CHAPTER 7 Diseases of the myocardium and pericardium 304 Table 7.2 Causes of myocarditis Viral (usually mild and self- limiting) Enterovirus (Coxsackie B) most common in Western co untries CMV Dengue Hepatitis EBV In \202 uenza Varicella HIV Spirochetal Lyme disease \200\224about 10% of patients have cardiac involvement Leptospiros is Syphilis \200\224direct involvement of myocardium with gummae is rare Protozoal Trypanos oma cruzi causes Chagas disease \200\224signi \201 cant public health problem in S. Americ a. HF may present 20 years after initial infection Fungal This is rare (typically occurs in patients with concomitant malignant disease or who are otherwise immunosuppressed) Actinomyces, Aspergillus, Candida, Cryptococcus, Histoplas ma Rickettsial Q fever rarely causes myocarditis Rocky Mountain spotted fever Scrub typhus Toxic/metabolic Anthracyclines Cocaine Catecholamines Carbon monoxide Hypocalcemia Hypersen sitivity \200\224eosinophilic in \201 ltration Bacterial Diptheria \200\224myocarditis occurs in up to 20% of diptheria infections Meningo coccus Mycoplasma Whipple \200\231s disease ETIOLOGY 305 Pericardial diseases Etiology The pericardium may be involved in the large number of diseas e processes listed in Table 7.3. In some patients, pericardial disease is the primary disea se process dominating the clinical picture, whereas in others it is a manifestation of a sy stemic disease. The most common causes are idiopathic (or viral), uremic, neoplastic, tuberculous pericardi tis, and acute myocardial infarction. The spectrum of pericardial disease is determined by the age and social circumstances of the patient. In an elderly North American and Western European population, the most common causes are ma lignancy, followed by uremia and myocar- dial infarction. In less privileged communities an d in developing countries, tuberculosis is still the predominant cause of pericardial disea se. Table 7.3 Etiology of pericardial diseases Infections Bacterial: Mycobacterium tuberculosis, Staphylococcus, Pneumococcus, Meningococcus, Mycopla sma Viral: Coxsackie, CMV, ECHO, EBV, in \202 uenza, HIV, mumps, parvo B19, rubella, varic ella Fungal: Histoplasmosis, blastomycosis Parasitic: Amoebiasis Collagen vascular diseases: systemic lupus erythematosus, polyarteritis nodosa, scleroderma , dermatomyositis Type 2 autoimmune disorders: rheumatic fever, autoreactive pericarditis, postmyocardial infarction, and postpericardiotomy syndromes Drug-induced: hydralazine, proc ainamide, penicillin, phenylbutazone Other autoimmune disorders: rheumatoid arthritis, anky losing spondylitis Autoimmune and hypersensitivity diseases Heart: acute myocardial infarction, myocarditis Lung: pulmonary infarction, pneumonia Pericardial involvement by disease in surrounding organs Malignant disease Primary: mesothelioma, sarcoma, \201 broma, lipoma Secondary: lung carcinoma, breast carc inoma, melanoma, lymphoma, leukemia Bleeding into the pericardium Trauma: penetrating and nonpenetrating Dissecting aortic aneurysm Hemorrhagic diathesis: le ukemia, scurvy Anticoagulants: warfarin Metabolic disorders Uremia, dialysis-related, myxedema, gout Miscellaneous Acute idiopathic pericarditis, radiation, sarcoidosis, amyloidosis, familial Mediterranean fever CHAPTER 7 Diseases of the myocardium and pericardium 306 Syndromes of pericardial disease Pericardial reaction to the various disease processes is l imited. The fol- lowing \201 ve clinical and pathological forms of pericarditis can thus be distinguished: 1. Acute pericarditis without effusion 2. Pericardial effusion with or wi thout tamponade 3. Constrictive pericarditis 4. Effusive \200\223constrictive pericarditis 5. Calci \201 c pericarditis without constriction ACUTE PERICARDITIS WITHOUT EFFUSION 307 Acute pericarditis without effusion ( \200\234Dry \200\235 pericarditis) Etiology Any of the causes listed in Table 7.3 may cause dry pericarditis. The clinical syn drome is commonly seen in acute viral pericarditis or after myocardial infarction. Pathology There is a \201 brinous exudate with an in \202 ammatory reaction involving th e visceral and parietal pericardium. The epicardium is also involved and this accounts for the ECG changes seen and the rise in cardiac enzymes (e.g., troponin I). Symptoms Sharp, stabbing, central chest pain is common, with radiation to the shoul- ders a nd upper arm. It is relieved by sitting up and leaning forward and is aggravated by lying d own. It may be accentuated by inspiration, cough, swallowing, or movement of the trunk. Fever, night sweats, and other constitutional symptoms may be present, depending on the underlying cause. Signs A pericardial friction rub is frequently heard. This is a super \201 cial, scratchy, grating sound that is best heard in the second to fourth intercostal spaces when pressure is exerted on the diaphragm of the stethoscope. Positioning the patient leaning forward and listening in held inspiration may bring it out. The rub is classically described as being triphasic. Usually at least two components are he ard because of atrial systole and ventricular systole. Occasionally a third component attri buted to rapid ventricular \201 lling is heard. ECG The patient is usually in sinus rhythm, but atrial \201 brillation may occur. In the early stages there is widespread ST-segment elevation with concavity directed upward, and P R segment deviation opposite the polarity of the P wave. After a few days the ST segments f ollowed by PR segments return to normal, and T waves become inverted. CXR The cardiac shadow is not enlarged in dry pericarditis. Differential diagnosis Acute pericarditis must be differentiated from myocardial infarction , spon- taneous pneumothorax, and pleurisy. CHAPTER 7 Diseases of the myocardium and pericardium 308 Diagnosis Diagnosis of acute pericarditis is based on typical symptoms of chest pain, peric ardial rub, and/or characteristic ECG changes. Management Treat the underlying cause. Good pain relief can be achieved by the use of nonsteroidal ant i-in \202 ammatory drugs (NSAIDs). PERICARDIAL EFFUSION TAMPONADE 309 Pericardial effusion tamponade Etiology Any of the causes listed in Table 7.3 may cause pericardial effusion. Large effusions are common with neoplastic, TB, uremic pericarditis, and myxedema. Pathology In addition to \201 brinous in \202 ammation, there is signi \201 cant \202 uid exudation. The pericardial \202 uid may be serous, serosanguineous, hemorrhagic, or purulent depending on the underlying cause. Hemorrhagic effusion is common in tuberculosis or neoplasia. Brownish \202 uid with an an chovy sauce appearance of the pus is highly suggestive of amoebic pericarditis. Clinical features Clinical presentation varies depending on the rate of accumulation of \202 uid, the amount of \202 uid that accumulates, and the stage at which the patient is \201 rst seen. Symptoms Chest pain may be typically pericardial (as described under Dry Pericarditis), or it may be dull and heavy from distension of the pericardium. Dyspnea is common, and orthopnea may occur later in the course of the disease. Cough may be present from compression of surrounding structures. Any constitutional symptoms depend on the cause of the disease. Signs Typically, precordial dullness extends beyond the apex beat (which may be impalpable) , and dullness is present to the right of the sternum. Dullness and bronchial breathing (Ew art \200\231s sign) at the left base posteriorly due to compression of the left lower lobe bronchus may be found. Cardiac tamponade should be considered in a patient with hypotension, raised JVP, and quiet heart sounds (Beck \200\231s triad). The primary defect in cardiac tamponade is interferen ce with diastolic \201 lling of the heart. Other clinical features of cardiac tamponade are presence of tachycar- dia, pulsus paradoxu s (fall in systolic blood pressure on inspiration of >10 mmHg or inspiratory fall in systol ic blood pressure that exceeds half the pulse pressure), elevated JVP with brisk x descents and absent y descents, dyspnea or tachypnea with clear lungs, and hepatomegaly. Investigations ECG Sinus tachycardia and generalized low-voltage QRS complexes with non- sp eci \201 c ST segment and T-wave changes are found. Electrical alternans, involving the QR S complex, suggests the presence of a massive pericardial effusion. Total electrical altern ans (P-QRS-T), which is uncommon, is path- ognomonic of cardiac tamponade. CHAPTER 7 Diseases of the myocardium and pericardium 310 CXR This commonly shows a large globular heart usually with clear lung \201 elds. Echocardiography This is diagnostic, showing an echo-free zone surrounding the heart. The \202 uid may not be evenly distributed. Signs of tamponade on echocardio- gram include the following: \200 \200 \200 \200 Diastolic collapse of the right ventricle (most speci \201 c) Diastolic collapse of the ri ght atrium Excessive respiratory variation of the mitral in \202 ow (>25%) Plethoric, nonc ollapsible inferior vena cava (IVC) The following features are more common in patients with marked in \202 am- matory or malig nant causes of pericardial effusion: \200 \200 \200 Soft tissue density masses Thickening of the visceral pericardium Presence of \201 brinou s strands The hallmark of benign, idiopathic effusion is a clear echo-free space, whereas malignancy, bacterial infection, and hemorrhagic effusions are more likely to have solid components or stranding. Cardiac catheterization This modality can be used to establish the diagnosis and severity o f tam- ponade but is rarely necessary. The important \201 ndings are 1. Equilibration of mean right atrial, right ventricular end-diastolic, and mean capillary wedge pressure, 2. Rapid x descent on the right atrial pressure waveform, and 3. Pulsus paradoxus. Differential diagnosis This includes myocardial infarction and pulmonary embolism. Management \200 When an effusion does not cause hemodynamic impairment and the cause is known (e.g., uremia , myxedema), then no further investigations are necessary and the treatment consists of tre ating the underlying cause. If the cause is not known, then pericardiocentesis must be cons idered. Aspiration of the \202 uid helps to establish the nature of the effusion. Cardiac tamponade is a life-threatening condition. Urgent pericardial aspiration is necessary (See Pericardiocentesis, p. 698). Surgical drainage is indicated for hemopericardium or purulen t pericarditis. CONSTRICTIVE PERICARDITIS 311 Constrictive pericarditis Etiology Constrictive pericarditis is usually due to tuberculosis . Other causes are mediastinal irradiation, purulent pericarditis, previous trauma (surgica l or nonsurgical) with infection of the pericardial space, and, very rarely, viral pericard itis. Pathology The pericardium becomes a dense mass of \201 brous tissue that may be cal ci \201 ed. This results in encasement of the heart within a nonexpansile pericardium. Clinical features Symptoms include dyspnea, edema, and abdominal swelling due to ascites an d hepatomegaly. Signs include small volume pulse and pulsus paradoxus. JVP is always elevated, usually very high with prominent x and y descents. A diastolic knock is usually felt at the left sterna l border due to the sudden halting of the ventricles during diastolic \201 lling. Apex be at may be impalpable. Heart sounds are usually soft, and an early third sound coincident wi th the dia- stolic knock is usually heard. In the pulmonary area there is sudden instantaneous widened splitting of the second sound t hat occurs following the \201 rst heartbeat of inspiration (Vogelpoel \200\223Beck sign). The liver is commonly grossly enlarged, ascites is marked, and peripheral edema is present . Investigations \200 ECG (i.e., generalized low-voltage QRS complexes, and widespread \202 attening and invers ion of T waves). Atrial \201 brillation is common in the chronic form. CXR: Normal or nea r-normal cardiac size in the presence of marked venous distension or heart failure is sugge stive of constrictive pericarditis or restrictive cardiomyopathy. Pericardial calci \201 c ation is diagnostic; its incidence varies from 5% to 70% of cases in different series. Echo cardiography: mitral \201 lling pattern on Doppler, with respiratory variation of >25% ov er the atrioventricular valves. CT and MRI scans Cardiac catheterization demonstrates eleva tion and equalization of \201 lling pressures. In the typical case, the difference in \201 lling pressures between the right ventricle and left ventricle does not exceed 6 mmHg. The right atrial waveform shows rapid x and y descents, and the mean pressure does not dec rease normally with inspiration or may show Kussmaul \200\231s sign. is abnormal in virtually every case, but changes are nonspeci \201 c Pericardial thickening may be present. A restrictive \200 demonstrate pericardial thickening (>5 mm). Management Constrictive pericarditis is treated by surgical removal of the \201 brous con strictive tissue (pericardiectomy). CHAPTER 7 Diseases of the myocardium and pericardium 312 Effusive \200\223constrictive pericarditis Etiology This is characteristically encountered in active TB pericarditis where there are signs of both pericardial effusion or tamponade a nd constriction. It may also occur in neoplastic, radiation, and septic pericarditis. Clinical presentation Symptoms are usually those of constriction. Examination reveals pulsu s paradoxus and raised JVP with prominent x and y descents. The CXR shows an enlarged cardi ac silhouette like that of pericardial effusion. Diagnosis The clue to diagnosis is persistent signs of constriction following adequate peri cardial drainage. Management Treatment is of the underlying cause and pericardial drainage if indicated. Peri cardiectomy is indicated should hemodynamics not improve. Calci \201 c pericarditis without constriction This condition is usually discovered during routine radiological examin- ation, which demonstrates pericardial calci \201 cation. The re are no symp- toms and signs of constriction and the cause is usually unknown. TUBERCULOUS PERICARDITIS 313 Viral pericarditis Clinical presentation Most patients present with a history of upper resp iratory tract infection within the preceding 3 weeks. The viruses most frequently responsib le include coxsackie B, echo, mumps, in \202 uenza, and varicella. Pericardial pain, fever, and malaise are typical. The physical examination reveals a perica rdial friction rub and/or characteristic changes of acute pericarditis on ECG. Course In most patients the illness resolves spontaneously in 1 \200\2232 weeks. In some pa tients, the illness recurs on at least one occasion in the next few weeks or months, and in 20% of patients there are multiple recurrences in the ensuing months or years (benign rela psing pericarditis). Diagnosis It is important to search for an underlying disease (see Table 7.3) that may requ ire speci \201 c therapy. In most cases of suspected viral pericarditis, spe- cial studies for etiologic agents are not necessary because of the low diag- nostic yield of viral stud ies and lack of speci \201 c therapy for viral disease. Management \200 NSAIDs are effective in most patients with viral pericarditis. There are no data from rando mized controlled trials to guide treatment for relapsing patients who do not respond to NSA IDs. Corticosteroids (prednisone 1 \200\2231.5 mg/kg for at least 1 month) provide symptoma tic relief in most patients; symptoms recur in many patients when the prednisone dose is re duced. Colchicine (2 mg/day for 1 \200\2232 days, followed by 1 mg/day) may be effective wh en NSAIDs and corticosteroids fail to prevent relapses. If patients do not respond adequate ly, azathioprine (75 \200\223100 mg/day) or cyclophosphamide may be added. Pericardiectomy is indicated only in frequent and highly symptomatic recurrences resistant to medical thera py. However, postpericardiectomy recurrences may occur, possibly due to incomplete resectio n of the pericardium. Tuberculous pericarditis Tuberculous (TB) pericarditis is uncommon in the developed world, but is very common in developing countries. The incidence appears to be increasing in sub-S aharan Africa in parallel with the HIV/AIDS epidemic. The disease presents in three forms: constrictive pericarditis, effusive- constrictive pericarditis, and pericardial effusion. CHAPTER 7 Diseases of the myocardium and pericardium 314 TB constrictive pericarditis Clinical presentation Most cases have an active in \202 ammat ory \201 brocaseous tissue surrounding the heart and involve visceral and parietal perica rdium. The clinical presentation is highly variable. Patients range from being asymptomatic to sho wing severe signs and symptoms of constriction. The diagnosis is often missed on cursory cl inical and echocardiographic examination. It is uncommon to \201 nd concomitant pulmonary TB, and pericardial cal- ci \201 cation is found in <5% of cases. Treatment The initial management of patients with noncalci \201 c TB constrictive peri- ca rditis is with anti-TB therapy. Since the process is an active \201 bro-caseous condition , resolution of constriction occurs in 15% \200\22320% of patients with medical management over 3 \200\2234 months. Pericardiectomy is recommended if no improvement has occurred after 6 weeks of anti-TB trea tment or unsatisfactory improvement after several months of treatment. By contrast, calci \201 c TB pericarditis is treated by early pericardiectomy and anti-TB chemotherapy. TB effusive \200\223constrictive pericarditis Features This mixed form is a common presenta tion of TB pericarditis. There is increased pericardial pressure due to effusion in the pre sence of visceral constriction. The echocardiogram shows porridge-like exudation with loculation of the \202 uid. Treatment Treatment of this condition is by standard four-drug therapy. The role of adjuvan t steroids is not known. The mortality is about 10%, and about 30% of cases will come to peri- cardiectomy over 2 years of follow-up. TB PERICARDIAL EFFUSION 315 TB pericardial effusion Pathology Pericardial effusion is the most common mode of presentat ion with or without tamponade. The effusion is bloodstained in over 95% of cases, and may e ven resemble venous blood. The absence of parenchymatous lung disease and the presence of hilar lymphadenopathy in man y of these patients suggest a direct spread from a TB hilar node to the pericardium. Clinical presentation Systemic symptoms are variable. Typical pericardial pain is uncommon and the classic ECG features of pericarditis are not seen. CXR shows an enlarged globular heart, small pleural effusions on one or both sides, and evi dence of pulmonary tuberculosis in only 30% of cases. The echocardiogram shows features of pericardial effusion, typically asso- ciated with soft tissue density masses, thickening of the visceral pericar- dium, and \201 brinous strands. Diagnosis A de \201 nite diagnosis of TB pericardial effusion is based on demonstration of tubercle bacilli in the pericardial \202 uid or on histological section of the pericardi um. Pericardial \202 uid should be sent for microscopy (to identify acid-fast bacilli [AF B]) and culture of tubercle bacilli. The chances of a positive culture are improved by beds ide inoculation of the \202 uid into dou- ble-strength Kirschner culture medium. Pericard ial biopsy and drainage offer the advantage of a histological diagnosis and early complete drain- age of the pericardium. This can be performed via the subxiphisternal approach under local anesthesia. A probable diagnosis is made when there is proof of TB elsewhere in a patient with unexplai ned pericardial effusion. Palpation in the supracla- vicular fossa will frequently reveal e nlarged lymph nodes, which should be biopsied. AFB-positive sputum will only be found in ab out 10% of cases. Tuberculin skin testing is of little value in endemic and nonendemic area s. It is not known whether the enzyme-linked immunospot (ELISPOT) test that detects T cells speci \201 c for M tuberculosis antigen will perform better in TB pericarditis than the t uberculin skin test. Several tests have been developed for the rapid diagnosis of TB in the pericardial \202 u id. Polymerase chain reaction (PCR) can identify DNA of M. tuberculosis rapidly from only 1 L of pericardial \202 uid (sensitivity 75%, speci \201 city 100%). An adenosine deami nase (ADA) level >40 U/L has a sen- sitivity of 83% and a speci \201 city of 78%. A high i nterferon-G level is also a highly sensitive (92%) and speci \201 c (100%) marker of peric ardial TB. CHAPTER 7 Diseases of the myocardium and pericardium 316 Treatment Treatment is by means of standard four-drug anti-TB chemotherapy for 6 months. Ov erall mortality is about 8%. Repeat pericardiocentesis is required in about 15% of patients, and dur- ing a 2-year follo w-up period, about 10% of patients will require surgical pericardiectomy. While some clinicians favor the addition of steroids to conventional therapy, their role in improving survival is not clearly established, particu- larly in the context of HIV infect ion. UREMIC PERICARDITIS 317 Uremic pericarditis Pathology Uremia produces a \201 brinous, often hemorrhagic in \202 ammation that may lead to tamponade, and constriction in some cases. Management Symptomatic pericarditis that occurs before the initiation of dialysis will resp ond to repeated peritoneal or hemodialysis. Heparin-free hemodialy- sis should be used to a void hemopericardium. Many patients who develop pericardial effusion while on dialysis (dial- ysis-associated per icarditis) will respond to intensi \201 cation of the dialysis regime. Those who do not re spond will require drainage by either subxy- phoid pericardiotomy or pericardial window. Pericardiocentesis is associated with a high risk of intrapericardial hemorrhage. CHAPTER 7 Diseases of the myocardium and pericardium 318 Neoplastic pericardial disease Etiology This condition is usually secondary to malignancy o f the bronchus, breast, or kidney. Primary tumors of the pericardium are rare and usually r esult from mesothelioma following asbestos exposure. Diagnosis Metastases may produce a large hemorrhagic effusion or severe constric- tion when tumor encases the heart. Malignant cells may be found in 85% of cases of aspirated pericar dial \202 uid. Tumor markers such as carcinoembryonic antigen (CEA), A-fetoprotein (AFP), and carbohydrate antigens (e.g., CA 125) have been used in the diagnosis of malignant effusion. The differentiation of TB and neoplastic effusion is virtually absolute with low ADA and high CEA levels. Management This depends on the type and stage of malignancy, condition of the patient, and presence of cardiac compression. Pericardiocentesis is effective in relieving neoplastic ca rdiac tamponade in the vast majority of cases. Commonly, neoplastic pericardial disease is a preterminal event. Palliation for recurrent t amponade can be obtained by subxyphoid surgi- cal pericardiotomy (which can be performed un der local anesthesia) or by percutaneous balloon pericardiotomy to create a pleuropericardi al win- dow to allow \202 uid drainage into the pleural space in patients with large mali gnant pericardial effusions and a limited life expectancy. Partial pericardiectomy (pericardial window) through a left thoracot- omy should be reserve d for patients who have a better prognosis and are likely to respond to chemotherapy or rad iation. Intrapericardial instillation of cytostatic/sclerosing agents has also been used to prevent recurrences of large pericardial effusion. Tetracycline as a sclerosing agent controls mal ignant pericardial effusion in up to 85% of cases, but side effects, such as fever, chest p ain, and atrial arrhythmias, and constriction in long-term survivors, are common. MYXEDEMATOUS EFFUSION 319 Myxedematous effusion Clinical presentation Effusion with high protein content is common in untreated myxedema. Myxedema must always be excluded in a patient with chronic, asymptom- atic, pericardial effusion associated with bradycardia, low voltage of the QRS complexes, a nd a history of radiation-induced thyroid dysfunction. Cardiac tamponade does not occur. Diagnosis This is based on serum levels of thyroxine and thyroid-stimulating hormone. Treatment Treatment with thyroid hormone causes resolution of the pericardial effusion. CHAPTER 7 Diseases of the myocardium and pericardium 320 Nontuberculous bacterial (purulent) pericarditis Predisposing conditions These include immu nosuppression (e.g., immunosuppressive drugs, lymph- oma, or HIV/AIDS), pre-existing perica rdial effusion, cardiac surgery, and chest trauma. In children, pharyngitis, pneumonia, otitis media, endocarditis, and arth- ritis are found. Precipitating factors These include cardiac surgery, pericardial aspiration, extension of a ortic root endocarditis into the pericardial sac, or hematogenous spread from a septic focu s such as osteomyelitis or pneumonia. Causative organisms In adults these are Staphylococcus aureus, gram-negative bacilli, and a naer- obes. In children they are Haemophilus, Staphylococcus aureus, and N. meningitides. Clinical presentation This is rare in adults. It presents as an acute, fulminating infectio us illness and is fatal if not treated. The most important reason for this poor outlook is failure to suspect the condition in debilitated patients with overwhelm- ing systemic infec tion. Diagnosis Pericardiocentesis shows purulent \202 uid, with high white cell count, low glu cose, positive Gram stain for the organism, and positive pericardial \202 uid and blood c ulture. Treatment Complete pericardial drainage is preferable through a subxyphoid pericar- diotomy , and systemic antibiotic therapy should be given. The mortality rate is 40% even with appr opriate management. DRUG- OR TOXIN-INDUCED PERICARDITIS 321 Radiation pericarditis Clinical features This condition usually follows treatment for lymph oma and breast cancer. Incidence of pericarditis depends on how much of the heart is includ ed in the \201 eld of radiation, the dose, the duration of treatment, and age of the pati ent. Radiation is a common cause of effusive \200\223constrictive peri- carditis and also m ay result in myocarditis and premature coronary atherosclerosis. Clinical evidence of pericarditis may occur during, or shortly after treat- ment, but is mo re usually delayed for approximately 1 year. The presenta- tion is one of pericarditis with some effusion. In about 50% of cases there is evidence of tamponade, which requires draina ge. Management Up to 20% of patients will require pericardiectomy because of severe constrictio n. The operative mortality is high (21%) and the postoperative 5-year survival is very low (1%), mostly due to underlying myocardial \201 bro- sis and severe thickening of the visc eral pericardium. Drug- or toxin-induced pericarditis Etiology Pericardial reactions to drugs and toxins are rare. Pericarditis may develop as part of the lupus erythematosus-like syndrome (hydralazin e, INH, pro- cainamide, methyldopa, reserpine), hypersensitivity reaction (cromolyn sodium, penicillins, streptomycin), serum sickness, or envenomation (scor- pion \201 sh sting). Management This consists of removal of the causative agent and symptomatic treatment. CHAPTER 7 Diseases of the myocardium and pericardium 322 Postcardiotomy syndrome Clinical presentation The syndrome occurs in approximately 30% of p atients undergoing any form of cardiac surgery. It is more common in patients receiving ami noc- aproic acid during the operation. After a latent period of 2 \200\2233 weeks, there is fever, pericarditis, pleuritis, and pn eumonitis with a marked tendency to relapse. \200\234Dry \200\235 pericarditis or pericard itis with effusion may occur. The illness is self-limited and varies in intensity and durat ion, but usually lasts only 2 \200\2234 weeks. Treatment Give NSAIDs and analgesics such as aspirin or colchicine. Steroids (oral or intrapericardial) induce prompt relief of symptom s but should be reserved for severely affected patients, since relapse may occur when they are dis- continued and dependency may result. Anticoagulants should be avoided because of risk of hemopericardium. Postinfarction pericarditis Clinical presentation Two forms of postinfarction pericarditis may be distinguished: 1. The usual early form of pericarditis occurs within a week in at le ast 20% of patients with transmural MI. It is a result of pericardial irritation by adjacen t infarcted myocardium. 2. Less commonly, a delayed autoimmune reaction may produce pericardi- tis 2 weeks to a few months after the infarct (Dressler syndrome). The delayed form behaves very similarly to t he postcardiotomy syndrome. Treatment Ibuprofen is said to increase coronary \202 ow, and is the NSAID of choice. Asp irin, up to 650 mg every 4 hours for 2 \200\2235 days, has also been used suc- cessfully. O ccasionally steroids may be required for relapse, but should be avoided as they may delay m yocardial healing. Anticoagulants should be avoided because of the risk of hemopericardium. Rheumatic fever This is an important cause of pericarditis worldwide, almost invariably ass ociated with severe pancarditis and valvular involvement. Rheumatic pericardial effusion is usually clear, straw-colored, and sterile. Cardiac tamponade is rare and the \202 uid us ually reabsorbs rapidly in response to salicylates or steroid therapy. Chronic constrictive pericarditis is never rheumatic in origin but adherent pericardium with \202 ecks of cal ci \201 cation is not uncommon. FUNGAL PERICARDITIS 323 Autoimmune pericarditis Pericarditis may be the presenting feature of systemic lupus erythe mato- sus (SLE) or complicate scleroderma and polyarteritis nodosa. Dry or effu- sive peric arditis may occur; and it is important to exclude these disorders in patients presenting wi th idiopathic benign pericarditis. Generally, other signs and symptoms of these collagen va scular diseases will be present. Granulomatous pericarditis is a complication of rheumatoid arthritis and ankylosing spondyl itis, leading to effusion and occasionally constriction. Aortitis and aortic insuf \201 ci ency may be associated and occasionally there is invasion of the interventricular septum, p roducing heart block. Traumatic pericarditis This produces two forms of pericarditis. One is a direct result of t rauma with hemorrhage into the pericardial cavity and formation of hemoperi- cardium. If as sociated myocardial or valvular injury is absent, complete recovery is the rule, although c hronic constriction may develop. The second type is a form of recurrent pericarditis associated with the postcardiotomy and post-MI syndromes, which may also result in chronic constriction. Fungal pericarditis Clinical presentation Fungal pericarditis occurs as a rare opportunistic infection in immuno- compromised patient s (e.g., immunosuppressive therapy, HIV/AIDS). It is caused by endemic fungi (Histoplasma, Coccidioides), nonendemic oppor- tunistic fungi (candida, Aspergillus, Blastomyces), and se mi-fungi (Norcadia, Actinomycosis). Diagnosis Fungal pericarditis may resemble TB pericarditis. Fungal staining and cul- ture o f aspirated pericardial \202 uid and pericardial biopsy are necessary to make the distinc tion. Treatment Antifungal treatment with \202 uconazole, ketoconazole, itraconazole, ampho- te ricin B, or amphotericin B lipid complex is indicated. NSAIDs are used for symptomatic reli ef. Sulfonamides are the drug of choice for Norcadiosis, and a combination of three antibiotics including penicillin should be given for Actinomycosis. CHAPTER 7 Diseases of the myocardium and pericardium 324 Amoebic pericarditis Pathology Amoebiasis (caused by Entamoeba histolytica) occurs mainly i n endemic areas and in travelers from them (in whom the syndrome may appear years later). P ericardial complications are rare, but when they do occur, they carry a high mortality, esp ecially with delayed or missed diagnosis. The usual cause of amoebic pericarditis is extension from an amoebic liver abscess in the l eft lobe. Rarely, spread may also occur from the right lobe or disease may reach the perica rdium from an amoebic lung abscess. Clinical presentation There are two modes of presentation: 1. Hepatic presentation: an absc ess near the pericardium, which has not yet ruptured, can cause a pericardial friction rub, a nonpurulent pericar- dial effusion, and ECG/CXR signs of pericarditis. 2. Cardiac presentation: perforation of a liver abscess into the pericar- dium results in a purulent pericarditis. The onset can be acute, with shock and death within a short time, o r the onset may be gradual with signs of tamponade as a result of the pericardial \202 ui d. Diagnosis Diagnosis is dif \201 cult but should be suspected if, in a patient with a purul ent pericarditis and signs of cardiac failure, tenderness of the liver in the epi- gastrium is much more marked than the rest of the palpable liver. A high and immobile left hemidiap hragm detected by \202 uoroscopy or CT scan is also suggestive. De \201 nite diagnosis is by pericardiocentesis (which typically shows brown- ish \202 u id; the pus may simulate anchovy sauce) and serological testing for antibodies resulting fr om invasive amoebiasis ( \202 uorescent antibody test or amoebic enzyme immunoassay or amo ebic gel diffusion test). Treatment With hepatic presentation the pericardial disease resolves with successful treatm ent of the liver abscess. With cardiac presentation, treat with pericardial drainage and metro- nidazole. Constrictive pericarditis is an occasional complication. PERICARDITIS VS. MYOCARDIAL DISEASE 325 Constrictive pericarditis vs. restrictive myocardial disease It is dif \201 cult to separa te constrictive pericarditis from restrictive myocar- dial disease. The distinction is vita l, because pericardiectomy is one of the most satisfying operations in terms of cure. Point s of differentiation are as presented in Table 7.4. An exploratory thoracotomy is justi \201 ed if all of these tests fail to make a distinction between the two entities. Table 7.4 Comparison of constrictive pericarditis and restrictive myocardial disease Constriction Restriction \200 and TR \200 myocardial disease Murmur of MR Clinical History of prior pericardial disease, prior pericardial trauma, prior cardiac surgery Known in \201 ltrative ECG Normal Left axis deviation, LVH, Q waves, LBBB CXR Pericardial calci \201 cation No pericardial calci \201 cation \200 LVH \200 \200\234Speckled \200\235 appearance o f myocardium \200 \200 <8 cm/sec \200 \200 Echocardiogram Septal bounce Tissue Doppler Ea Normal LV systolic function (cid:116)8 cm/sec LV dysfunction Tissue Doppler Ea CT or MRI Pericardium (cid:116)3 mm Normal pericardium Endomyocardial biopsy Normal myocardium Evidence of in \201 latrative disease (i.e., amyloid, hemochromatosis) \200 \200 diffe rence between LVEDP and RVEDP \200 PAS >50 mmHg \200 (RVSP increases with inspiration, LVSP decreases with inspiration) \200 Dip and plateau >6 mmHg Right heart catheterization (RHC) Dip and plateau Ventricular interdependence PAS <50 mmHg CHAPTER 7 Diseases of the myocardium and pericardium 326 Pericardial \202 uid analysis Analyzes of pericardial effusion can establish the diagnosi s of viral, bacter- ial, TB, fungal, amoebic, and malignant pericarditis. The appearance of the pericardial \202 uid should be noted. The tests should be ordered according to the c linical presentation of the patient. However, the following routine samples are useful: Biochemistry sample for protein and LDH estimation to distinguish between and exudate and a transudate Microbiology specimen for mi croscopy, culture, and sensitivity testing Cytology specimen \200 The following tests are requested for the diagnosis of the speci \201 c forms of pericardi tis: \200 \200 Viral pericarditis \200\224PCR for cardiotropic viruses Purulent pericarditis \200\224Gram stain, at least 3 blood cultures of pericardial \202 uid for aerobes and anaerobes and bl ood cultures TB pericarditis \200\224staining for AFB; bedside inoculation of pericardial \202 uid into double-strength Kirschner transportation medium and culture; ADA level Funga l pericarditis \200\224microscopy and culture Amoebic pericarditis \200\224brownish pericar dial \202 uid with anchovy sauce appearance of pus Malignant effusion \200\224cytology an d tumor markers (e.g., CEA) \200 Pericardiocentesis See Chapter 1. FURTHER READING 327 Further reading Akashi YJ, Goldstein DS, Barbaro G, Ueyama T (2008). Takotsubo cardiomyopathy: a new form o f acute, reversible heart failure. Circulation 118(25):2754 \200\2232762. Dote K, Sato H, Ishihara M (1991). Myocardial stunning due to simultanous coronary spasms: a review of 5 cases. J Cardiol 21(2):203 \200\223214. Kuowski V, Kaiser A, von Hof K, et al. (2007). Apical and midventricular transient left ven tricu- lar dysfunction syndrome (tako-tsubo cardiomyopathy): frequency, mechanisms, and pro gnosis. Chest 132(3):809 \200\223816. Nishimura RA, Holmes DR Jr (2004). Clinical practice. Hypertrophic obstructive cardiomyopat hy. N Engl J Med 350(13):1320 \200\2231327. Watkins H, McKenna WJ (2005). The prognostic impact of septal myectomy in obstructive hyper - trophic cardiomyopathy. J Am Coll Cardiol 46:477. Wigle ED, Rakowski H, Kimball BP, Williams WG (1995). Hypertrophic cardiomyopathy. Clinical spectrum and treatment. Circulation 92(7):1680 \200\2231692. This page intentionally left blank 329 Chapter 8 Congenital heart disease Introduction 330 Assessment of patients with CHD 332 Speci \201 c signs in patients with C HD 336 Surgical operations for CHD 337 Percutaneous transcatheter interventions for CHD 341 Speci \201 c management issues 343 Atrial septal defect 346 Ventricular septal defect 346 Atrioventricular septal/canal defect (endocardial cushion defect) 346 Pulmonary stenosis 347 Left ventricular out \202 ow tract (LVOT) obstruction 347 Coarctati on of the aorta 347 Anomalous pulmonary venous drainage 348 Transposition of the great arte ries 348 Tetralogy of Fallot 349 Fontan patients 349 Congenitally corrected transposition o f the great arteries 350 Ebstein \200\231s anomaly of the tricuspid valve 350 Extracardiac complications 351 CHAPTER 8 Congenital heart disease 330 Introduction Congenital heart disease (CHD) is one of the most common congenital defects, o ccurring in approximately 0.6% \200\2230.8% of newborns. Advances in therapy have led to a dramatic improvement in outcome such that over 85% of infants, even with complex CHD, are e xpected to reach adoles- cence and early adulthood. As a result of the success of pediatric cardiology and surgery, the number of adults with C HD is greater than the number of pediatric cases. In addition, there are patients with stru ctural or valvular CHD who may present late during adulthood. As of 2000 there were roughly 485,000 American adults with moderate to very complex CHD and 300,000 with simple forms of CHD.1 Many of those with CHD have had palliative or reparativ e rather than corrective surgery and will require further cardiac operations and procedures . Role of specialist CHD centers \200 \200 \200 \200 