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Myocarditis and Cardiomyopathies

Sue-Ann R. Locnen, M.D.

November 19, 2010

LEGEND Normal text : lecture and audio recording Italics : Harrisons Principles of Internal Medicine 17th Edition MYOCARDITIS Inflammation of myocardium resulting in injury to cardiac myocytes(necrosis and/or degeneration ) not typical of IHD Inflammation can be caused by: Drugs Pathogen: Bacteria, Fungi, Viruses Immune reaction Deposition of certain substances Other cause of inflammatory reaction and cell damage in myocarditis and not in ischemic disease: Injury Infections Cardiac allograft rejection Collagen vascular diseases Drug hypersensitivity Sarcoidosis **These cause inflammatory reaction & cell damage in myocarditis NOT in ischemic disease. Infectious Etiologic Agents I. Viruses Most common cause in USA Can effect young age group Coxsackieviruses A & B and other enteroviruses (most common agents) Cytomegalovirus HIV (HIV myocarditis) Others II. Bacteria The injury is by their toxins Lyme disease Due to Spirochete infection. It causes arthritis, fever & skin rash. A prominent cause of myocarditis abroad Diphtheria Injury from toxins of Corynebacterium diphtheria Associated with weakness, hypovolemia & heart failure. When examining the heart there will be NO Bacteria. III. Parasites Chagas disease (Trypanosoma cruzi) Epidemiologically is found in South America (Brazil). Toxoplasmosis Trichinosis Trichinella spiralis *** Research companies from America are tapping patients from the Philippines and other Asian countries as part of clinical trials since previous landmark trials did not have Asian subjects. *** We do not even study Dengue to be a cause of myocarditis when in fact there are so many dengue patients in the country, it is however not included in the list of causes of myocarditis in foreign books. We are the one who should be providing data but how could we do that when a patient couldnt even afford 2d Echo so as to be diagnosed of myocarditis. Morphology Gross Cardiac dilation Myocardium flabby, pale, with focal hemorrhages The myocardium will be flabby (soft), weak & the patient will have severe heart failure.

Figure 1 Gross appearance of the heart with myocarditis. The heart is congested, edematous, flabby, soft, dilated & weak in pumping. Microscopic Acute viral Edema Inflammatory infiltrate of lymphocytes and other mononuclear cells Myocyte degeneration and/or necrosis Viral inclusions may be present Parasitic Organisms identified Bacterial Neutrophilic inflammatory infiltrate

Toxoplasma Giant Cell Myocarditis A granulomatous reaction Fiedlers myocarditis MN giant cells Lymphocytes, eosinophils, necrosis Differential: Sarcoidosis, hypersensitivity, Tuberculosis Aggressive clinical course: indication for transplant Characterized by rapidly progressive CHF and ventricular tachyarrhythmia and occurs most commonly in the third or fourth decades Distinctive features include cardiac enlargement, ventricular thrombi, grossly visible serpiginous areas of necrosis in both ventricles, and microscopic evidence of giant cells within an extensive inflammatory infiltrate Treatment with immunosuppressive therapy may help some patients, cardiac transplantation is often necessary Hypersensitivity Myocarditis Interstitial infiltrate of macrophages, eosinophils No or little necrosis No granulomas, but multinucleated cells may be present Fewer clinical manifestations than other forms of myocarditis Associated with long list of drugs (classically, methyldopa) Cardiac Sarcoidosis A multi-systemic granulomatous disease that produces NonCaseating / Non-Necrotising granuloma in lymph nodes, liver, spleen, lungs & heart. Cardiac involvement in 20-30% of patients with clinical sarcoid at autopsy

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5% of sarcoid patients will have symptomatic heart disease (cardiomyopathy, arrhythmias) Sites of predilection: Interventricular Septum, LV free wall, but all sites, including pericardium, may be involve When arrhythmia develops, the conduction system of myocardium is involved (Purkinje fibers & nodes). Rheumatic Carditis Disease of the endocardium (valves: chordae tendineae & cusps) Uncommon cause of myocarditis Aschoff nodule Anitschkow cell Macrophages Collagenolysis Interstitial fibrosis Pancarditis Clinical Presentation May be asymptomatic, inferred only from ST-T changes on the ECG May also present as a fulminant condition characterized by heart failure and arrhythmias and death. Acute fulminant myocarditis has 2 outcome, death or complete recovery. Chronic fulminant myocarditis may simulate AMI and have elevated cardiac enzymes PE is often normal but in the severe forms may have S3 and murmur of MR (muffled first heart sound, along with a third heart sound and a murmur of mitral regurgitation) May be self limited without any sequelae but severe involvement may occur Likely progresses to a chronic form and to dilated cardiomyopathy Patients with viral myocarditis often present with flulike illness Patients who present with CHF may respond to usual measures Death due to arrhythmias had been reported e.g. a 45 year old female patient with a 3 day history of viral illness, hyperventilating in the ER with elevated ST segment was thought to be having MI since she is also diabetic, coronary angiogram was however normal, the patient went back to the CCU and had ventricular fibrillation and ventricular tachycardia, she died within 24 hours (sometimes a physician should ask whether the patient had a prolonged viral illness) Isolation of virus from the stool, pharyngeal washings, or other body fluids and changes in specific antibody titers may be helpful clinically Endomyocardial biopsy may show round-cell infiltration and necrosis of adjacent myocytes Treatment Exercise is deleterious and serious activity is not recommended Patients with fulminant myocarditis may require mechanical cardiopulmonary support or cardiac transplantation Treat them like any patient with CHF, since they may have CHF having an underlying problem of myocarditis. CHF is found to be a manifestation of certain structural problems in the heart. *** The problem in myocarditis lies within the cardiac muscle, coronary artery is normal. There is no problem with the blood supply but more on the muscle. This condition is actually more difficult to treat since there is no transplantation in the country. CARDIOMYOPATHIES It is a primary disorder of the heart muscle that causes abnormal myocardial performance and is not the result of disease or dysfunction of other cardiac structures, myocardial infarction, systemic hypertension, valvular stenosis or regurgitation Definition: Heart muscle disorder of unknown etiology Disease of myocardium; the muscle it self (myocyte) Diagnosis of exclusion: Excluding the causes: ischemia, drugs, viruses, etc. Confusion: some exclude any disorder of known etiology, while some do not

Can be divided to 3 groups: Dilated (congestive) (90%) Commonest type. Big heart on chest X-Ray. Patient comes with heart failure. Hypertrophic (< 10%) Restrictive (< 2%) Least common Classification Etiology- primary or secondary Primary type-heart muscle disease involving the myocardium of unknown cause Secondary type-known cause, associated with a systemic disease such as amyloidosis or chronic alcohol use Gross anatomy Histology Genetics Biochemistry Immunology Hemodynamics Functional Prognosis Treatment Primary cardiomyopathies in which the clinically relevant disease processes solely or predominantly involve the myocardium:

There are so many ways to classify cardiomyopathies, for our purposes, functional classification shall be used. Functional Classification (based on morphology) Dilatated (congestive, DCM, IDC) - 90% Ventricular enlargement and systolic dysfunction EF is low Hypertrophic (IHSS, HCM, HOCM) - 10% Inappropriate myocardial hypertrophy in the absence of HTN or aortic stenosis Thickened heart muscles due to genetic mutations and not secondary to HTN You cannot say hypertrophic cardiomyopathy in hypertensive patients since what a hypertensive has is a hypertrophied muscle not hypertrophic Restrictive (infiltrative)-<2%, least common Abnormal filling and diastolic function Restrictive heart muscles are infiltrated by certain substances, the notorious one would be amyloidosis

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Core Povenum like a heart of a cow Predicting Prognosis in IDC

with normal walls dilated cardiomyopathy the cavity is big but the walls may be normal or filled up hypertrophic cardiomyopathy with a very small left ventricular cavity since the heart muscles are so thick restrictive cardiomyopathy with thick heart muscles but not as thick or as severe as that in hypertrophic DILATED CARDIOMYOPATHY It is a disease of unknown etiology that principally affects the myocardium LV and/or right dilatation and systolic dysfunction, arrhythmias, heart failure LV or RV systolic pump function is impaired leading to progressive cardiac dilatation(remodeling) Progressive cardiac hypertrophy, dilation, and systolic dysfunction (pump failure) Affects both ventricles Pathology: Gross Increased heart size (enlarged and flabby) and weight (may exceed 900 g) Ventricular dilatation, normal wall thickness Dilation and hypertrophy in all 4 chambers Heart dysfunction out of portion to fibrosis Mural thrombi due to stasis CAD minimal or absent Histology Myocyte hypertrophy Interstitial fibrosis Wavy fiber change or myofiber loss Scanty mononuclear inflammatory infiltrate Arrhythmogenic RV Dysplasia Uncommon, familial form that affects young adults causing sudden death RV is markedly thinned & replaced by fat CPC required (fat may be seen in normal) Causes of Dilated CMP Postviral myocarditis (late stage) Peripartum During the last trimester or within 6mo of delivery Alcohol toxicity Those who consume >90g/d for many years Familial Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D)-autosomal dominant w/ multiple mutations. It is characterized by progressive fibrofatty replacement of the right ventricle and, to a much lesser degree, of the LV myocardium Drugs Doxorubicin (Adriamycin) Anti-cancer therapy. Trastuzumab(Herceptin) tx for breast cancer Imatinib mesylate(Gleevac) tx of chronic myeloid leukemia Idiopathic Neuromuscular Disease Cardiac involvement common in muscular dystrophies like in Duchennes progressive muscular dystrophy and in myotonic dystrophy Clinical Manifestations Highest incidence in middle age Becomes apparent in 3rd r 4th decade of life Blacks 2x more frequent than whites Men 3x more frequent than women Symptoms may be gradual in onset Acute presentation Misdiagnosed as viral URI in young adults Uncommon to find specific myocardial disease on endomyocardial biopsy Right: Normal Heart. Left: Dilated CMP History and Physical Examination Symptoms of heart failure Pulmonary congestion (left HF), dyspnea (rest, exertional, nocturnal), orthpnea Systemic congestion (right HF), edema, nausea, abdominal pain, nocturia low cardiac output, fatigue and weakness

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Hypotension, tachycardia, tachypnea, JVD Syncope due to arrhythmias and systemic embolism may occur Cardiac Imaging Chest radiogram Big heart on chest X-Ray Electrocardiogram Sinus tachycardia or atrial fibrillation, ventricular arrhythmias, left atrial abnormality, low voltage, diffuse nonspecific ST-T-wave abnormalities, and sometimes intraventricular and/or AV conduction defects 24-hour ambulatory ECG (Holter) Lightheadedness, palpitation, syncope, arrythmias Two-dimensional echocardiogram Central in diagnosis Radionuclide ventriculography Cardiac MRI Used in tissue determination, it has tissue characterization In end stage coronary artery disease the heart muscles may also be affected and may become myopathic which is called ischemic cardiomyopathy. In MRI there are certain patterns in which they can say if the affectation of the heart muscle is due to ischemia or due to myocarditis. Cardiac catheterization Age >40, ischemic history, high risk profile, abnormal ECG Used to exclude any correctable lesions There should be benefit of the doubt, coronary angiogram is performed since there is a possibility to correct something, e.g. cardiomyopathy is an indication for heart transplant in doing angiogram there is a possibility to correct if the condition is indeed a muscle problem instead of a coronary problem and in doing so you may prevent an unnecessary heart transplant. Clinical Indications for Endomyocardial Biopsy **Endomyocardial Biopsy is not done in the Philippines Definite Monitoring of cardiac allograft rejection Monitoring of anthracycline cardiotoxicity Possible Detection and monitoring of myocarditis Diagnosis of secondary cardiomyopathies Differentiation between restrictive and constrictive heart disease HYPERTROPHIC CARDIOMYOPATHY First described by the French and Germans around 1900 Uncommon with occurrence of 0.02 to 0.2% A hypertrophic heart and a non-dilated left ventricle in the absence of another disease Small LV cavity, asymmetrical septal hypertrophy (ASH), systolic anterior motion of the mitral valve leaflet (SAM) Additional Notes: Hypertrophic Cardiomyopathy in this manner effects a segment, which is left ventricle. The heart is stiff to contract during systole & is soft to dilate during diastole. But when the heart becomes very dilated , it can not pump enough. Resulting in heart failure. If the heart is very stiff, it can not fill during diastole. Leading to reduced Ejection Fraction & ventricular out flow obstruction. The manifestations of this condition are: ischemia & sudden death. due to coronary circulation problems. Forms of Hypertrophic Cardiomyopathy: Septum may be hypertrophic while the lateral walls are both normal You may have both walls hypertrophied Just an apical hypertrophy Both walls affected but more affectation on the septum

Mitral leaflet is supposedly closed during systole but because of the thickness of the septum the anterior mitral leaflets are being sucked by the septum, this event is called the Venturi effect. Venturi effect is the suctioning effect of the septum. What happens during the systole is that the blood should go to the periphery but there is an obstruction, a dynamic LV outflow tract obstruction meaning it changes unlike in aortic stenosis where the LV outflow tract obstruction is fixed. CAUSES of CARDIAC HYPERTROPHY Pressure Overload Hypertension Valvular Stenosis (Aortic) Volume Overload Valvular Insufficiency Myocardial Injury Myocardial infarction Myocarditis Cardiomyopathy FAMILIAL HCM First reported by Seidman et al in 1989 Occurs as autosomal dominant in 50% 5 different genes on at least 4 chromosome with over 3 dozen mutations * chromosome 14 (myosin) * chromosome 1 (troponin T) * chromosome 15 (tropomyosin) * chromosome 11 (?) Pathophysiology Systole Dynamic outflow tract gradient/obstruction Diastole Impaired diastolic filling, filling pressure Elevated LV end-diastolic pressure Myocardial ischemia

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muscle mass, filling pressure, O2 demand vasodilator reserve, capillary density Abnormal intramural coronary arteries Systolic compression of arteries The heart is stiff to contract during systole & is soft to dilate during diastole. But when the heart becomes very dilated, it can not pump enough. Resulting in heart failure. If the heart is very stiff, it can not fill during diastole. Leading to reduced. Ejection Fraction & ventricular out flow obstruction. The manifestations of this condition are: ischemia & sudden death due to coronary circulation problems. Morphology Gross Myocardial hypertrophy (90% asymmetric) Abnormal diastolic filling (impaired relaxation) Left atrial dilatation Intermittent ventricular outflow obstruction (50-75%) Mutations on chromosome 14 in gene coding for beta myosin heavy chain in 50% of cases Sudden death, dyspnea, angina Cardiomegaly, septal asymmetry, myofiber disarray Microscopic Haphazard arrangement of hypertrophied myocytes, surrounded by loose, basophilic ground substance Myocardial fibrosis may occur late

Atrial and ventricular arrhythmias Cardiac silhouette in chest X-ray Echocardiogram shows LV hypertrophy, with septum >1.3 times thickness of post LV free wall and ground-glass appearance Natural History Annual mortality 3% in referral centers probably closer to 1% for all patients Risk of SCD higher in children may be as high as 6% per year majority have progressive hypertrophy Clinical deterioration usually is slow Progression to DCM occurs in 10-15% Risk Factors for SCD Young age (<30 years) Malignant family history of sudden death Gene mutations prone to SCD (ex. Arg403Gln) Aborted sudden cardiac death Sustained VT or SVT Recurrent syncope in the young Nonsustained VT (Holter Monitoring) Brady arrhythmias (occult conduction disease) Recommendations for Athletic Activity Avoid most competitive sports (whether or not symptoms and/or outflow gradient are present) Low-risk older patients (>30 yrs) may participate in athletic activity if all of the following are absent Low-risk older patients (>30 yrs) may participate in athletic activity if all of the following are absent Ventricular tachycardia on Holter monitoring Family history of sudden death due to HCM History of syncope or episode of impaired consciousness Severe hemdynamic abnormalities, gradient 50 mmHg Exercise induced hypotension Moderate or sever mitral regurgitation Enlarged left atrium (50 mm) Paroxysmal atrial fibrillation Abnormal myocardial perfusion Management Beta-adrenergic blockers Calcium antagonist Disopyramide Amiodarone, sotolol DDD pacing Myotomy-myectomy Plication of the anterior mitral leaflet HCM vs. Aortic Stenosis HCM Aortic Stenosis Carotid Pulse Spike and dome Parvus et Tardus Murmur valsalva, standing Radiate to carotids squatting, handgrip passive leg extension Systolic thrill 4th left interspace 2nd right interspace Systolic click absent present *parvus slow; tardus - late Most murmurs would increase with increase in blood volume but in HCM murmurs, it decreases because the cavity increases. When you do Valsalva, strain would take place leading into increased intrathoracic pressure thus decreasing venous return. Decrease in venous return promotes a smaller cavity thus HCM murmur increases. Aortic stenosis murmur however decreases In squatting, you increase venous return so you decrease HCM murmur When standing up, you decrease venous return so you increase HCM murmur Other Causes of Hypertrophy Clinical mimics Glycogen storage, infants of diabetic mothers, amyloid Genetic

Hypertrophy in all the layers in every whereConcentric hypertrophy. Commonest cause is long standing systemic hypertension. Pressure over-load in the aortic valve stenosis or regurgitation that will end up in dilatation & heart failure.

Hypertrophic CMP showing thickening of muscle fibers Clinical Manifestation Asymptomatic, echocardiographic finding Symptomatic Dyspnea in 90% Angina pectoris in 75% Fatigue, pre-syncope, syncope, risk of SCD in children and adolescents(common cause of SCD in young competitive athletes) Palpitation, PND, CHF, dizziness less frequent Double or triple apical precordial impulse and a fourth heart sound Hallmark of obstructive HCM is a systolic murmur, which is harsh, diamond-shaped, and usually begins well after the first heart sound, best heard at lower left sternal border as well as at the apex, where it is often more holosystolic and blowing in quality, due to the mitral regurgitation that usually accompanies obstructive HCM ECG commonly shows LV hypertrophy and widespread deep, broad Q waves

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Noonans, Friedreichs ataxia, Familial restrictive cardiomyopathy with disarray Exaggerated physiologic response Afro-Caribbean hypertension, old age hypertrophy, athletes heart HCM vs Athletes Heart

Causes of Restrictive CMP Endomyocardial Fibrosis Most common cause wordwide 10% of cases of childhood heart disease in tropical regions The heart becomes less compliant due to fibrosis during diastole the heart will not dilate properly Infiltrative Heart Disease Amyloid Hemosiderin Deposition (HH) Idiopathic Morphology Gross Varies depending on cause Atrial dilation (bilateral) Endocardium thickened and opaque Valvular thickening Mural thrombi may be present Microscopic Dense endocardial fibrosis, extending into subendocardial myocardium Eosinophils in some cases

Hypertensive HCM of the Elderly Characteristics Modest concentric LV hypertrophy (<22 mm) Small LV cavity size Associated hypertension Ventricular morphology greatly distorted with reduced outflow tract Sigmoid septum and grandma SAM RESTRICTIVE CARDIOMYOPATHY Primary decrease in ventricular compliance, resulting in impaired ventricular filling during diastole. Restricting the heart movement Restricting the heart movement. Constrictive / restrictive precarditis precardium is enclosed in fibrous wall. Least common type of cardiomyopathy Hallmark Abnormal diastolic function Rigid ventricular wall with impaired ventricular filling Bear some functional resemblance to constrictive pericarditis Constrictive / restrictive precarditis precardium is enclosed in fibrous wall. Importance lies in its differentiation from operable constrictive pericarditis Classification Idiopathic An idiopathic enzymatic or metabolic disturbance has been postulated in some patients Clinical features include severe pulmonary congestion, arrhythmias, heart block, and cardiac failure Rapid fatal course in children, >5 yrs survival in adults Myocardial a. Noninfiltrative Idiopathic Scleroderma b. Infiltrative Amyloid-hemosiderin deposits (amyloidosis is diagnosed through rectal biopsy) Sarcoid Gaucher disease Hurler disease c. Storage Disease Hemochromatosis Fabry disease Glycogen storage Endomyocardial Endomyocardial fibrosis- most common cause worldwide, 10% of cases of childhood heart disease in tropical region is from this Hyperesinophilic syndrome Carcinoid Metastatic malignancies Radiation, anthracycline

Dilated Atria (bilateral dilatation but normal ventricles) Clinical Manifestations Symptoms of right and left heart failure Exercise intolerance and dyspnea are usually prominent due to inability of ventricles to fill which limits the cardiac output and raises the filing pressure Dependent edema, ascites, enlarged, tender, pulsatile liveer due to persistently elevated systemic pressure Heart sounds may be distant, and third and fourth heart sounds are common Jugular Venous Pulse Prominent x and y descents Echo-Doppler Abnormal mitral inflow pattern Prominent E wave (rapid diastolic filling) Reduced deceleration time ( LA pressure) Constrictive - Restrictive Pattern Square-Root Sign or Dip-and-Plateau

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Hemochromatosis: iron deposition in all orgens Often the result of multiple transfusions or a hemoglobinopathy, most frequently thalassemia Familial (autosomal recessive) form should be suspected if cardiomyopathy occurs in the presence of diabetes mellitus, hepatic cirrhosis, and increased skin pigmentation Diagnosis may be confirmed by endomyocardial biopsy Drug-Induced Cardiomyopathy Adriamycin most common drug Other drugs (chloroquine, amiodarone) These drugs treat cancer & are cardiotoxic Additional from Harrisons but not included in the lecture: Hypertophic Cardiomyopathy: Tako-Tsubo (Stress) Cardiomyopathy Apical ballooning syndrome Uncommon cardiac syndrome, characterized by the abrupt onset of severe chest discomfort preceded by a very stressful emotional or physical event Occurs most commonly in women >50 years and is accompanied by ST-segment elevations and/or deep T-wave inversions in the precordial leads. "Ballooning" of the left ventricle in end-systole, especially of the LV apex is seen Unclear mechanism, although it is likely that an adrenergic surge that includes circulating catecholamines are involved. Reversible within 37 days Left Ventricular Noncompaction Recently characterized uncommon congenital cardiomyopathy that may present at any age with symptoms of CHF, thromboembolism, or ventricular arrhythmias Results from the arrest of normal embryogenesis, with the persistence of the deep recesses and sinusoids in the myocardium that characterize the embryonic heart Multiple deep trabeculations into the myocardium in echocardiography Inherited Metabolic Cardiomyopathies with Left Ventricular Hypertrophy Cardiac Danon Disease Caused by mutations in an X-linked lysosome-associated membrane protein (LAMP2). Characterized by enlarged ventricular myocytes with periodic acid Schiff (PAS)positive inclusions Patients present in childhood with CHF and serious arrhythmias ECG shows severe LV hypertrophy and ventricular preexcitation Glycogen Storage Cardiomyopathy Caused by a mutation in the 2 regulatory subunit (PRKAG2) of adenosine monophosphateactivated protein kinase (AMPK). Characterized by ventricular hypertrophy resembling that observed in hypertrophic cardiomyopathies and enlarged myocytes with vacuoles in the myocytes that stain for glycogen Fabry Disease X-linked autosomal recessive lysosomal storage disorder , caused by deficiency of lysosomal -galactoside A and can lead to the accumulation of glycosphingolipids in the heart, with ventricular hypertrophy resembling HCM May be associated with AV conduction abnormalities and ventricular tachyarrhythmias Treatment consists of enzyme replacement therapy with agalsidase Friedreich's Ataxia Autosomal recessive spinocerebellar degenerative disease caused by inadequate levels of frataxin, a protein involved in mitochondrial iron metabolism Symmetric LV hypertrophy or asymmetric hypertrophy of the interventricular septum compared with the free wall is seen

Square Root sign Restriction vs. Constriction History provides important clues Constrictive pericarditis History of TB, trauma, pericarditis, sollagen vascular disorders Restrictive cardiomyopathy amyloidosis, hemochromatosis Mixed mediastinal radiation, cardiac surgery *** Sometimes it is very hard to differentiate Restrictive Cardiomyopathy from Constrictive Pericarditis because it is hard to look into the pericardium in a usual 2d echo. Most of the time youll be having a mixed type. Treatment No satisfactory medical therapy Drug therapy must be used with caution Diuretics for extremely high filling prssures Vasodilators may decrease filling pressure ? Calcium channel blockers to improve diastolic compliance Digitalis and other inotropic agents are not indicated Cardiac Amyloidosis Characterized by the extracellular deposition of fibrillary proteins, often resulting in diastolic and systolic dysfunction. Amyloid proteins are diverse, but share several characteristics. By light microscopy, amyloid is amorphous and eosinophilic, demonstrating apple green birefringence with polarized light after Congo red staining. Ultrastructurally, amyloid fibrils measure 7-10 nm in diameter and do not branch. The protein demonstrates a crossed b pleated sheet structure by infrared and x-ray diffraction techniques. Three main types AL (light chains, plasma cell dyscrasias) AA (chronic inflammatory diseases, rare in heart) AS (transthyretin, senile amyloid) Pathology 10 nm non-branching extracellular fibrils Homogeneous waxy material present in interstitium, vessels or nodular Congo red positive, apple green birefringence Clinical Setting Constrictive vs. restrictive disease patient presents with congested neck veins, synosis & edema. Amyloid causes malignancy called Multiple Myloma. Hemochromatosis Iron not normally found in heart Pathology Perinuclear distribution Epicardial portions preferentially involved C282Y or H63D mutation (chromosome 6) HLA-associated TfR gene Clinical Setting Dilated or restrictive cardiomyopathy

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Restrictive Cardiomyopathy Eosinophilic Endomyocardial Disease Loeffler's endocarditis and fibroplastic endocarditis, occuring in temperate countries Cardiac damage the apparent result of the toxic effects of eosinophilic proteins Cardiac imaging typically reveals ventricular thickening, especially of the posterobasal LV wall Mitral regurgitation is frequently present on Doppler echocardiography Management usually includes diuretics, afterload-reducing agents, anticoagulation, glucocorticoids and hysroxyurea Myocardial sarcoidosis Associated with other manifestations of systemic disease There is increased stiffness of the myocardium and diminished systolic contractile function Common cardiac manifestation is RV overload due to pulmonary hypertension as a result of parenchymal pulmonary involvement Treated empirically by glucocorticoids Carcinoid syndrome Results in endocardial fibrosis and stenosis and/or regurgitation of the tricuspid and/or pulmonary valve MYOCARDITIS Myocarditis in Patients with HIV Most common finding is LV dysfunction that in some cases appears to be due to infection of the myocardium by the virus itself CHF in HIV myocarditis may respond, at least transiently, to standard anti-HIV therapy Bacterial Myocarditis Uncommon, it is usually as a complication of infective endocarditis in which abscess formation involves the valve rings and interventricular septum. Diphtheritic myocarditis Develops in over one-quarter of patients with diphtheria One of the more serious complications and the most common cause of death in this infection. Cardiac damage is due to the liberation of a toxin that inhibits protein synthesis and leads to a dilated, flabby, hypocontractile heart Cardiomegaly and severe CHF typically appear after the first week of illness Lyme Carditis caused by a tick-borne spirochete 10% of patients develop symptomatic cardiac involvement during the acute phase of the disease. AV conduction abnormalities are the most common manifestations of involvement and may lead to syncope Chagas Heart Disease Caused by the protozoan Trypanosoma cruzi and transmitted by an insect vector, the reduvid bug Extensive myocarditis that typically becomes evident years after the initial infection One of the more common causes of heart disease encountered in Central and South America;2075% of the population may be affected in rural, endemic Varies widely, from asymptomatic to severe cardiac failure involving the left ventricle, and sometimes the right ventricle, and/or AV block More frequent in males Characterized by dilatation of several cardiac chambers, fibrosis and thinning of the ventricular wall, aneurysm formation in the left ventricle , and mural thrombi Survival is poor in patients who develop overt CHF,the cause of death is either intractable CHF or SCD secondary to an arrhythmia, with a minority of patients dying from embolic phenomena.

By this all will know that you are my disciples, if you have love for one another John13:35 as always, hello to my brods and sisses, kenj, tristan, ana, hayz, vin, tel, mel, faith, charm, and rors, hi na din sa 2013 and 2014 brods and sisses, =). to my basketball teammates from 2011-2014, CHAMPIONS! YEYYYYY! =) sa PPG, hey guys, la pang wish list! hiyee, sa mga groupmates ko lalo na sa surge, malapit na ang kinakatakutan natin! and finally sa nakabunot sakin ayoko ng cash, =P -jessa p.s. salamat charlowe, sa pagsend ng audio

Salamat sa Med Trans Team! Vincent Reolalas Hayzle Mallari Abby Maralit Ana Santos Rors Reyes Vin Sanchez Den Palines Virra Rosales James Rondal Jessa Marapao Trish Navarro Armi Menchavez Pepin Mendieta Joie Silva Co-Neil Relato Marth Tarroza Glen Ventanilla Khei Villanueva Yvonne Vias Car Real Nats Quan Jess Prego Emma Sinco God bless 2012! Salamat Gayle Limos and Trish Navarro para sa lahat ng Audio - carlo benjamin z. taada

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