Dilated Cardiomyopathy Hypertrophic Cardiomyopathy Restrictive Cardiomyopathy Arrhythmogenic Cardiomyopathy Many occur for no known reason and are called idiopathic  Some are secondary, which refers to a problem that is linked to another form of heart disease, illness, substance abuse, and other conditions Characterized by a dilated LV cavity and decreased global systolic function. Etiologies  Primary:  idiopathic (familial, perpartum) Secondary: ischemic, toxic, infectious, bacterial, hypertension, renal disease, metabolic, endocrine, connective tissue, end stage valvular disease, substance abuse Signs & Symptoms: Dyspnea, fatigue & weakness, orthopnea, paroxysmal noctural dyspnea, pulmonary edema, chest pain, syncope, ventricular arrhythmias, atrial fibrillation, hemoptysis, nocturia, swelling of the ankles, palpitations Treatment: surgically implanted devices, balloon heart pump, LVAD, heart transplantation, medications Medication: angiotensin, ARBs, beta blockers, diuretics, digitalis 2D findings: left ventricular dilation increasing sphericity of LV geometry, left atrial “smoke,” increased left atrial volume M-mode: mitral valve double diamond appearance, EPSS, B-bump mitral valve closure, rounding of aortic valve closure Doppler: mitral valve, tricuspid valve, tissue Doppler, impaired, restrictive, or pseudonormal filling pattern An impairment of diastolic function from a stiff ventricle with abnormal thickness of the ventricular muscle which may present with or without obstruction Etiologies Primary:  idiopathic, autosomal Secondary: hypertension, aortic stenosis Signs & Symptoms: orthopnea,  dyspnea,  paroxysmal nocturnal dyspnea,  syncope,  chest pain,  fatigue,  pulmonary edema,  palpitations,  asymptomatic (non-obstructive) Medication: calcium channel blockers, beta blockers 2D findings: near cavity obliteration, ASH, SAM of mitral valve leaflet, enlarged mitral valve, anomalous insertion of anterolateral papillary muscle into anterior mitral leaflet M-mode: assymetrical septal hypertrophy, SAM, mid-systolic closure of the aortic valve, LV end-diastolic cavity dimension < 45mm Doppler: small stroke volume, increased LVOT, mitral regurgitation, prolonged IVRT, e-wave, DT, A-wave An infiltration of the myocardium resulting in rigid ventricular walls which may impede diastolic filling Etiologies Primary:  idiopathic Secondary: Endocardial Fibroelastosis, Endomyocardial Fibrosis, Loffler Endocarditis, Amyloidosis, Hemachromatosis, Pompe’s disease, radiation, Cardiac neoplasms, Carcinoid   Signs & Symptoms: chest pain or pressure, dyspnea (on exertion, at night, or supine), weakness,  fatigue, edema, palpitations, distended neck veins, congestive heart disease    Medication: diuretics, corticosteroids, heart transplantation, Autologous stem cell transplant, Balloon valvuloplasty, Iron chelation with desferrioxamine 2D findings: speckled or grain-glass appearance of the myocardium, endocardium, and subendocardium, normal or slightly thickened mitral valve, increased atria size M-mode: granular or sparkling appearance of the myocardium, increased symmetrical LV and/or RV wall thickness, small ventricular chambers Doppler: mitral regurgitation, tricuspid regurgitation, aortic regurgitation, restrictive filling pattern, decrease A duration, pulmonary & hepatic vein flow A genetic, progressive heart condition in which the right ventricular myocardium is replaced by fat and fibrosis. Primary Etiology: familial Signs & Symptoms: syncope, palpitations, ventricular tachycardia, lower extremity edema, sudden death Medication: beta blockers, arrhythmic agents, anticoagulants, catheter ablation Devices & Procedure: implantable medical devices (implantable cardioverter-defibrillator); heart transplantation 2D findings: Hypokinetic right ventricle with a paper-thin RV free wall ,  RV dilatation (can be segmental),  paradoxical septal motion, possible localized RV aneurysms,  decreased RV ejection fraction M-mode: Thin RV free wall, decreased RV function, dilated RV, possible paradoxical septal motion Doppler:  tricuspid regurgitation, RVSP By the end of this course, you will be able to: Define cardiomyopathy Describe the primary and secondary etiologies of each type Identify the type of cardiomyopathy presented by the patient via echocardiographic findings and symptoms that are presented or listed in the patient's medical record List the complications from dilated, restrictive, hypertrophic, and arrythmogenic cardiomyopathies A. Normal B. Impaired C. Pseudo-     normal D. Restrictive Filling pattern depends on the severity of left ventricular function Normal Impaired: Decreased E-wave, prolonged DT, & Increased A-wave Pseudonormal: Normal E/A with low flow propagation velocity (<50 cm/sec) Restrictive: Increased E-wave, shortened DT, & Decreased A-wave​