A Review of Pericardial Diseases: Clinical, ECG and Hemodynamic Features and Management

REVIEW

SHAM IK AIKAT, MD SASAN GHAFFARI, MD Department of Cardiology, Cleveland Clinic Department of Cardiology, Cleveland Clinic

A review of pericardial diseases: Clinical, ECG and hemodynamic features and management

ABSTRACT ECAUSE PERICARDIAL DISEASES are common and often misdiagnosed, physi- Pericardial diseases are common, have multiple causes, and cians need to he familiar with their presenta- are often misdiagnosed. Physicians need to recognize the tions and distinguishing features. characteristic and distinguishing features of the three most This article reviews the clinical features, important pericardial conditions: acute pericarditis, cardiac diagnosis, and management of acute pericardi- tamponade, and constrictive pericarditis. In these tis, cardiac tamponade, and constrictive peri- conditions, proper diagnosis and appropriate management carditis. can significantly reduce morbidity and mortality. • THE PERICARDIUM

KEY POINTS The pericardium envelopes the heart, extend- ing on to the adventitia of the great vessels. It Acute pericarditis is an important part of the differential is 1 to 2 mm thick and consists of an outer and diagnosis of chest pain syndromes and needs to be an inner layer. The tough, fibrous outer layer is distinguished from acute myocardial infarction both called the parietal pericardium, and the serous clinically and electrocardiographically. inner layer is called the visceral pericardium. The parietal and visceral pericardium are sep- Suspect pericardial tamponade in any patient with acute arated by fluid: 15 to 50 mL of an ultrafiltrate dyspnea, especially in the presence of a pericardial rub, of plasma that acts as a lubricant.1-2 elevated jugular venous pressure, and hypotension. The pericardium limits excessive cardiac movement and acute cardiac distension. By providing a nondistendible outer layer, it gen- Two-dimensional echocardiography with Doppler is erates a negative intrapericardial pressure dur- essential for a rapid and accurate confirmation of ing inspiration, which contributes to chamber pericardial tamponade and for safe and effective filling and establishes ventricular interdepen- therapeutic pericardiocentesis. dence in maintaining parity between right and left heart filling and cardiac output. In addi- Constrictive pericarditis is often a diagnostic challenge and tion, the pericardium acts as a barrier to infec- requires a high index of clinical suspicion. Elevated jugular tion and the extension of cancer. venous pressures distinguish it from primary liver disease, but echocardiography, magnetic resonance imaging, • ACUTE PERICARDITIS computed tomography, hemodynamic studies, and even endomyocardial biopsy are often needed. Acute pericarditis is common, but its true incidence is not precisely known because it often goes unrecognized and has a self-limiting course. In different autopsy series, pericarditis

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TABLE 1 Idiopathic. In most cases of acute pericarditis the cause is unknown. A Spanish Causes of acute pericarditis study6 followed 100 consecutive patients Idiopathic admitted to a university hospital because of acute pericarditis, and the investigators were Infection able to determine a cause in only 22%. Viral: coxsackievirus B (most common viral cause), Viral infections. Patients with idiopathic coxsackievirus A, echovirus, human immunodeficiency virus pericarditis are often thought to have been Bacterial: Staphylococcus aureus, Pneumococcus species, infected by a cardiotropic virus such as cox- Mycoplasma Mycobacterial: tuberculosis (most common cause worldwide) sackievirus B. Other known viral pathogens are Fungal: Histoplasma, Aspergillus echovirus 8, mumps, influenza, and Epstein- Protozoal: Toxoplasma Barr virus. However, investigations to prove a Other: Rickettsia, anaerobes, parasites viral cause, even in patients with a typical viral prodrome, have an extremely low yield.7 Malignancy Secondary neoplasms (common): breast, lung, lymphoma, Neoplastic pericardial involvement is leukemia, melanoma seen at autopsy in up to 10% of patients with Primary neoplasms (rare): pericardial mesothelioma, a known malignancy.7 The most common angiosarcoma malignancies that affect the pericardium are lung and breast cancers, melanomas, lym- Connective tissue disease phomas, and leukemias. Primary pericardial Systemic lupus erythematosus, rheumatoid arthritis, sarcoidosis, scleroderma tumors are rare and usually take the form of benign pericardial cysts.5-7'8 Myocardial infarction Radiation therapy Symptoms and signs of acute pericarditis Chest pain is the cardinal symptom of Cardiac surgery acute pericarditis. The pain: Uremia • Is often located retrosternally or precor- Myxedema dially • Is sharp, knifelike, and pleuritic Aortic dissection • Worsens with inspiration and in the Drugs (as part of drug-induced systemic lupus erythematosus) supine position Hydralazine • Is relieved on sitting and leaning forward Methyldopa • Sometimes alters or recurs with each beat Procainamide of the heart Others: cromolyn sodium, doxorubicin, penicillin • Can radiate to the left shoulder, the arm, neck, back, or epigastric region. Radiation ADAPTED FROM REFERENCES 1,5,7,8,24 of the pain to the trapezius ridge is an uncommon but specific sign of acute pericarditis.5 was detected in 2% to 6% of the sample Dyspnea is the other prominent symp- groups. Acute pericarditis is estimated to be tom, though not always present. clinically present in 1 of every 1,000 hospital- Other symptoms may include fever, ized patients.4 cough, hoarseness, nausea, vomiting, palpita- Acute pericarditis runs a time course of tions, dizziness, and lightheadedness. Certain less than 6 weeks. If pericarditis lasts 6 weeks symptoms depend on the cause of the peri- to 6 months, it is termed "subacute."4 It is con- carditis: eg, flulike symptoms (fever, myalgias) sidered chronic when it has been present 6 in viral pericarditis or a fulminant and rapid months or longer.4 course with prominent systemic septic features in purulent bacterial pericarditis. Chronic Causes of acute pericarditis cough, history of weight loss, night sweats, and Inflammation of the pericardium has a variety generalized malaise may represent tuberculous of causes (TABLE 1). pericarditis.

904 CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 67 • NUMBER 1 2 DECEMBER 2000 Downloaded from www.ccjm.org on September 29, 2021. For personal use only. All other uses require permission. Acute pericarditis progresses through stages

Stage 1 begins with the onset of chest pain, and lasts several days. Note the concave upward ST elevations seen in the precordial and limb leads. The T waves remain upright in those leads with ST-segment elevation.

Stage 2. The ST segments return to the baseline, T waves become flat, and the PR segment may become depressed.

II aVL ~V2 I I 1 V5 Stage 3. T-wave inversion may be seen. This may persist for weeks, or even indefinitely.

Stage 4. Normalization.

FIGURE 1. The electrocardiographic stages of acute pericarditis

A friction rub is the pathognomonic sign changes in acute pericarditis (FIGURE I).5>8 of acute pericarditis. This is a scratchy, superfi- Note: the first stage is dominated by ST- cial, "Velcro-like" sound, best heard with the segment elevation, which can also be seen in diaphragm of the stethoscope applied firmly to acute myocardial infarction or early repolar- the left lower sternal border with the patient ization. It is vital to distinguish between acute leaning forward and momentarily holding his or myocardial infarction and acute pericarditis, her breath at end-expiration. The rub is often since thrombolytic therapy can result in hem- evanescent and may be triphasic, having an orrhagic cardiac tamponade in patients with atrial systolic, a ventricular systolic, and a dias- pericarditis. The distinction can be difficult. tolic component.'' It is uncommon for all three Important differentiating features are outlined phases to be present; often only a monophasic in TABLE 2 and FIGURE 2. or biphasic rub may be heard. When an effusion Chest radiography may be normal, but develops, the rub may become subdued or may the cardiac silhouette may be enlarged if even disappear, but occasionally the rub persists enough fluid (at least 200-250 mL) accumu- even when the effusion is large. lates in the pericardial sac.1'9 Echocardiography. A pericardial effusion Diagnostic studies for acute pericarditis may accompany acute pericarditis. The pres- Electrocardiography. Serial electrocar- ence of a significant effusion or accumulation diograms commonly show four stages of of an effusion on serial echocardiographic

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TABLE 1 Electrocardiographic features of acute pericarditis, acute myocardial infarction, and early repolarization

ELECTROCARDIOGRAPHIC ACUTE PERICARDITIS ACUTE MYOCARDIAL EARLY REPOLARIZATION FEATURE INFARCTION

ST-segment concavity Upward Downward Upward Loss of R wave, Absent Present Absent presence of Q wave, reciprocal changes Location of Limb and precordial leads In leads corresponding Precordial leads ST-segment elevation (except Vv aVR) to the infarct territory PR-segment elevation Specific to acute Absent Absent pericarditis, especially when seen in aVR ST-to-T amplitude ratio > 0.25, helps to <0.25 <0.25 inV6 distinguish acute pericarditis from acute myocardial infarction T-wave inversion In leads exhibiting In leads exhibiting ST-segment elevation ST-segment elevation Absent after the ST segment has normalized to baseline Arrhythmias Sinus tachycardia common Any type may occur Do not occur Supraventricular in the absence of underlying tachyarrhythmias, Including myocardial disease atrial fibrillation or flutter

ADAPTED FROM REFERENCES 1,4,7,f

studies supports the diagnosis. Echocardiogra- ered in the differential diagnosis. phy is the most sensitive technique to detect Pericardiocentesis and pericardial biopsy fluid in the pericardial sac, as it can detect as are not routinely indicated for diagnosing sus- little as 15 mL.1 pected acute pericarditis. However, when peri- Computed tomography of the chest may cardiocentesis is performed therapeutically, it be a sensitive method to detect an effusion or may yield diagnostic information. A serous pericardial thickening, especially in those who (nonhemorrhagic, nonpurulent) effusion is do not have a good echocardiographic "win- associated with idiopathic or viral pericarditis. dow" (eg, patients with chronic obstructive A serohematogenous or hematogenous effu- pulmonary disease, thoracic skeletal deformi- sion may indicate an idiopathic, viral, tuber- ties).1 culous, uremic, neoplastic, or posttraumatic Laboratory studies. Nonspecific markers cause.6 of acute inflammation such as C-reactive pro- High adenosine deaminase (ADA) activi- tein and the erythrocyte sedimentation rate ty levels in the effusion may denote a tubercu- are usually elevated. Nonspecific leukocytosis lous process.6 Cytologic study of the fluid and may also be seen. A mild increase in the crea- histopathologic study of the pericardium may tine kinase MB isozyme (CK-MB) may be help determine a specific infective agent or a seen.1-10 If CK-MB is significantly elevated, a neoplastic or connective-tissue disease panmyopericarditis should be strongly consid- process.

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III aVF V3 V6

Acute pericarditis. Note the upward-concavity ST elevations in limb leads I, II, aVF, and aVL and in precordial leads V3, V4, V5 and V6 (blue arrows) and the PR-segment elevation in aVR (green arrow).

Acute myocardial infarction. Note the concavity-downward ST elevation in leads I, aVL, V1( V2, V3, V4, V5, and V6 (blue arrows), indicating a large anterior myocardial infarction.

Early repolarization. Note the elevation of the J point (blue arrows) with pseudoelevation of the ST segment in V4, V5, and V6.

FIGURE 2

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Treatment nerve can cause hoarseness. Rapid accumula- of uncomplicated acute pericarditis tion of an effusion, especially if the pericardial The underlying cause should always be treated sac is diseased, nonsupple, nonyielding, or if known. In addition: fibrotic, may result in the dreaded complica- Nonsteroidal anti-inflammatory drugs tion of cardiac tamponade, pulseless electrical (NSAIDs) relieve symptoms and suppress fur- activity, and death. ther inflammation. Aspirin 650 mg by mouth four times a day, naproxen 250 to 500 mg by • PERICARDIAL TAMPONADE mouth twice a day, or indomethacin 50 to 75 mg by mouth three times a day are all accept- Cardiac tamponade occurs when enough fluid able.7 accumulates within the pericardial sac to com- Corticosteroids are controversial in the press the heart and impair diastolic filling. treatment of simple acute pericarditis. Animal experimentation by means of Prednisone 60 mg by mouth daily for 2 to 3 intrapericardial fluid instillation and con- days followed by a rapid taper can be used in comitant hemodynamic monitoring reveal patients whose condition does not respond to evolving stages of hemodynamic compromise. NSAIDs or who have contraindications to Initially, the cardiac stroke volume falls but NSAIDs.11 Corticosteroids are also of value in the cardiac output is maintained by a com- treating recurrent or relapsing pericarditis.11 pensatory tachycardia. With further instilla- Colchicine has demonstrated efficacy in tion of fluid, the cardiac output starts to acute or recurrent pericarditis in patients with diminish; however, blood pressure is main- familial Mediterranean fever, and it also has tained by increased systemic arterial resis- shown benefit in a few studies in idiopathic tance. At this critical juncture, small increas- recurrent pericarditis.4'12 es in the amount of the effusion result in dra- matic increases in the intrapericardial pressure Complications of acute pericarditis and decreases in cardiac output.4'14 Potential complications of acute pericarditis The blood-pressure response also is greatly Acute include recurrent pericarditis, pericardial effu- influenced by the state of suppleness of the peri- pericarditis is sion, cardiac tamponade, and chronic pericardium, the rate of fluid accumulation, the carditis. state of the myocardial contractility, and the often viral, but Recurrent pericarditis occurs in an esti- intravascular volume.4'14 A rapid accumulation this is often mated 15% to 30% of patients who suffer of effusion in a fibrotic pericardium, combined acute pericarditis. Recurrences can cause with impaired left ventricular function and a difficult to bouts of disabling chest pain and may even be hypovolemic state, can precipitate pericardial confirm complicated by myocarditis, pericardial effu- tamponade even if the effusion is small.14 sions, pericardial tamponade, and sometimes, chronic constrictive pericarditis. Recurrent Symptoms and signs pericarditis and acute pericarditis share com- of pericardial tamponade mon causes; however, recurrences are more Acute dyspnea is the most common common when the pericarditis is due to a con- symptom of pericardial tamponade. nective-tissue disease, radiation therapy, or Orthopnea, precordial pain, and diminished neoplastic pericardial involvement.5 urine output are other symptoms. The condi- Pericardial effusion is a nonspecific tion should be suspected in any patient with response of the pericardium to any insult, and sudden, unexplained dyspnea, especially in it may develop slowly and silently.13 Effusions the presence of a pericardial rub, elevated often resolve spontaneously. jugular venous pulses, or hypotension. Large effusions of up to 2 L of fluid can The three cardinal clinical signs in tam- cause symptoms related to compression of ponade, as described by Beck in 1935, are: mediastinal structures. Pressure on the • Hypotension bronchi can cause dyspnea and cough, com- • Raised jugular venous pressure pression of the esophagus can cause dysphagia, • Muffled heart sounds.1 and compression of the left recurrent laryngeal The triad is typical of acute tamponade caused

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FIGURE 3. Electrical alternans in pericardial tamponade due to the swinging of the heart within the pericardial space.

FROM LONGO MJ, JAFFE CC. IMAGES IN CLINICAL MEDICINE.ELECTRICAL ALTERNANS N ENGL J MED 1999; 341:2060, USED WITH PERMISSION

by intrapericardial hemorrhage following a expiration by sphygmomanometry is usually ruptured ventricular aneurysm or a pericardial considered normal. However, sphygmo- extension of an aortic dissection. manometry can be inaccurate: a 10-mm Hg Other important signs are tachycardia, decrease by sphygmomanometry can corre- tachypnea, and pulsus paradoxus. spond to a 25-mm Hg decrease by intra-arter- Pulsus paradoxus is an exaggeration of ial blood pressure measurement. Therefore, in the normal decrease in left ventricular stroke an appropriate clinical setting, a 10-mm Hg 2-D Doppler volume and blood pressure that occurs during sphygmomanometric difference should be confirms the inspiration. Patients may not exhibit this sign considered significant.7 if they have left ventricular dysfunction, an diagnosis of atrial septal defect, severe aortic regurgitation, Diagnostic studies pericardial or a pulmonary embolus. Conversely, those for pericardial tamponade with obstructive airway disease may exhibit Electrocardiography. Common electro- tamponade pulsus paradoxus and an elevated jugular cardiographic findings include tachycardia, venous pressure in the absence of pericardial low-voltage QRS complexes, T-wave flatten- tamponade.7 ing, and electrical alternans (FIGURES).15 Measurement of pulsus paradoxus. With Echocardiography. Two-dimensional the patient respiring normally in a semire- Doppler echocardiography confirms the diag- cumbent position, the blood pressure is man- nosis of pericardial tamponade. The main ually measured. The pressure at which the echocardiographic findings are outlined in Korotkoff sounds first become intermittently TABLE 3. The Doppler findings are based on a audible represents the systolic blood pressure characteristic disproportionate rise in right- during expiration. The cuff is then slowly sided intracardiac pressure and fall in left- deflated, and the pressure at which all the sided intracardiac pressure on inspiration. Korotkoff sounds are audible represents the Swan-Ganz catheterization can confirm systolic pressure during inspiration. The pul- that the systemic venous pressures are elevat- sus paradoxus equals the difference between ed and the right heart diastolic pressures are the expiratory systolic pressure and the inspi- equalized. (Right atrial, pulmonary arterial ratory systolic pressure.7 diastolic, and pulmonary capillary wedge pres- A difference in the systolic blood pressure sures within 5 mm Hg of each other strongly of up to 10 mm Hg between inspiration and suggest pericardial tamponade.)

TABLE 1 coronary arteries (FIGURE 4).4 Emergency pericardiocentesis can be performed "blindly" (ie, Echocardiographic features without echocardiographic guidance), but of pericardial tamponade echocardiographic guidance is preferred. Blind Two-dimensional echocardiography pericardiocentesis is associated with mortality rates of up to 6% and with complication rates An echo-free space throughout the cardiac cycle, of up to 50%. Complications include trauma seen when the pericardial effusion is > 25 mL to the liver, myocardium, coronary vessels, Late diastolic right atrial compression and lungs.16-17 and right ventricular early diastolic collapse If the pericardial effusion is loculated in Dilated Inferior vena cava without inspiratory collapse the posterolateral region and is inaccessible by pericardiocentesis or the cardiac tamponade is Decreased mitral valve excursion and ejection fraction slope recurrent, then the surgical creation of a peri- Swinging of heart (seen when the pericardial effusion Is large) cardial window is indicated. Treat the underlying cause. The underly- Doppler echocardiography ing cause of the pericardial effusion should Abnormal Increase (> 40%) in Inspiratory flow velocity always be sought and appropriately treated to across the tricuspid valve eliminate the risk of recurrence. Abnormal decrease (> 25%) in Inspiratory flow velocity across the mitral valve • CONSTRICTIVE PERICARDITIS

Delayed Inspiratory mitral valve opening In constrictive pericarditis, the pericardial sac with subsequent lengthening of the "Isovolumlc relaxation becomes fibrotic and thickened, resulting in time" (IVRT) and decreased mitral E velocity (reciprocal impaired diastolic filling of the heart. changes on the right side of the heart) Sometimes a tense, chronic pericardial effu- In the pulmonary vein, decreased diastolic forward flow during sion can restrict diastolic filling, a condition inspiration and increased diastolic flow during expiration for which Hancock popularized the term In the hepatic vein, Increased reverse hepatic flow during chronic "effusive constrictive" pericardi- expiration tis.18-19 Constrictive pericarditis leads to a

ADAPTED FROM REFERENCES 4,22,23 congestive state with peripheral interstitial ("third-space") accumulation of fluid. The differential diagnosis of constrictive pericarditis includes congestive heart failure, Treatment of pericardial tamponade restrictive cardiomyopathy, cirrhosis, nephro- Pericardial tamponade is a medical emergency. tic syndrome, and chronic malabsorption Hemodynamically unstable patients should be states. managed in an intensive care unit. Leg elevation, volume expansion with Causes of constrictive pericarditis wide-open intravenous infusion of 0.9% nor- Any of the conditions that cause acute peri- mal saline or colloids, and immediate echocar- carditis may also lead to constrictive pericardi- diographically guided pericardiocentesis are tis. TABLE 4 outlines the etiologies. the most important measures in the manage- Idiopathic. Because acute pericarditis is ment of pericardial tamponade. Note: posi- often self-limiting and clinically missed, it is tive-pressure ventilation can further critically not surprising that idiopathic constrictive diminish cardiac return and decrease cardiac pericarditis without any antecedent peri- output in this setting. carditic episode heads the list of causes of con- Pericardiocentesis. In a patient with strictive pericarditis. The second most impor- pulseless electrical activity and suspected peri- tant cause is constrictive pericarditis following cardial tamponade, emergency bedside peri- acute idiopathic pericarditis. cardiocentesis can be life-saving. The sub- Tuberculosis continues to be a leading xiphoid approach is considered the safest cause of constrictive pericarditis worldwide, because its route is extrapleural and avoids the but its incidence has declined in western

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FIGURE 4. In cardiac tamponade, pericardiocentesis can be lifesaving but poses the risk Emergency of puncturing the ventricle and coronary arteries. Ideally, the procedure is done in a procedure room with echocardiographic monitoring. In an emergency, however, it can be bedside done at the bedside. Insert a 16-gauge or 18-gauge needle into the left xiphocostal angle, pericardio- 30° to 45° to the skin, towards the left shoulder. centesis countries and now accounts for fewer than 6% organs commonly affected: muscle, iiver, can be of all cases.18-19 Tuberculosis accounted for brain, and eye.) life-saving only 0.7% of all patients who underwent surgery for constrictive pericarditis at the Symptoms and signs Mayo Clinic between 1985 and 1995.20 of constrictive pericarditis Cardiac surgery is complicated by con- Constrictive pericarditis tends to develop and strictive pericarditis in fewer than 0.5% of all progress over many months and years. cases; however, with more than 700,000 car- Common symptoms are: diac surgeries performed in the United States • Exertional dyspnea (the most common annually, this is emerging as an important symptom) cause of constrictive pericarditis.20 • Pitting peripheral edema Other important causes of constrictive • Abdominal swelling pericarditis are mediastinal irradiation21 and • Fatigue connective-tissue diseases.20 Uremia is a less • Weight gain common cause. Rarer causes include asbesto- • Abdominal discomfort (often associated sis and mulibrey nanism, an exotic hereditary with nausea and vomiting). multisystem disorder associated with a Less-common symptoms are chest pain, dwarfism seen most commonly in Finns.18'22'23 orthopnea, paroxysmal nocturnal dyspnea, (Mulibrey is a mnemonic acronym for the and right upper-quadrant pain.

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TABLE 1 sounds are muffled. A pericardial "knock" may be heard. This is a higher-pitched early Causes of constrictive pericarditis diastolic variant of the S3, occurring just Idiopathic (no history of recent acute pericarditis) after the second heart sound, best audible Acute idiopathic pericarditis with the diaphragm of the stethoscope. It was Cardiac surgery, trauma first described by Corrigan in 1842 and is thought to be due to the sudden diastolic Radiotherapy restriction of left ventricular filling by the Infection pericardium.23 Tuberculosis Coxsackievirus B Diagnostic studies Bacteria for constrictive pericarditis Fungi (histoplasmosis) Chest radiography. Typically, the car- Parasites (echinococcosis) diopericardial silhouette is normal-sized in Connective tissue disease constrictive pericarditis, and the lung fields Rheumatoid arthritis are clear. The cardiopericardial silhouette may Systemic lupus erythematosus be enlarged in cases of "effusive-constrictive" Uremia pericarditis. Left atrial enlargement, Dressier syndrome "eggshell"-shaped pericardial calcification, Hereditary (mulibrey dwarfism) and pleural effusions may also be seen. Drug-induced lupus Pulmonary edema is reported in 5% to 10% of 18 23 Rare causes cases. ' Asbestosis, Whipple disease, actinomycosis, Lassa fever Electrocardiography. The electrocardio- gram is often abnormal. Left atrial enlarge- ADAPTED FROM REFERENCES 6,17,20,21 ment, P mitrale (abnormally wide, notched P

••••••••••MKSgRflnBBMMBiMBMHHB^HHMHHBMMHMMHMHHHHBHBHMHi waves), nonspecific T-wave changes, and low- voltage QRS complexes may be seen.25 Atrial Increased abdominal girth is often seen arrhythmias such as atrial flutter and fibrilla- on physical examination. First described by tion can occur in up to one third of cases. Pick in 1896, the common presence of ascites Echocardiography. M-mode, two-dimen- and hepatomegaly at presentation of these sional, and Doppler studies provide important patients led to the use of the eponym Pick's supportive information when diagnosing con- disease or pseudocirrhosis in the past.19 strictive pericarditis. M-mode and two-dimen- Elevated jugular venous pressure distin- sional echocardiographic findings include the guishes constrictive pericarditis from primary following: liver disease. Careful bedside interpretation of • Pericardial thickening (which often goes the jugular venous pulsations may reveal a undetected) rapid, often deep Y descent. This constitutes • Premature pulmonary valve opening due the right atrial W (or M) wave form first to elevated right ventricular diastolic described by Bloomfield.18 pressures Kussmaul's sign, first described by • Enlargement of both atria Kussmaul in 1873, is a paradoxical inspiratory • Paradoxical systolic septal movement rise in jugular venous pulsation, or failure of (septal bounce) the jugular venous pulsations to fall with • Diastolic flattening of the left ventricular inspiration.23 The pathophysiologic basis of posterior wall Kussmaul's sign may be that the rigid, nondis- • Dilated inferior vena cava with minimal tendible pericardium fails to transmit respiratory variation intrathoracic pressure changes to the cardiac • Well-preserved left ventricular size and chambers, and the increased inspiratory return function. results in elevated central venous pressures. Doppler studies may reveal respiratory Abnormal heart sounds. The cardiac variation in right and left ventricular filling apical impulse is often absent, and the heart velocities similar to those seen in pericardial

912 CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 67 • NUMBER 1 2 DECEMBER 2000 Downloaded from www.ccjm.org on September 29, 2021. For personal use only. All other uses require permission. Constrictive pericarditis: Hemodynamic signs

FIGURE 5. Ventricular hemodynamics in constrictive pericarditis. Left, the left ventricular and right ventricular pressure curves show early diastolic dips with a sharp transition to a plateau—the "square root" sign. The waves also show diastolic equilibration. Right, right atrial pressure curve shows the "W" wave form.

tamponade. After inspiration, the velocity of ed the value of computed tomography in the flow through the mitral valve and the pul- diagnosis of constrictive pericarditis.18-19 The monary veins decreases; on expiration, the normal pericardium is less than 3 mm thick.21 velocity increases. Across the tricuspid valve In a patient with a hemodynamic pattern com- and the hepatic veins the pattern is reversed: patible with constrictive pericarditis, a firm the flow velocity increases after inspiration diagnosis may be made if the pericardial thick- and decreases on expiration. The deceleration ness is more than 3 mm by computed tomogra- Surgery time of the early filling wave (E) of the mitral phy or magnetic resonance imaging.23 can cure and tricuspid valves shows the following However, this feature may be absent in up to changes: 25% of patients with constrictive pericarditis.19 constrictive • Mitral inflow: inspiratory E is less than Endomyocardial biopsy may be helpful pericarditis, expiratory E (> 25% change); decelera- when a specific cardiomyopathic process is tion time is usually less than 160 ms detected. Nonspecific histopathologic find- but it carries a • Hepatic venous flow: diastolic forward ings favor the diagnosis of constrictive peri- substantial risk flow is markedly decreased on expira- carditis. Sometimes both the pericardium and tion.24 the myocardium may be involved in the dis- with Hemodynamic studies. Patients suspect- ease process, as in sarcoidosis, and it may be deterioration in ed of having constrictive pericarditis should quite difficult to determine the predominant undergo simultaneous right and left heart pathophysiological process.23 NYHA class catheterization. In the right ventricle, pres- Surgical inspection. Despite all the diag- sure tracings show elevated pressures with an nostic studies listed above, constrictive peri- early dip and a plateau ("square-root" wave carditis may be a difficult diagnosis to con- form; FIGURES). Pressure is also elevated in the firm. Surgical inspection is the gold standard left ventricle. In the right atrium, elevated for the diagnosis and, in some patients, may be pressure with prominent X and Y descents the only way to differentiate it from restrictive results in the "M" or "W" wave form classical- card iomyopathy.20 ly described in constrictive pericarditis.19 This waveform may also be seen in mixed restric- Treatment of constrictive pericarditis tive-constrictive diseases. The underlying cause should be sought and Magnetic resonance imaging and comput- treated appropriately: eg, antituberculous ed tomography. In 1982 Isner18-19 demonstrat- chemotherapy for a patient with tuberculous

chronic constrictive pericarditis. In addition: Pericardectomy provides excellent relief Loop diuretics, used judiciously, can pro- of symptoms in those who overcome the early vide symptom relief of peripheral edema and mortality risk. However, in up to one third of ascites. However, because patients with con- patients who survive the surgery, new or recur- strictive pericarditis have impaired biventricu- rent NYHA class III or IV symptoms develop; lar filling, excess diuresis increases the risk for in some, this may be due to incomplete surgi- hypotension and syncope. cal resection, pulmonary interstitial disease, or Corticosteroids can sometimes relieve recurrence of primary or secondary neoplasms subacute cases of constrictive pericarditis.23 after chemotherapy.20 Pericardial resection, first performed by Therefore, patients with stable symptoms Churchill in 1929, can cure constrictive peri- and with NYHA class I or II function can be carditis but carries substantial risk: the overall managed conservatively and surgery can be operative mortality rate is around 6%.6 Late deferred. On the other hand, patients with mortality is estimated at 20% at 5 years and progressive symptoms and late NYHA class II 40% at 10 years.20 function should undergo surgery as soon as The mortality risk is directly proportional possible to reduce excessive early postopera- to the patient's New York Heart Association tive mortality risk from a low-output state.20 (NYHA) functional class, age, and a prior The low-output syndrome is felt to result from history of radiotherapy.18"20 Patients in inadequate decortication and progressive NYHA class I or II have an operative mortal- myocardial atrophy.19 ity rate of approximately 1%, those in class III Pericardial resection offers the hope of a approximately 10%, and those in class IV up cure for patients with constrictive pericarditis, to 45%. especially for younger patients without postra- Patients operated on in the last decade are diation pericarditis or poor NYHA function. older than those in the previous era, and their However, older, more debilitated patients may constrictive pericarditis is often secondary to have a less favorable outcome. Cardiac trans- postmediastinal radiotherapy and cardiac plantation may be a better alternative for surgery.20 some of these unfortunate patients. •

• REFERENCES 13. Corey RG, Campbell PT, Van Trigt P, et al. Etiology of large pericardial effusions. Am J Med 1993; 95:209-213. 14. Fowler NO. Cardiac tamponade: A clinical or an echocardiography 1. Pawsat D, Lee JY, et al. Inflammatory disorders of the heart. Emerg diagnosis? Circulation 1993; 87:1738-1741. Med Clin North Am 1998; 16:665-681. 15. Longo MJ, Jaffe CC. Images in clinical medicine. Electrical alter- 2. Olson TR. A.D.A.M. Student atlas of anatomy. Baltimore: Williams and nans. N Engl J Med 1999; 341:2060. Wilkins, 1996. 16. Tsang TS, Freeman WK, Sinak LJ, Seward JB. 3. Fukuda N, Okl T, luchl TA, et al. Pulmonary and systemic venous flow Echocardlographlcally guided pericardiocentesis. Evolution and patterns assessed by transesophageal Doppler echocardiography in state-of-the-art technique. Mayo Clin Proc 1998; 73:647-652. congenital absence of the pericardium. Am J Cardiol 1995; 75:1286-1288. 17. Seward JB, Callahan JA, et al. 500 Consecutive echo-directed peri- cardiocenteses [abstract], J Am Coll Cardiol 1992; 19(suppl):356A. 4. Lorell BH. Pericarditis. In Braunwald E, editor. Heart Disease, 5th edi- 18. Mehta A, Mehta M, Jain AC. Constrictive pericarditis. Clin Cardiol tion, W.B. Saunders, 1997:1478-1534. 1999; 22:334-344. 5. Marinella MA. Electrocardiographic manifestations and differential 19. Osterberg L, Vagellos R, Atwood JE. Case presentation and diagnosis of acute pericarditis. Am Fam Physician 1998; 57:699-704. review: Constrictive pericarditis. West J Med 1998; 169:232-239. 6. Zayas R, Anguita M, Torres F, et al. Incidence of specific etiology and 20. Lieng LH, Oh JK, Schaff HV, et al. Constrictive pericarditis In the role of methods for specific etiologic diagnosis of primary acute peri- modern era. Circulation 1999; 100:1380-1386. carditis. Am J Cardiol 1995; 75:378-382. 21. Benoff LJ, Schweitzer P. Radiation therapy-induced cardiac injury. 7. Sternbach GL. Pericarditis. Ann Emerg Med 1994; 17:214-220. Am Heart J 1995; 129:1193-1196. 8. Roberts WC. Primary and secondary neoplasms of the heart [editori- 22. Trogrllc S, Gevenois PA, Schroeven M, De Vuyst P. Pericardial effu- al]. Am J Cardiol 1997; 80:671-682. sion associated with asbestos exposure. Thorax 1997; 9. Wang K, Hughes M. Detection of normal pericardium and of pericar- 52:1097-1098. dial effusion in the lateral chest radiograph. Circulation 1996; 93:1254. 23. Fowler NO. Constrictive pericarditis: Its history and current status. 10. Man Y, Oren R, Ben-Chetrit E. Acute pericarditis: etiology, treatment, Clin Cardiol 1995; 18:341-350. and prognosis. A study of 115 patients. Jpn Heart J 1991; 32:315-321. 24. Oh JK, Seward JB, Tajik AJ. The echo manual: from the Mayo 11. Marcolongo R, Russo R, Leveder F, Noventa F, Agostini C. Clinic. Boston: Little, Brown and Company, 1994. Immunosuppressive therapy prevents recurrent pericarditis. J Am Coll Cardiol 1995; 26:1276-1279. 12. Adler Y, Finkelstein Y, Guindo J, et al. Colchicine treatment for recur- ADDRESS: Sasan Ghaffari, MD, Department of Cardiology, Fl 5, The rent pericarditis: A decade of experience. Circulation 1998; Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195; e- 97:2183-2185. mail [email protected].

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