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PERICARDIAL DISEASES GENERAL CONSIDERATIONS • The pericardium consists of two layers: the inner visceral layer, which is attached to the epicardium, and an outer parietal layer. • About 50 mL of serous fluid is normally present and provides lubrication between the two layers. • The pericardial reflection encompasses the heart and great vessels. • The pericardium stabilizes the heart in anatomic position and reduces contact between the heart and the surrounding structures. • It is composed of fibrous tissue and although it will permit moderate changes in cardiac size, it cannot stretch rapidly enough to accommodate rapid dilation of the heart or accumulation of fluid without increasing intrapericardial (and, therefore, intracardiac) pressure. • The pericardium is often involved by processes that affect the heart, but it may also be affected by diseases of adjacent tissues and may itself be a primary site of disease. Physiology of the pericardium • The normal pericardium is not essential to life: the pericardial space is often obliterated after open heart surgery and both layers may be removed in patients with constrictive pericarditis without apparent ill effect. • Whether restraint by the normal pericardium is of any pathophysiological importance as a mechanism limiting stroke volume in disease remains uncertain. Acquired pericardial disease • Diseases of the pericardium may be considered from two points of view. • The first is etiological, the second is in terms of the physiological and clinical disturbances that result. • There is no fixed relation between the two, so that an account will be given of the different diseases affecting the pericardium and then of the three main syndromes: acute pericarditis, pericardial tamponade and pericardial constriction. Pericarditis • Pericarditis is a condition in which the sac-like covering around the heart (pericardium) becomes inflamed. • Pericarditis and cardiac tamponade involve the potential space surrounding the heart or pericardium. • Pericarditis is one cause of fluid accumulation in this potential space and cardiac tamponade is the hemodynamic result of fluid accumulation. Who gets pericarditis and what does it feel like? • This problem occurs most often in men ages 20 to 50. • Chest pain is common, especially pain behind the breastbone. Sometimes this pain spreads to the neck and left shoulder. • Pain from pericarditis is different from angina. Angina feels like pressure, but pericarditis usually is a sharp, piercing pain over the center or left side of the chest. Often this pain gets worse if the person takes a deep breath. Less often the pain is dull. • A fever is also common. • Often people with pericarditis report feeling sick. • Some have pain when they swallow. Acute pericarditis • By far the most common pathologic process involving the pericardium. • May be classified both clinically and etiologically. • Acute (< 2 weeks) inflammation of the pericardium may be infectious in origin or may be due to systemic diseases (autoimmune syndromes, uremia), neoplasm, radiation, drug toxicity, hemopericardium, postcardiac surgery or contiguous inflammatory processes in the myocardium or lung. Acute pericarditis • Incidence – Post mortem 1-6%, diagnosed in only 0.1% of hospitalized patients. 5% of patients seen in emergency rooms with CP and no MI. • Sequelae – Cardiac tamponade Recurrent pericarditis Pericardial constriction Etiology • • • • • • • • • • • Viral or idiopathic After MI Infectious diseases With dissecting aortic aneurysms Trauma Metastasis XRT Uremia After cardiac or other thoracic surgery Autoimmune diseases Medications Viral infections • especially infections with coxsackieviruses and echoviruses but also influenza, Epstein–Barr, varicella, hepatitis, mumps and HIV viruses are the most common cause of acute pericarditis and probably are responsible for many cases classified as idiopathic. • Males—usually under age 50 years—are most commonly affected. • The differential diagnosis is primarily with myocardial infarction. Tuberculous pericarditis • has become rare in developed countries but remains common in other areas • It results from direct lymphatic or hematogenous spread; clinical pulmonary involvement may be absent or minor, although associated pleural effusions are common. Bacterial pericarditis • has become rare and usually results from direct extension from pulmonary infections • Pneumococci can cause a primary pericardial infection. • Borrelia burgdorferi, the organism responsible for Lyme disease, can also cause myopericarditis. Uremic pericarditis • is a common complication of renal failure • the pathogenesis is uncertain • it occurs both with untreated uremia and in otherwise stable dialysis patients Neoplastic pericarditis • Spread of adjacent lung cancer as well as invasion by breast cancer, renal cell carcinoma, Hodgkin disease and lymphomas are the most common neoplastic processes involving the pericardium and have become the most frequent causes of pericardial tamponade in many countries. Dressler syndrome • Pericarditis may occur 2–5 days after infarction due to an inflammatory reaction to transmural myocardial necrosis postmyocardial infarction or postcardiotomy pericarditis . Post-radiotherapy pericarditis • Radiation can initiate a fibrinous and fibrotic process in the pericardium, presenting as subacute pericarditis or constriction. • Radiation pericarditis usually follows treatments of more than 4000 cGy delivered to ports including more than 30% of the heart. Other causes of pericarditis • connective tissue diseases, such as lupus erythematosus and rheumatoid arthritis • drug-induced pericarditis (minoxidil, penicillins) • myxedema Clinical presentation • History • Physical exam - 85% have audible friction rub during the course of their disease ( the rub is high pitched scratchy or squeaky sound best heard at the left sternal border at end of expiration with the patient leaning forward) • The rub has three components- atrial systole, ventricular systole and rapid ventricular filling during early diastole. Symptoms and Signs • The presentation and course of inflammatory pericarditis depend on its cause, but all syndromes are often (not always) associated with chest pain, which is usually pleuritic and postural (relieved by sitting). • The pain is substernal but may radiate to the neck, shoulders, back or epigastrium. • Dyspnea may also be present and the patient is often febrile. • A pericardial friction rub is characteristic , with or without evidence of fluid accumulation or constriction Clinical findings • There are three main components to the clinical syndrome of acute pericarditis: 1. chest pain 2. pericardial rub 3. ECG changes Chest pain • The pain is usually retrosternal, continuous and sharp or 'raw' in character. • It is frequently aggravated by sudden movements or deep inspiration and is relieved by sitting up. • Less commonly it may resemble angina pectoris, or may be mild and 'atypical'. • Painful breathing causes dyspnoea. • The onset of the pain is usually sudden, but in idiopathic pericarditis, it may have been preceded by several days' malaise or other non-specific symptoms. Pericardial pain • Characteristically, however, pericardial pain may be relieved by sitting up and leaning forward and is intensified by lying supine. Pericardial friction rub • the most important physical sign of acute pericarditis • may have up to three components per cardiac cycle • high-pitched, scratching and grating • it is heard most frequently during expiration with the patient in an upright and leaning forward position • the rub is often inconstant and the loud to-andfro leathery sound may disappear within a few hours, possibly to reappear the following day Tuberculous pericarditis • The presentation tends to be subacute, but nonspecific symptoms (fever, night sweats, fatigue) may be present for days to months. • Pericardial involvement develops in 1–8% of patients with pulmonary tuberculosis. Bacterial pericarditis • Symptoms and signs are similar to those of other types of inflammatory pericarditides, but patients appear toxic and are often critically ill. Uremic pericarditis • can present with or without symptoms • fever is absent Neoplastic pericarditis • often is painless • the presenting symptoms relate to hemodynamic compromise or the primary disease Postmyocardial infarction or postcardiotomy pericarditis (Dressler syndrome) • Usually presents as a recurrence of pain with pleuralpericardial features • A rub is often audible, and repolarization changes on the ECG may be confused with ischemia. • Large effusions are uncommon and spontaneous resolution usually occurs in a few days. • Dressler syndrome occurs weeks to several months after myocardial infarction or open heart surgery, may be recurrent and probably represents an autoimmune syndrome. • Patients present with typical pain, fever, malaise and leukocytosis. • Rarely, other symptoms of an autoimmune disorder, such as joint pain and fever, may occur. • Tamponade is rare with Dressler syndrome after myocardial infarction but not when it occurs postoperatively. Radiation pericarditis • The clinical onset is usually within the first year but may be delayed for many years. PERICARDIAL EFFUSION • Pericardial effusion can develop during any of the processes previously discussed. • The speed of accumulation determines the physiologic importance of the effusion. • Because the pericardium stretches, large effusions (> 1000 mL) that develop slowly may produce no hemodynamic effects. Conversely, smaller effusions that appear rapidly can cause tamponade. • Tamponade is characterized by elevated intrapericardial pressure (> 15 mm Hg), which restricts venous return and ventricular filling. As a result, the stroke volume and pulse pressure fall and the heart rate and venous pressure rise. Shock and death may result. Pericardial effusion • Differentiation from cardiac enlargement may be difficult on physical examination, but heart sounds tend to become faint with pericardial effusion; the friction rub may disappear and the apex impulse may vanish, but sometimes it remains palpable, albeit medial to the left border of cardiac dullness. • The base of the left lung may be compressed by pericardial fluid, producing Ewart’s sign, a patch of dullness beneath the angle of the left scapula. • The chest roentgenogram may show a “water bottle” configuration of the cardiac silhouette, but may also be normal or almost so. Symptoms • Pericardial effusions may be associated with pain if they occur as part of an acute inflammatory process or may be painless, as is often the case with neoplastic or uremic effusion. • Dyspnea and cough are common, especially with tamponade. • Other symptoms may result from the primary disease. SIGNS • A pericardial friction rub may be present even with large effusions . • In cardiac tamponade, tachycardia, tachypnea, a narrow pulse pressure and a relatively preserved systolic pressure are characteristic. • Pulsus paradoxus—a greater than 10 mm Hg decline in systolic pressure during inspiration due to further impairment of LV filling—is the classic finding, but it may also occur with obstructive lung disease. • Central venous pressure is elevated and there is no evident y descent in the RA, RV or LV hemodynamic tracings. • Edema or ascites are rarely present; these signs favor a more chronic process. Investigations • Electrocardiogram • Chest X-ray • Echocardiography ECG • in acute pericarditis without massive effusion usually displays changes secondary to acute subepicardial inflammation • usually there are no significant changes in QRS complexes, except for some reduction in voltage in patients with large pericardial effusions • elevation of the ST segments, often with upward concavity, involving two or three standard limb leads and V2 to V6, with reciprocal depressions only in aVR and sometimes V EKG • Stages of pericarditis: I- Diffuse ST elevation and PR segment depression (seen in more than 80%) II- Normalization of the ST and PR III- Widespread T- wave inversions IV- Normalization of the T waves • The most reliable distinguishing feature may be the ratio of ST segment elevation (in millimeters) to Twave amplitude in lead V6; ratio > 0.24 PERICARDIAL DISEASE This 12-lead electrocardiogram is representative of pericarditis. Stage 1 electrocardiograph changes in a patient with acute pericarditis. Stage 2 ECG changes in patient with acute pericarditis obtained 3 days into clinical course. ECG criteria: • 1. 2. • 1. 2. • 1. 2. 3. • 1. 2. ACUTE PERICARDITIS: Concave (saddle-shaped) ST segment elevation; ST may be normal or nonspecific (10% cases). PERICARDIAL EFFUSION: Low voltage QRS complexes; Alternating QRS morphologies (electrical alternans). CONSTRICTIVE PERICARDITIS: Large T wave and bifid; Low voltage QRS complexes; ST plan, isoelectric or depressed. CARDIAC TAMPONADE: Low voltage QRS complexes; Electrical alternans. Chest X-ray • may suggest enlargement of heart tissue and can be used to rule out other problems within the chest Chest radiographs revealing markedly enlarged cardiac silhouette and normal-appearing lung parenchyma in prepericardiocentesis (A) and postpericardiocentesis (B). Echo • The cardiologist looks for the presence of fluid in the pericardial sac, although in many mild cases of acute pericarditis, there is no pericardial fluid seen with echocardiography. This ultrasonogram demonstrates a normal subcostal 4-chamber view of the heart. The pericardium is brightly reflective (echogenic or white in appearance). LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle. This is a modified subcostal ultrasonographic view of the heart in which a thick anechoic (dark or black) stripe is seen surrounding the heart (H). The stripe represents a large amount of fluid (F) in the pericardium, indicating tamponade. Essentials of Diagnosis • Anterior pleuritic chest pain that is worse supine than upright. • Pericardial rub. • Fever common. • Erythrocyte sedimentation rate usually elevated. • ECG reveals diffuse ST segment elevation with associated PR depression. Cardiac tamponade • Cardiac tamponade is a clinical syndrome caused by the accumulation of fluid in the pericardial space, resulting in reduced ventricular filling and subsequent hemodynamic compromise. • Cardiac tamponade is a medical emergency. • The overall risk of death depends on the speed of diagnosis, the treatment provided and the underlying cause of the tamponade. Reddy et al describe 3 phases of hemodynamic changes in tamponade • Phase I: The accumulation of pericardial fluid causes increased stiffness of the ventricle, requiring a higher filling pressure. During this phase, the left and right ventricular filling pressures are higher than the intrapericardial pressure. • Phase II: With further fluid accumulation, the pericardial pressure increases above the ventricular filling pressure, resulting in reduced cardiac output. • Phase III: A further decrease in cardiac output occurs, which is due to equilibration of pericardial and left ventricular (LV) filling pressures. Symptoms • Patients with pericardial tamponade often complain of shortness of breath, but the diagnosis is most commonly made by noting the characteristic physical examination findings associated with pericardial tamponade. • Dyspnea is the most common symptom of pericardial tamponade. The pathogenesis probably relates to a reduction in cardiac output and, in some patients, the presence of pulmonary edema. Clinical findings • • • • Systemic arterial hypotension Tachycardia Elevated JVP Pulsus paradoxus (most sensitive but not specific) • 15% of patients with idiopathic pericarditis or as many as 60% of those with neoplastic, tuberculous or purulent pericarditis can present with cardiac tamponade. The Beck triad or acute compression triad • Described in 1935, this complex of physical findings refers to increased jugular venous pressure, hypotension and diminished heart sounds. • These findings result from a rapid accumulation of pericardial fluid. Pulsus paradoxus or paradoxical pulse • This is an exaggeration (>12 mm Hg or 9%) of the normal inspiratory decrease in systemic blood pressure. • The paradox is that while listening to the heart sounds during inspiration, the pulse weakens or may not be palpated with certain heartbeats, while S1 is heard with all heartbeats. ECG • ECG shows tachycardia, often with low-voltage QRS complexes, but without Q waves or conduction disturbances. If the effusion is large, electrical alternans is present, when alternate QRS complexes show differing morphology, because the heart swings to and fro in a large (therefore usually malignant) effusion. Electrocardiogram from a patient with massive malignant pericardial effusion showing electrical alternans. Note that all are sinus beats with the same PR interval, but that the QRS axis alternates. Chest X-ray • Chest radiography shows a large globular heart, similar to that seen in dilated cardiomyopathy. • More useful in making the diagnosis, therefore, is the absence of any evidence of pulmonary congestion, which would be expected if myocardial disease were the main abnormality. • Pulmonary oedema is most unusual in pure tamponade: if it is present, it suggests additional myocardial disease. Chest X-ray • Chest radiography findings may show cardiomegaly, water bottle–shaped heart This anteroposterior-view chest radiograph shows a massive bottleshaped heart and conspicuous absence of pulmonary vascular congestion. Echocardiography • An echo-free space posterior and anterior to the left ventricle and behind the left atrium • Swinging of the heart in its sac • Inferior vena cava plethora with minimal or no collapse with inspiration Echo • Although echocardiography provides useful information, cardiac tamponade is a clinical diagnosis • Conditions that may simulate pericardial effusion on 2dimensional echocardiography include the following: A large left pleural effusion - Any tumor surrounding the heart - Mitral annular calcification - A descending thoracic aorta - A catheter in the right ventricle - An enlarged left atrium - An annular subvalvular LV aneurysm - A bronchogenic cyst Echo Findings in Pre-Tamponade Physiology • Diastolic right ventricular collapse • Right atrial collapse/inversion • Exagerated respiratory variation in inflow velocity • Exagerated respiratory variation in inferior vena cava flow • Dilated IVC in the right setting Echo-Guided Pericardiocentesis • Multiple Echo windows should be used to determine the distribution of the fluid. Specifically, the distribution and depth from the surface of the chest at which contact with the fluid is anticipated by the pericardiocentesis needle should be determined. If the location of a pericardiocentesis needle is in question, agitated saline can be injected to further define the location of the tip. Pericardiocentesis and Biopsy • In those with pericardial tamponade and in those with known or suspected purulent or neoplastic pericarditis. • In a study involving 230 patients with acute pericarditis in whom the cause was unknown, pericardiocentesis and pericardial biopsy provided a diagnosis in only 6% and 5% respectively. Laboratory evaluation • • • • • Red and white cell count. Cytology TG CTX No evidence for PH, glucose, LDH and protein measurement. • PCR > 30 U/L for adenosine deaminase activity may help in identifying MTB • Clinical presentation should guide the ordering of additional tests. Routine serologic testing, including testing for ANA and RF, reveals a source for the pericarditis for only 10-15% of patients. • Plasma troponin concentrations are elevated in 3550% of patients with pericarditis. The magnitude of the ST elevation appears to correlate with magnitude of troponin elevation. A troponin elevation lasting more than 2 weeks, suggests associated myocarditis. Prognosis Several indicators of poor prognosis: • >38°C • Subacute onset • Immunosuppressed • After trauma • Anticoagulation use • Myopericarditis • Large effusion or tamponade CHRONIC PERICARDITIS • Chronic pericardial effusions are sometimes encountered in patients without an antecedent history of acute pericarditis. • They may cause few symptoms per se, and their presence may be detected by finding an enlarged cardiac silhouette on chest roentgenogram. Tuberculosis • It is important to consider this condition in a middleaged or elderly person with fever and enlargement of the cardiac silhouette of undetermined origin, with or without elevation of venous pressure. • Weight loss, fever, and fatigability are sometimes observed. Inasmuch as treatment is quite effective, overlooking a tuberculous pericardial effusion may have serious consequences. • If the etiology of chronic pericardial effusion remains obscure, a pericardial biopsy, preferably by a limited thoracotomy, should be performed. Myxedema • Myxedema may be responsible for a pericardial effusion that is sometimes massive but rarely, if ever, causes cardiac tamponade. • The cardiac silhouette is markedly enlarged and an echocardiogram is necessary to distinguish cardiomegaly from pericardial effusion. • The diagnosis of myxedema is frequently overlooked. It is important, therefore, to carry out appropriate tests for thyroid function. Other causes • Neoplasms, SLE, rheumatoid arthritis, mycotic infections, radiation therapy, pyogenic infections, severe chronic anemia and chylopericardium may also cause chronic pericardial effusion and should be considered and specifically looked for in such patients. Tests • Aspiration and analysis of the pericardial fluid are often helpful in diagnosis, especially in patients with chronic large effusions that are nonresponsive to nonsteroidal anti-inflammatory drugs. • Fluid should be sent for hematocrit, cell count, protein, culture and cytology. • In infections the organism can often be identified by smear or culture and should lead to treatment with appropriate systemic antibiotics. • Grossly sanguineous pericardial fluid results most commonly from a neoplasm, tuberculosis, uremia or slow leakage from an aortic aneurysm. Laboratory Findings and Diagnostic Studies • The diagnosis of viral pericarditis is usually clinical and leukocytosis is often present. • Rising viral titers in paired sera may be obtained for confirmation. • Cardiac enzymes may be slightly elevated, reflecting a myocarditic component. • The echocardiogram is often normal or reveals only a trivial amount of fluid during the acute inflammatory process. Laboratory Findings and Diagnostic Studies • The diagnosis of tuberculous pericarditis can be inferred if acid-fast bacilli are found elsewhere. • The tuberculous pericardial effusions are usually small or moderate but may be large. • The yield of organisms by pericardiocentesis is low; pericardial biopsy has a higher yield but may also be negative and pericardiectomy may be required. Laboratory Findings and Diagnostic Studies • If bacterial pericarditis is suspected on clinical grounds, diagnostic pericardiocentesis may be of value. Laboratory Findings and Diagnostic Studies • In uremic patients not on dialysis, the incidence of pericarditis correlates roughly with the level of blood urea nitrogen (BUN) and creatinine. • The pericardium is characteristically "shaggy" in uremic pericarditis and the effusion is hemorrhagic and exudative. Laboratory Findings and Diagnostic Studies • The diagnosis of neoplastic pericarditis can occasionally be made by cytologic examination of the effusion or by pericardial biopsy, but it may be difficult to establish clinically if the patient has received mediastinal radiation within the previous year. • Neoplastic pericardial effusions develop over a long period of time and may become quite huge (> 2 L). Laboratory Findings and Diagnostic Studies • The sedimentation rate is high in postmyocardial infarction or postcardiotomy pericarditis. • Large pericardial effusions and accompanying pleural effusions are frequent. • Myxedema pericardial effusions due to hypothyroidism usually are characterized by the presence of cholesterol crystals. CHRONIC CONSTRICTIVE PERICARDITIS • This disorder results when the healing of an acute fibrinous or serofibrinous pericarditis or a chronic pericardial effusion is followed by obliteration of the pericardial cavity with the formation of granulation tissue. • The latter gradually contracts and forms a firm scar, encasing the heart and interfering with filling of the ventricles. May follow • • • • • • • • • • trauma cardiac operation of any type mediastinal irradiation purulent infection histoplasmosis neoplastic disease (especially breast cancer, lung cancer, and lymphoma) acute viral or idiopathic pericarditis rheumatoid arthritis SLE chronic renal failure with uremia treated by chronic dialysis Physiopathology • The basic physiologic abnormality in symptomatic patients with chronic constrictive pericarditis, as in those with cardiac tamponade, is the inability of the ventricles to fill because of the limitations imposed by the rigid, thickened pericardium or the tense pericardial fluid. • In constrictive pericarditis, ventricular filling is unimpeded during early diastole but is reduced abruptly when the elastic limit of the pericardium is reached, while in cardiac tamponade, ventricular filling is impeded throughout diastole. Symptoms • slowly progressive dyspnea • fatigue • weakness Signs • Chronic edema, hepatic congestion and ascites are usually present. • Ascites often seems out of proportion to the degree of peripheral edema. The examination reveals these signs and a characteristically elevated jugular venous pressure. • Kussmaul sign—a failure of the JVP to fall with inspiration—is also a frequent finding. • The apex may actually retract with systole and a pericardial "knock" may be heard in early diastole. • Pulsus paradoxus is unusual. • Atrial fibrillation is common. ECG Radiographic findings • The chest radiograph may show normal heart size or cardiomegaly. Pericardial calcification is best seen on the lateral view and is uncommon. It rarely involves the LV apex and finding of calcification at the LV apex is more consistent with LV aneurysm. • Cardiac CT and MRI are only occasionally helpful. Pericardial thickening of > 4 mm must be present to establish the diagnosis, yet no pericardial thickening is demonstrated in 20–25% of patients with constrictive pericarditis. MRI fast spin echo image with blood suppression in a ventricular short-axis plane showing circumferential thickening of the pericardium (black rim between the epicardial and pericardial fat) (arrow). Echocardiography • Echocardiography rarely demonstrates a thickened pericardium. • A septal "bounce" reflecting the rapid early filling is common, though. • RV/LV interaction may be demonstrated by a reduction in the mitral inflow pattern of > 25%, much as in tamponade. (A) Apical four-chamber view showing pericardial effusion. (B) Modified apical view showing a spider-web appearance formed by the fibrin strands. DIFFERENTIAL DIAGNOSIS • Cor pulmonale - chronic constrictive pericarditis may be associated with severe systemic venous hypertension but little pulmonary congestion; the heart is usually not enlarged and a paradoxical pulse may be present. • Tricuspid stenosis may also simulate chronic constrictive pericarditis; congestive hepatomegaly, splenomegaly, ascites, and venous distention may be equally prominent, and the manifestations of left-sided heart failure may be inconspicuous. • However, in tricuspid stenosis, a characteristic murmur as well as mitral stenosis are usually present. In tricuspid stenosis, a paradoxical pulse and a steep, deep y descent in the jugular venous pulse do not occur, serving to differentiate it from chronic constrictive pericarditis. Echo • The echocardiogram in chronic constrictive pericarditis characteristically shows pericardial thickening, i.e., a distinct echo posterior to the left ventricular wall and paradoxical septal motion. • The left ventricular wall moves sharply outward in early diastole on Doppler myocardial imaging. Marked respiratory variations in atrioventricular flow velocities on Doppler echocardiography are also characteristic of constrictive pericarditis but not restrictive cardiomyopathy Effusive constrictive pericarditis • Is a clinical hemodynamic syndrome in which constriction of the heart by the visceral pericardium occurs in the presence of tense effusion in a free pericardial space. • The hallmark is the persistence of elevated right atrial pressure after intrapericardial pressure has been reduced to normal levels by removal of pericardial fluid. Etiology • • • • • • • Tubercolosis pericarditis Hemopericardium Viral pericarditis Rheumatoid arthritis Cardiac surgery Radiation therapy Idiopathic Clinical features Systemic signs of venous congestion: -Ascities -Hepatomegaly and raised JVP -Dependent edema -Anorexia and postprandial fullness. Clinical features • Signs of impaired ventricular filling: -markedly raised JVP with pominent “x” and “y” descent. -Kussmaul’s sign. -Friedreich’s sign: raised JVP with sharp diastolic collapse. -pulsus paradoxis. -pericardial knock: early diastolic sound. Clinical features • Features of left sided congestion: - dyspnea - cough - ortopnea but usually are less frequent. Essentials of Diagnosis • Evidence of right heart failure with an elevated JVP, edema, hepatomegaly and ascites. • No fall or an elevation of the JVP with inspiration (Kussmaul sign). • Echocardiographic evidence for septal bounce and reduced mitral inflow velocities with inspiration. • Catheterization evidence for RV-LV interaction, a "square root" sign, equalization of diastolic pressures and normal pulmonary pressure. DIESEASES OF THE MYOCARDIUM Diseases of the myocardium • CARDIOMYOPATHY • DILATED CARDIOMYOPATHY (DCM) • HYPERTROPHIC CARDIOMYOPATHY (HCM) • INTERGRADE CARDIOMYOPATHIES • MYOCARDITIS • INFECTIOUS CAUSES • NON-INFECTIOUS CAUSES Cardiomyopathies • The cardiomyopathies are a heterogeneous group of entities affecting the myocardium primarily and not associated with the major causes of cardiac disease, ie, ischemic heart disease, hypertension, pericardial disease, valvular disease or congenital defects. • More recently, two additional entities have been added to the list: a transient cardiomyopathy due to high catecholamine discharge (Tako-Tsubo cardiomyopathy) and an embryologic defect resulting in massive trabeculation in the LV (ventricular noncompaction). • Although some have specific causes, many cases are idiopathic. • The classification of cardiomyopathies is based on features of presentation and pathophysiology Dilated cardiomyopathy • characterized by unexplained dilatation and impaired contractile performance of the left ventricle • Idiopathic • Alcoholic • Major catecholamine discharge • Myocarditis • Postpartum • Doxorubicin • Endocrinopathies • Genetic diseases Symptoms and Signs • In most patients, symptoms of heart failure develop gradually. • The physical examination reveals rales, an elevated JVP, cardiomegaly, S3 gallop rhythm , often the murmurs of functional mitral or tricuspid regurgitation, peripheral edema or ascites. • In severe CHF, Cheyne-Stokes breathing, pulsus alternans, pallor and cyanosis may be present. ECG • ST–T changes, conduction abnormalities, ventricular ectopy • Sinus tachycardia is common. • Other common abnormalities include left bundle branch block and ventricular or atrial arrhythmias. A 12-lead ECG from a young woman showing the most common electrocardiographic abnormality found in arrhythmogenic right ventricular cardiomyopathy, T-wave inversion in the precordial leads V1–V4. Chest X-ray • The chest radiograph reveals cardiomegaly, evidence for left and/or right heart failure, and pleural effusions (right > left). ECHO • An echocardiogram is indicated to exclude unsuspected valvular or other lesions and confirm the presence of dilated cardiomyopathy and reduced systolic function (as opposed to diastolic heart failure). • Mitral Doppler inflow patterns also help in the diagnosis of associated diastolic dysfunction. • Color flow Doppler can reveal tricuspid or mitral regurgitation and continuous Doppler can help define PA pressures. Echocardiographic appearances of a young patient with familial dilated cardiomyopathy. Panel A: parasternal long-axis view showing significant left atrial (LA) and biventricular dilatation with a thin intraventricular septum (IVS). Panel B: apical four-chamber view demonstrating dilatation of all four chambers. There is failure of the tricuspid leaflets to coapt in systole (arrow). LV, left ventricle; RA, right atrium; RV, right ventricle. MRI • Cardiac MRI is particularly helpful in infiltrative processes, such as sarcoidosis or hemochromatosis and is the diagnostic study of choice for RV dysplasia. • MRI can also help define an ischemic etiology by noting gadolinium enhancement consistent with myocardial scar. A transverse plane spin-echo MRI in a young woman with arrhythmogenic right ventricular cardiomyopathy demonstrating a circumscribed area of enhanced MR signal intensity in the right ventricular (RV) free wall (arrows) due to fatty infiltration. Laboratory • The serum ferritin is an adequate screening study for hemochromatosis. • The erythrocyte sedimentation rate may be low due to liver congestion. • The serum level of BNP or pro-BNP can be used to help quantitate the severity of CHF. Essentials of Diagnosis • Symptoms and signs of heart failure. • Echocardiogram confirms LV dilation, thinning and global dysfunction. Hypertrophic cardiomyopathy • Myocardial hypertrophy unrelated to any pressure or volume overload reduces LV systolic stress, increases the EF and can result in an "empty ventricle" at end-systole. • The interventricular septum may be disproportionately involved (asymmetric septal hypertrophy), but in some cases the hypertrophy is localized to mid ventricle or to the apex. • The LV outflow tract is often narrowed during systole between the bulging septum and an anteriorly displaced anterior mitral valve leaflet, causing a dynamic obstruction . • The obstruction is worsened by factors that increase myocardial contractility (sympathetic stimulation, digoxin, postextrasystolic beat) or that decrease LV filling (Valsalva maneuver, peripheral vasodilators). The amount of obstruction is preload and afterload dependent and can vary from day to day. Epidemiology • It has been increasingly appreciated that hypertrophic obstructive cardiomyopathy (HOCM) is inherited as an autosomal dominant trait with variable penetrance and is caused by mutations of one of a large number of genes, most of which code for myosin heavy chains or proteins regulating calcium handling. • The prognosis is related to the specific gene mutation. These patients usually present in early adulthood. • Elite athletes may demonstrate considerable hypertrophy that can be confused with HOCM, but generally diastolic dysfunction is not present. Transverse short axis section through the ventricles from patients with cardiomyopathy. Upper left shows symmetrical left ventricular hypertrophy in hypertrophic cardiomyopathy. Upper right shows dense white fibrous tissue obliterating the apex of both ventricles in endomyocardial fibrosis. Lower left shows a globular, dilated left ventricle in a child with dilated cardiomyopathy. Lower right shows a grossly dilated right ventricle with adipose infiltration of the right ventricular free wall in arrhythmogenic right ventricular dysplasia. Symptoms • The most frequent symptoms are dyspnea and chest pain. • Syncope is also common and is typically post exertional, when diastolic filling diminishes and outflow obstruction increases. • Arrhythmias are an important problem. • Atrial fibrillation is a long-term consequence of chronically elevated LA pressures and is a poor prognostic sign. • Ventricular arrhythmias are also common, and sudden death may occur, often in athletes after extraordinary exertion. Signs • Features on physical examination are a bisferiens carotid pulse, triple apical impulse (due to the prominent atrial filling wave and early and late systolic impulses) and a loud S4. • The JVP may reveal a prominent a wave due to reduced RV compliance. • In cases with outflow obstruction, a loud systolic murmur is present along the left sternal border that increases with upright posture or Valsalva maneuver and decreases with squatting . • These maneuvers help differentiate the murmur of HOCM from that of aortic stenosis. • Mitral regurgitation is frequently present as well. ECG • LVH is nearly universal in symptomatic patients, though entirely normal ECGs are present in up to 25%, usually in those with localized hypertrophy. • Exaggerated septal Q waves inferolaterally may suggest myocardial infarction. Chest X-ray • The chest radiograph is often unimpressive. • Unlike aortic stenosis, the ascending aorta is not dilated. ECHO • The echocardiogram is diagnostic, revealing asymmetric LVH, systolic anterior motion of the mitral valve, early closing followed by reopening of the aortic valve, a small and hypercontractile LV and delayed relaxation and filling of the LV during diastole. • The septum is usually 1.3–1.5 times the thickness of the posterior wall. Septal motion tends to be reduced. • Doppler ultrasound reveals turbulent flow and a dynamic gradient in the LV outflow tract and, commonly, mitral regurgitation. • Abnormalities in the diastolic filling pattern are present in 80% of patients. An echocardiogram (parasternal long axis view) of a patient with hypertrophic obstructive cardiomyopathy demonstrating hypertrophy of the interventricular septum (IVS), enlargement of the left atrium (LA), and systolic anterior motion of the mitral valve, bringing it into contact with the septum (arrow). • Myocardial perfusion imaging may suggest septal ischemia in the presence of normal coronary arteries. • Cardiac MRI confirms the hypertrophy and contrast enhancement frequently reveals evidence for scar at the junction of the RV attachment to the septum. • Cardiac catheterization confirms the diagnosis and assesses the presence of CAD. • Frequently, coronary arterial bridging (squeezing in systole) occurs, especially of the septal arteries. Prognosis • The natural history of HOCM is highly variable. • Several specific mutations are associated with a higher incidence of early malignant arrhythmias and sudden death and definition of the genetic abnormality provides the best estimate of prognosis. • Some patients remain asymptomatic for many years or for life. • Sudden death, especially during exercise, may be the initial event. • The highest risk patients are those with a family history of sudden death, those with marked hypertrophy and those that do not increase their systemic BP with exercise. • HOCM is the pathologic feature most frequently associated with sudden death in athletes. • Pregnancy is generally well tolerated. • Endocarditis prophylaxis is indicated. • A final stage may be a transition into dilated cardiomyopathy in 5– 10% of patients. Essentials of Diagnosis • May present with dyspnea, chest pain, syncope. • Though LV outflow gradient is classic, symptoms are primarily related to diastolic dysfunction. • Echocardiogram shows septal hypertrophy, which is usually asymmetric, and enhanced contractility. Systolic anterior motion of the anterior mitral valve is present if there is outflow tract obstruction. • The highest risk group for sudden death includes those with a marked LVH, with a family history for sudden death, with ventricular ectopy, and with an abnormal BP response to exercise. Restrictive cardiomyopathy • Restrictive cardiomyopathy is characterized by impaired diastolic filling with reasonably preserved contractile function. • The LV systolic function may be mildly depressed and the atria are generally enlarged; if present, hypertrophy of the interatrial septum is a helpful additional finding diagnostically. • The condition is relatively uncommon, with the most frequent causes being amyloidosis. Causes • endomyocardial fibrosis, a specific entity in which there is severe fibrosis of the endocardium, often with eosinophilia (Löffler syndrome) • infiltrative cardiomyopathies (eg, hemochromatosis, sarcoidosis) • connective tissue diseases (eg, scleroderma) Symptoms and Signs • Restrictive cardiomyopathy must be distinguished from constrictive pericarditis. • The key feature is that ventricular interaction is accentuated with respiration in constrictive pericarditis and that interaction is absent in restrictive cardiomyopathy. • Pulmonary pressure is invariably elevated in restrictive cardiomyopathy and is normal in uncomplicated constrictive pericarditis. Diagnostic studies • Conduction disturbances are frequently present. Low voltage on the ECG combined with ventricular hypertrophy revealed by echocardiography are suggestive. • The echocardiogram reveals a small thickened LV with bright myocardium, rapid early diastolic filling revealed by Doppler, and biatrial enlargement. Atrial septal thickening may be evident. • Cardiac MRI presents a distinctive pattern of hyper-enhancement of the gadolinium image in amyloidosis and is a useful screening test. • Rectal, abdominal fat, or gingival biopsies can confirm systemic involvement, but myocardial involvement may still be present if these are negative and requires biopsy for confirmation. • Demonstration of tissue infiltration on biopsy specimens using special stains followed by immunohistochemical studies and genetic testing is essential to define which specific protein is involved. Essentials of Diagnosis • Right heart failure tends to dominate over left heart failure. • Pulmonary hypertension present. • Amyloidosis is the most common cause. • Echocardiography is key to diagnosis. Rapid early filling is present with diastolic dysfunction. Normal or near normal EF. • MRI and cardiac catheterization are helpful. Myocardial biopsy can confirm. MIOCARDITIS • Cardiac dysfunction due to primary myocarditis is presumed to be caused by either an acute viral infection or a postviral immune response. • Secondary myocarditis is the result of inflammation caused by nonviral pathogens, drugs, chemicals, physical agents or inflammatory diseases such as systemic lupus erythematosus. Causes • The list of infectious causes of myocarditis is extensive and includes viruses with DNA and RNA cores. • The coxsackie virus is the predominant agent, but many others have been implicated. • Rickettsial myocarditis occurs with scrub typhus, Rocky Mountain spotted fever and Q fever. • Diphtheritic myocarditis is caused by the exotoxin and is often manifested by conduction abnormalities as well as heart failure. Causes • Myocarditis may also result from a hypersensitivity to drugs or may be caused by radiation, chemicals or physical agents. • In an unknown number of cases, acute myocarditis progresses to chronic dilated cardiomyopathy. Clinical findings • The clinical manifestations range from an asymptomatic state, with the presence of myocarditis inferred only by the finding of transient electrocardiographic ST-T-wave abnormalities, to a fulminant condition with arrhythmias, heart failure and death. • In some patients, myocarditis simulates acute myocardial infarction, with chest pain, electrocardiographic changes and elevated serum levels of myocardial enzymes. • Patients with myocarditis and pulmonary hypertension are at a particularly high risk of death. • Clinical examination may reveal signs of cardiac failure. The onset of heart failure may be gradual or may be abrupt and fulminant. Pleural-pericardial chest pain is common. Signs • The physical examination is often normal, although more severe cases may show a muffled first heart sound, along with a third heart sound and a murmur of mitral regurgitation. • A pericardial friction rub may be audible in patients with associated pericarditis. • Examination may reveal tachycardia, gallop rhythm and other evidence of heart failure or conduction defect. Natural history • viral myocarditis is most often self-limited and without sequelae • severe involvement may recur • it is likely that acute viral myocarditis occasionally progresses to a chronic form and to dilated cardiomyopathy BACTERIAL MYOCARDITIS • Bacterial involvement of the heart is uncommon, but when it does occur, it is usually as a complication of endocarditis. • Myocardial abscess formation may involve the valve rings and interventricular septum. • Diphtheritic myocarditis develops in over one-quarter of the patients with diphtheria, is one of the most serious complications and is the most common cause of death. • Cardiac damage is due to the liberation of a toxin that inhibits protein synthesis and leads to a dilated, flabby, hypocontractile heart; the conduction system is frequently involved as well. Investigations • ECG • Echocardiography • Chest X-ray • CT • MRI • Scinti ECG • ECG may show sinus tachycardia, other arrhythmias, nonspecific repolarization changes and intraventricular conduction abnormalities. • Ventricular ectopy may be the initial and only clinical finding. High troponin in first set of lab was highly indicative of myocarditis. During initial evaluation, the patient suddenly developed third degree heart block on the monitor but he remained asymptomatic This young man came to the emergency department with 12 hours of continuing anterior chest pain. His pain was precipitated by exertion and movement. There was no significant pleuritic component. He is not a smoker and denied history of illicit drug or cocaine use. His CPK-MB and Troponin were both abnormal. The ECG shown above was the first one taken. At this point, he was having 10 of 10 chest pain with minimal relief from nitroglycerin and morphine. Fearing an Acute Myocardial Infarction (Heart Attack), the patient was taken emergently for cardiac catheterization. Fortunately, coronary arteriography showed normal coronary arteries without any evidence of atherosclerosis. His left ventricular function was abnormal and the left ventricular cavity appeared slightly dilated. His pain persisted, off and on, for the next 2-3 days. During this time, his cardiac enzymes continued to rise and the ECG abnormalities worsened. Five days later, echocardiography showed global left ventricular dysfunction. The patient left the hospital with a diagnosis of Pericarditis/Myocarditis. Eight weeks later, his echocardiogram showed normal left ventricular function. His pain disappeared completely 96 hours after admission to the hospital. Chest X-ray • Chest radiograph is nonspecific, but cardiomegaly is frequent, though not universal. • Evidence for pulmonary venous hypertension is common and frank pulmonary edema may be present. Diagnostic studies • There is no specific laboratory study that is consistently present, though the white blood cell count is usually elevated and the sedimentation rate may increase. • Troponin I levels are elevated in about one-third of patients, but CK-MB is elevated in only 10%. • Echocardiography provides the most convenient way of evaluating cardiac function and can exclude many other processes. • Gallium-67 scintigraphy may reveal increased cardiac uptake in acute or subacute myocarditis, but it is not very sensitive. • MRI with gadolinium enhancement reveals spotty areas of injury throughout the myocardium. • Paired serum viral titers and serologic tests for other agents may indicate the cause. Two-dimensional parasternal long-axis view depicting disproportionate thickening, increased echogenicity, and dyskinesis of the inferolateral wall relative to the septum; findings are consistent with tissue oedema. Acute myocarditis in a 35-year old male presenting with acute chest pain, showing normal coronary arteries. Delayed enhancement MRI shows a strong subepicardial enhancement in the anterolateral LV wall as shown in the shortaxis view (white arrows), b the spread in longitudinal direction can be well appreciated on the vertical long-axis view (white arrows) Endomyocardial biopsy • Pathologic examinations may reveal a lymphocytic inflammatory response with necrosis, but the patchy distribution of abnormalities makes the test relatively insensitive. • By biopsy, the diagnosis of myocarditis has been established by the 1986 "Dallas" criteria. The diagnosis is dependent on describing the severity of an inflammatory infiltrate with necrosis and degeneration of adjacent myocytes. • The type of infiltrate is dependent on the causal agent; usually this is lymphocytic in viral disease, but it may be neutrophilic, eosinophilic, giant cell, granulomatous or mixed. Caption: Myocarditis. Light micrograph of human cardiac muscle with chronic myocarditis (inflammation). In this longitudinal section, muscle fibres run diagonally (purple). Abnormal features are a loss of organisation of the muscle fibres which are normally closely spaced, with the spaces between fibres (white, blue) representing oedema. There are neutrophils (white blood cells, purple dots) infiltrating the oedematous areas, as an early inflammatory response and to prevent infection. Chronic myocarditis is caused by rheumatic fever and virus infections, with enlargement of the heart. Magnification: x50 at 35mm size. Essentials of Diagnosis • Often follows an upper respiratory infection. • May present with chest pain (pleuritic or nonspecific) or signs of heart failure. • Echocardiogram documents cardiomegaly and contractile dysfunction. • Myocardial biopsy, though not sensitive, may reveal a characteristic inflammatory pattern.