UEU-co logo

MD Consult: Books: Goldman: Cecil Medicine: Chapter 77 – PERICARDIAL DISEASE

Goldman: Cecil Medicine, 23rd ed.

Copyright © 2007 Saunders, An Imprint of Elsevier

Chapter 77 – PERICARDIAL DISEASE

Warren J. Manning

The pericardium is composed of two distinct layers. The fibrous parietal pericardium provides a protective sac around the heart to prevent sudden cardiac dilation and to minimize bulk cardiac motion. The inner, visceral pericardium is intimately related to the surface of the heart. These two layers are normally separated by 10 to 50 mL of clear fluid, an ultrafiltrate of plasma that is produced by the visceral pericardium and functions as a lubricant to minimize frictional forces between the heart and the pericardium. In health, the intrapericardial pressure is slightly negative.

Although congenital total absence of the pericardium is not associated with clinical disease, partial or localized absence of pericardium, specifically around the left atrium, may be associated with focal herniation and subsequent strangulation. This condition, usually diagnosed by thoracic computed tomography (CT) or magnetic resonance imaging (MRI), has been associated with atypical chest pain or sudden death; surgical repair often is recommended when a partial pericardial defect is confirmed. Benign pericardial cysts are rare and often seen as rounded or lobulated structures adjacent to the usual cardiac silhouette on the chest radiograph or adjacent to the right atrium on transthoracic echocardiography ( Chapter 53 ). Thoracic CT and MRI ( Fig. 77-1 ) are useful for the diagnosis of these cysts.

Acquired pericardial disease may have numerous causes, most of which produce responses that are pathophysiologically and clinically similar. These responses most frequently result in acute pericarditis, pericardial effusion, or constrictive pericarditis.

FIGURE 77-1  A, Transverse (axial) magnetic resonance image. Note the anterior pericardial cyst (straight white arrows) and the normal pericardium (curved white arrow). B, Transthoracic echocardiogram from the apical four-chamber view demonstrating a pericardial cyst (Cy) anterior to the right atrium (RA). The left atrium (LA) and descending thoracic aorta (DO) are also seen in this view.  A, (Courtesy of Robert R. Edelman, MD).

   ACUTE PERICARDITIS

Definition

The most common clinical pathologic process involving the pericardium is acute pericarditis. Although multiple causes are possible ( Table 77-1 ), the most common are viral infection and unknown (idiopathic). Classically, this disorder is characterized by chest pain, pericardial friction rub, diffuse electrocardiographic changes, and pericardial effusion, although sometimes neither electrocardiographic changes nor a pericardial effusion is present. The clinical syndrome is often relatively brief (days to weeks) in duration and uncomplicated, although vigilance for progression to tamponade is always prudent.


TABLE 77-1   — 
ETIOLOGY OF PERICARDITIS

INFECTIOUS PERICARDITIS

   Viral (coxsackieviruses A and B, echovirus, mumps, adenovirus, Epstein-Barr, human immunodeficiency virus, influenza)
   Mycobacterium tuberculosis
   Bacterial (Pneumococcus, Streptococcus, Staphylococcus, Legionella)
   Fungal (histoplasmosis, coccidioidomycosis, candidiasis, blastomycosis)
   Other (syphilis, parasites, Q fever)
NONINFECTIOUS PERICARDITIS

   Idiopathic
   Neoplasm
   Metastatic (lung cancer, breast cancer, melanoma, lymphoma)
   Primary (mesothelioma)
   Renal failure
   Trauma
   Irradiation (especially for breast cancer, Hodgkin’s disease)
   Myocardial infarction
   Hypothyroidism
   Aortic dissection with hemopericardium
   Chylopericardium (thoracic duct injury)
   Trauma
   Post pericardiotomy
   Chest wall injury or trauma
   Pneumonia
HYPERSENSITIVITY PERICARDITIS

   Collagen vascular disease (systemic lupus erythematosus, rheumatoid arthritis, scleroderma, acute rheumatic fever,
   Sjögren’s syndrome, Reiter’s syndrome, ankylosing spondylitis)
   Drug induced (procainamide, hydralazine, isoniazid; smallpox vaccine)
   Post myocardial infarction (Dressler’s syndrome)
   Familial Mediterranean fever
Clinical Manifestations

Chest pain of acute infectious (viral) pericarditis typically develops in young adults (18 to 30 years) 1 to 2 weeks after a “viral illness.” The symptoms are sudden and severe in onset, characteristically with retrosternal or left precordial pain and referral to the back and trapezius ridge. Pain may be preceded by low-grade fever (in contrast to myocardial infarction, in which the pain precedes the fever). Although radiation to the arms in a manner similar to myocardial ischemia also may occur, it is less common. The pain is often pleuritic (e.g., accentuated by inspiration or coughing) and may be aggravated (supine or left lateral decubitus posture) or relieved (upright posture) by changes in posture.

The physical examination in patients with acute pericarditis is most notable for a pericardial friction rub. Although classically described as triphasic, with systolic and early (passive ventricular filling) and late (atrial systole) diastolic components, more commonly a biphasic (systole and diastole) or a monophasic rub may be heard. The rub may be transient and positional, often best appreciated in the supine or left lateral decubitus posture. Low-grade fever, resting tachycardia, and atrial ectopy are common, but atrial fibrillation is unusual.

Diagnosis

Diagnosis must proceed expeditiously to exclude emergent problems ( Fig. 77-2 ). Electrocardiographic changes ( Fig. 77-3 ) are common, particularly with an infectious etiology because of associated inflammation of the superficial epicardium. During the initial few days, diffuse (limb leads and precordial leads) ST segment elevations occur in the absence of reciprocal ST segment depression. PR segment depression also is common and reflects atrial involvement. After several days, the ST segments normalize and then the T waves become inverted (in contrast to the electrocardiographic changes seen with myocardial infarction, in which the temporal relationship of the T wave inversions is earlier and precedes normalization of the ST segment changes). In a large pericardial effusion, tachycardia, loss of R wave voltage (absolute R wave magnitude of 5 mm or less in all limb leads and 10 mm or less in all precordial leads), and electrical alternans ( Fig. 77-4 ) also may be seen (see Pericardial Effusion). Blood tests reflect an inflammatory state, with an elevated sedimentation rate, C-reactive protein level, and usually, leukocyte count. A mildly increased creatine kinase MB fraction and elevated troponin level occur in up to half of patients and are thought to represent epicardial inflammation rather than myocardial necrosis. If the biomarker elevation persists for several weeks or is associated with ventricular dysfunction, myocarditis with or without concomitant pericarditis should be considered.

FIGURE 77-2  Initial management of patients with pericarditis. ASA, aspirin; CXR, chest radiograph; ECG, electrocardiogram; JVP, jugular venous pressure; NSAIDs, nonsteroidal anti-inflammatory drugs.  (Modified from Malik F, Foster E: Pericardial disease. In Wachter RM, Goldman L, Hollander H [eds]: Hospital Medicine, 2nd ed. Philadelphia, Lippincott Williams & Wilkins, 2005, p 448.)

FIGURE 77-3  A 12-lead electrocardiogram from a patient with acute pericarditis. Note the diffuse ST-T wave changes and PR elevation in lead aVR and PR segment depression in leads II and aVF and in the precordial leads.  (Courtesy of Ary L. Goldberger, MD.)

FIGURE 77-4  Lead II rhythm strip taken from a patient with acute pericarditis complicated by a large pericardial effusion and tamponade physiology. Note the resting sinus tachycardia with relatively low voltage and electrical alternans.  (Courtesy of Ary L. Goldberger, MD.)

If the pericardial effusion is minimal, the chest radiograph is often unrevealing, although a small left pleural effusion may be seen. With larger effusions (see Pericardial Effusion), there may be a loss of distinct cardiac contours and “water bottle” appearance to the cardiac silhouette ( Fig. 77-5 ).

FIGURE 77-5  Posteroanterior chest radiograph in a patient with a large pericardial effusion. Note the loss of customary heart borders and a “water bottle” configuration.  (Courtesy of Sven Paulin, MD.)

Treatment

In the absence of significant pericardial effusion (see later), treatment that is directed primarily at relieving the patient’s symptoms can be successful in 85% or so of cases on an outpatient basis. Among nonsteroidal anti-inflammatory drugs, indomethacin (25 to 50 mg three times daily) is commonly prescribed, but ibuprofen (300 to 800 mg three or four times a day) or aspirin (325 to 650 mg three times daily) also may be used. Glucocorticoids (prednisone, 20 to 60 mg/day) may be useful for resistant situations. Anti-inflammatory drugs should be continued at a constant high dose until the patient is afebrile and asymptomatic for 5 to 7 days, followed by a gradual taper during the next several weeks. The use of warfarin or heparin should be avoided to minimize the risk of hemopericardium, but anticoagulation may be required in atrial fibrillation or in the presence of a coexistent prosthetic valve. Avoidance of vigorous physical activity is recommended during the acute and early convalescent periods. For patients with a first episode of viral or idiopathic pericarditis, colchicine (0.6 to 1.2 mg/day for 3 to 12 months) reduces the recurrence rate from about 32% to about 11%.[1] Colchicine is also effective in patients with familial Mediterranean fever ( Chapters 175 and 297 ).

Viral and idiopathic pericarditis usually is self-limited, but a quarter of patients may have recurrent pericarditis. For this group, prolonged treatment with nonsteroidal anti-inflammatory drugs (e.g., ibuprofen, 300 to 600 mg three times a day) plus colchicine (0.6 mg twice daily, declining to once daily after a year) should be considered.[2] For the 10 to 14% of patients who are intolerant of colchicine and have recurrent episodes despite high-dose nonsteroidal anti-inflammatory drugs (e.g., indomethacin, 50 mg three times a day, or ibuprofen, 800 mg four times a day), oral steroids (e.g., prednisone, 60 mg with a 2- to 4-week taper) and pericardiectomy should be considered. Patients with recurrent pericarditis are at increased risk for progression to constrictive pericarditis (see later).

   PERICARDIAL EFFUSION

Excess fluid may develop in the pericardial space in all forms of pericardial disease ( Table 77-2 ). Most commonly, the fluid is exudative and reflects pericardial injury or inflammation. Serosanguineous effusions are typical of tuberculous and neoplastic disease but also may be seen in uremic and viral or idiopathic disease or in response to mediastinal irradiation. Hemopericardium is seen most commonly with trauma, myocardial rupture after myocardial infarction, catheter-induced myocardial or epicardial coronary artery rupture, aortic dissection with rupture into the pericardial space, or primary hemorrhage in patients receiving anticoagulant therapy (often after cardiac valve surgery). Chylopericardium is rare and results from leakage or injury to the thoracic duct.


TABLE 77-2   — 
PRESENTATION AND TREATMENT OF THE MOST COMMON SPECIFIC CAUSES OF PERICARDITIS

Type of Cause Pathogenesis or Etiology Diagnosis Treatment Complications Comments
Viral

   Coxsackievirus B
   Echovirus type 8
   Epstein-Barr virus

   Leukocytosis
   Elevated erythrocyte sedimentation rate
   Mild cardiac biomarker elevation
Symptomatic relief, NSAIDs

   Tamponade
   Relapsing pericarditis
Peaks in spring and fall
Tuberculosis Mycobacterium tuberculosis

   Isolation of organism from biopsy fluid
   Granulomas not specific

   Triple-drug antituberculosis regimen
   Pericardial drainage followed by early (4–6 wk) pericardiectomy if signs of tamponade or constriction develop

   Tamponade
   Constrictive pericarditis
1–8% of patients with tuberculosis pneumonia; rule out HIV infection
Bacterial

   Group A streptococcus
   Staphylococcus aureus
   Streptococcus pneumoniae

   Leukocytosis with marked left shift
   Pericardial fluid purulent

   Pericardial drainage by catheter or surgery
   Systemic antibiotics
   Pericardiectomy if constrictive physiology develops
Tamponade in one third of patients Very high mortality rate if not recognized early
Post myocardial infarction 12 hours–10 days after infarction

   Fever
   Pericardial friction rub
   Echo: effusion

   Aspirin
   Prednisone
Tamponade rare

   More frequent in large Q wave infarctions
   Anterior > inferior
Uremic

   Untreated renal failure: 50%
   Chronic dialysis: 20%
Pericardial rub: 90%

   Intensive dialysis
   Indomethacin: probably ineffective
   Catheter drainage
   Surgical drainage

   Tamponade
   Hemodynamic instability on dialysis

   Avoid NSAIDs
   About 50% respond to intensive dialysis
Neoplastic In order of frequency: lung cancer, breast cancer, leukemia and lymphoma, others

   Chest pain, dyspnea
   Echo: effusion
   CT, MRI: tumor metastases to pericardium
   Cytologic examination of fluid positive in 85%

   Catheter drainage
   Subxiphoid pericardiectomy
   Chemotherapy directed at underlying malignant neoplasm

   Tamponade
   Constriction
 

Modified from Malik F, Foster E: Pericardial disease. In Wachter RM, Goldman L, Hollander H (eds): Hospital Medicine, 2nd ed. Philadelphia, Lippincott Williams & Wilkins, 2005, p 449.

CT = computed tomography; HIV = human immunodeficiency virus; MRI = magnetic resonance imaging; NSAIDs = nonsteroidal anti-inflammatory drugs.

Although the presence of pericardial effusion indicates underlying pericardial disease, the clinical relevance of the pericardial effusion is associated most closely with the rate of fluid collection, intrapericardial pressure, and subsequent development of tamponade physiology. A rapidly accumulating effusion, as in hemopericardium caused by trauma or aortic dissection, may result in tamponade physiology with collection of only 100 to 200 mL. By comparison, a more slowly developing effusion (hypothyroidism or chronic renal failure) may allow gradual stretching of the pericardium, with effusions exceeding 1500 mL in the absence of hemodynamic embarrassment.

Diagnosis

Pericardial effusion often is suspected clinically when the patient has symptoms and signs of tamponade physiology (see later), but it also may be suggested first by unsuspected cardiomegaly on the chest radiograph, especially if loss of the customary cardiac borders and a water bottle configuration are noted ( Fig. 77-5 ). Fluoroscopy, which may display minimal or absent motion of cardiac borders, is performed commonly when myocardial or epicardial coronary artery perforation is suspected during a diagnostic or interventional percutaneous procedure.

In most situations, two-dimensional transthoracic (surface) echocardiography is the diagnostic imaging procedure of choice for the evaluation and qualitative assessment of suspected pericardial effusion ( Fig. 77-6 ). In emergency situations, it can be performed at the bedside. The subcostal four-chamber view is the most informative imaging plane; it is particularly relevant because it allows the size and location of the effusion to be assessed from an orientation that determines whether the effusion can be drained percutaneously. Transudative effusions typically appear relatively echolucent (see Fig. 77-6 ), whereas organized-exudative and hemorrhagic effusions have an echo-filled or a ground-glass appearance ( Fig. 77-7 ). Stranding, which may be appreciated in organized or chronic effusions, suggests loculation and an inability to drain the effusion fully by percutaneous approaches. In patients with large effusions, which are associated with electrical alternans (see Fig. 77-4 ), the heart may appear to swing freely within the pericardial sac.

FIGURE 77-6  Transthoracic echocardiogram from the subcostal approach. Note the large echolucent area/pericardial effusion (arrows) surrounding the heart. The right ventricle is compressed.

FIGURE 77-7  Transthoracic echocardiogram from the parasternal long-axis window in a different patient than the one in Figure 77-6 . Note the large echo-filled pericardial effusion posterior (straight white arrows) to the left ventricle and anterior (curved white arrow) to the right ventricle. This patient had a hemorrhagic pericardial effusion that developed several weeks after aortic valve replacement and long-term warfarin treatment. A pleural effusion (black arrow) also is seen.

Cardiac Tamponade

Accumulation of fluid in the pericardium with a resultant increase in pericardial pressure and impairment of ventricular filling results in cardiac tamponade. Although progression to tamponade, which may be fatal if it is not recognized quickly and treated aggressively, occurs in 10 to 15% of patients with idiopathic pericarditis, it develops in more than 50% of patients with oncologic, tuberculous, or purulent pericarditis. The hallmarks of cardiac tamponade are increased intracardiac pressure and the resulting impaired ventricular filling and depressed cardiac output. In tamponade, ventricular filling is impaired throughout diastole; by comparison, early diastolic filling is relatively normal with pericardial constriction. Invasive hemodynamic assessment reveals equalization of right and left atrial and right and left ventricular diastolic pressures. Tamponade may not be an “all-or-none” phenomenon; mild or “low-pressure” tamponade can be seen when intrapericardial pressures are only modestly elevated, with resultant equalization of atrial pressures but not diastolic ventricular pressures.

Clinical Manifestations

The clinical features of cardiac tamponade may mimic those of heart failure, with dyspnea on exertion, orthopnea, and hepatic engorgement. Many clinical features help distinguish cardiac tamponade from constrictive pericarditis and restrictive cardiomyopathy ( Table 77-3 ). The typical physical examination with tamponade includes jugular venous distention with a prominent x descent ( Fig. 77-8 ), sinus tachycardia with hypotension, narrow pulse pressure, elevated (>10 mm Hg) pulsus paradoxus, and distant heart sounds. The pulsus paradoxus may be apparent with palpation, but more commonly it is measured with a sphygmomanometer during slow respiration; direct arterial monitoring is not generally necessary for quantification. A small (<10 mm Hg) pulsus is normal and is related to the ventricles being confined within the pericardium and sharing a common septum. With inspiration, right ventricular filling is enhanced, displacing the interventricular septum toward the left ventricle and exaggerating the reduction in left ventricular filling and resultant stroke volume. The exaggerated pulsus is not specific for tamponade; it also may be present with hypovolemic shock, chronic obstructive pulmonary disease, and bronchospasm.


TABLE 77-3   — 
COMPARISON OF PHYSICAL EXAMINATION FINDINGS AND DIAGNOSTIC TEST RESULTS FOR CARDIAC TAMPONADE, CONSTRICTIVE PERICARDITIS, AND RESTRICTIVE CARDIOMYOPATHY

Characteristic Cardiac Tamponade Constrictive Pericarditis Restrictive Cardiomyopathy
CLINICAL
Pulsus paradoxus + +/-
Prominent y descent +
Prominent x descent + +
Kussmaul’s sign +
S3 or pericardial “knock” + +
S4 +
ELECTROCARDIOGRAPHY
Low voltage + + +
Abnormal P waves + +/-
Electrical alternans + +
CHEST RADIOGRAPHY
Cardiomegaly +
Pericardial calcification +
ECHOCARDIOGRAPHY
Pericardial effusion +
Pericardial thickening +
Small right ventricle +
Thickened myocardium +
Enhanced respiratory variation in E wave + +
COMPUTED TOMOGRAPHY, MAGNETIC RESONANCE IMAGING
Pericardial thickening +
Pericardial calcification +
CARDIAC CATHETERIZATION      
Equalization of pressures + +
Abnormal myocardial biopsy +

FIGURE 77-8  Simultaneous right atrial (RA), intrapericardial, and femoral artery (FA) pressure recordings in a patient with cardiac tamponade. Note the elevated and equilibrated intrapericardial and right atrial pressures with a prominent x descent and blunted y descent suggestive of impaired right atrial emptying in early diastole. The arterial pulse pressure is narrowed.  (From Lorell BH: Profiles in constriction, restriction and tamponade. In Baim DS, Grossman W [eds]: Cardiac Catheterization, Angiography, and Intervention, 6th ed. Philadelphia, Williams & Wilkins, 2000, p 840.)

Diagnosis

For patients in whom the history or physical examination suggests tamponade, emergency transthoracic echocardiography is imperative and generally diagnostic. Echocardiographic evidence of tamponade physiology includes a compressed or small right ventricular chamber with late diastolic invagination of the right atrial and right ventricular free wall on two-dimensional imaging ( Chapter 53 ). Because of the frequent coexistence of tachycardia, diastolic invagination sometimes is appreciated best with higher temporal resolution M-mode echocardiography. In addition to diastolic invagination, M-mode echocardiography also may show exaggerated inspiratory septal motion and variation in the duration of aortic valve opening. Localized right atrial, left atrial, and left ventricular diastolic collapse also may be seen and is particularly relevant for loculated effusions, such as effusions after trauma and cardiac surgery. Pseudoprolapse of the mitral valve may be seen because of the compressed left ventricular cavity. When surface echocardiography is inadequate, as in a post-thoracotomy patient or a patient with chest wall trauma, transesophageal echocardiography may be helpful. Thoracic CT and MRI may be particularly valuable for delineation of loculated pericardial effusions. Finally, Doppler echocardiography may be used to assess transtricuspid and transmitral flow profiles, with an exaggerated peak E wave respiratory variation seen in tamponade. Many of these typical echocardiographic findings may be absent in patients who have significant pulmonary artery hypertension or are on a ventilator.

Treatment

When tamponade is suggested clinically and confirmed on echocardiography, acute management includes maintenance of systolic blood pressure with volume resuscitation. In dire circumstances, immediate pericardiocentesis may be life-saving ( Fig. 77-9 ). When time allows, right-sided heart catheterization should be performed to confirm elevated intrapericardial pressure and “equalization” of right atrial, left atrial, pulmonary capillary wedge, right ventricular diastolic, and left ventricular diastolic pressures. If echocardiography shows at least 1 cm of fluid anterior to the mid right ventricular free wall throughout diastole, percutaneous pericardiocentesis generally can be performed safely. During this procedure, a small catheter is advanced over a needle inserted into the pericardial cavity. Echocardiographic guidance is particularly useful for smaller effusions or if percutaneous pericardiocentesis is performed by less experienced operators. As much fluid as possible should be removed, with monitoring of filling pressures. Unless the cause already has been identified, pericardial fluid should be sent for evaluation (including pH, glucose, lactate dehydrogenase, protein, cell count, and cytology as well as staining and culture for bacteria, fungi, and tuberculosis). A flexible drainage catheter may be left in the pericardial space for several days to avoid early reaccumulation. Before the catheter is removed, serial echocardiography should be performed to confirm that the fluid has not reaccumulated.

Hemodynamically significant effusions of less than 1 cm, organized or multiloculated effusions, and focal effusions confined to the posterior or lateral cardiac borders or around the atria should be approached surgically through a limited thoracotomy-mediastinoscopy and pericardial window. For all effusions related to a malignant neoplasm and for which aggressive chemotherapy is not being administered, reaccumulation in the ensuing weeks or months is the norm, and elective surgery (pericardial window) should be considered before hospital discharge. Hemorrhagic effusions related to cardiac trauma or aortic dissection also are managed best by emergency surgery (if it is available) or in combination with temporizing pericardiocentesis. If the patient is in extremis, emergency pericardiocentesis should be performed at the bedside.

FIGURE 77-9  Aspiration of pericardial fluid is indicated in cardiac tamponade or to obtain fluid for diagnostic purposes. A wide-bore needle is inserted in the epigastrium below the xiphoid process and advanced in the direction of the medial third of the right clavicle. The procedure is preferably performed in a catheterization laboratory under echocardiographic guidance, but it may need to be performed emergently for life-saving purposes in other settings. If the needle is connected to the V lead of an electrocardiographic monitor, ST elevation usually is seen if the needle touches the epicardium. This can be useful in distinguishing a bloody pericardial effusion from accidental puncture of the heart. Other complications of the procedure may include arrhythmias, vasovagal attack, and pneumothorax.  (From Forbes CD, Jackson WF: Color Atlas and Text of Clinical Medicine, 3rd ed. London, Mosby, 2003, with permission.)

   Approach to Effusion without Tamponade

For patients with suspected pericardial effusion, transthoracic echocardiography is the initial test of choice and in most patients is definitive in confirming the presence or absence of a significant pericardial effusion (loculated effusions may be identified better by CT or MRI). If a small (0.5 to 1 cm) echolucent or “organized” pericardial effusion is seen, the patient generally can be observed with a follow-up echocardiogram in 1 to 2 weeks (sooner if clinical deterioration is evident). If the follow-up study shows a smaller effusion, subsequent echocardiograms are not necessary (unless the patient’s clinical condition changes). Assuming a clinical history of “viral” pericarditis, assessment of renal function and thyroid-stimulating hormone is reasonable, but the results probably will be normal. A tuberculin skin test should be performed routinely. One also should exclude a drug-induced etiology (e.g., cromolyn, hydralazine, isoniazid, phenytoin, procainamide, reserpine).

In a moderate (1 to 2 cm) or large (>2 cm) pericardial effusion, treatment and follow-up depend on the clinical scenario and echocardiographic findings. If the patient is clinically unstable and tamponade is suggested (see earlier), urgent cardiology consultation and diagnostic or therapeutic pericardiocentesis should be planned. If the patient is hemodynamically stable and tamponade is not suggested, the patient can be observed with a follow-up echocardiographic study performed in 1 to 7 days. The initial evaluation is the same as listed earlier for a small effusion. Follow-up echocardiographic studies should be continued until the size of the effusion is minimal, but echocardiograms need not be repeated until complete resolution. If bacterial or malignant pericarditis is suspected, diagnostic pericardiocentesis should be performed even in the absence of clinical instability or suggestion of tamponade; tuberculous pericarditis is diagnosed best by pericardial biopsy. A tuberculin skin test, complete blood count with differential, platelet count, and coagulation parameters also should be assessed. Anticoagulation with heparin or warfarin should be discontinued unless the patient has a mechanical heart valve or atrial fibrillation. Blood cultures are indicated if an infectious cause is suspected. Complement, antinuclear antibodies, and the sedimentation rate may be helpful if systemic lupus erythematosus is being considered, although isolated pericardial effusion is unlikely to be the first manifestation of this disorder. Pericarditis after myocardial infarction (Dressler’s syndrome) is now unusual; given experimental laboratory evidence that some of the nonsteroidal drugs promote left ventricular aneurysm formation in this setting, aspirin is the preferred agent to relieve pain in Dressler’s syndrome. The presence of an echo-filled effusion should raise concern for hemorrhagic or organized pericarditis, which may progress to constriction.

   Chronic or Recurrent Pericardial Effusions

With chronic or recurrent pericarditis from any cause, pericardial calcification develops and can be appreciated by thoracic CT ( Fig. 77-10 ). Symptoms are those of a chronic systemic illness and include weight loss, fatigue, and dyspnea on exertion ( Chapter 345 ).

FIGURE 77-10  Transverse computed tomography of a 32-year-old patient with anterior and posterior pericardial calcification (arrows).  (Courtesy of Noriko Oyama, MD.)

The evaluation of chronic pericarditis should exclude the possibility of tuberculosis; a tuberculin skin test, chest radiograph, and (when highly suspicious) analysis of gastric aspirates should be performed ( Chapter 345 ). Pericardial biopsy is more commonly diagnostic of tuberculous pericarditis than is pericardial fluid staining or culture. Aggressive drug treatment is indicated ( Chapter 345 ).

Hypothyroidism-myxedema is another common cause of large pericardial effusions, especially in the elderly ( Chapter 244 ). The effusion commonly is identified first on a chest radiograph and often is seen in the absence of resting tachycardia. Measurement of thyroid-stimulating hormone is diagnostic. The effusion and coexistent cardiomyopathy respond to hormone replacement, but sometimes slowly during several months. In the absence of hemodynamic compromise, pericardiocentesis often is not needed in this situation as the effusion has developed slowly and does not present hemodynamic compromise. Uremic pericardial effusions also are common and often respond to initiation of or more intensive dialysis ( Chapter 131 ).

Treatment of chronic or recurrent idiopathic effusions is similar to the treatment of recurrent pericarditis. If medical therapy is unsuccessful, creation of a pericardial window is indicated.

   CONSTRICTIVE PERICARDITIS

Constrictive pericarditis is an uncommon condition with impairment of mid and late ventricular filling from a thickened or noncompliant pericardium. In the classic form, fibrous scarring and adhesions of both pericardial layers lead to obliteration of the pericardial cavity. Early ventricular filling is unimpeded, but diastolic filling subsequently is reduced abruptly as a result of the inability of the ventricles to fill because of physical constraints imposed by a rigid, thickened, and sometimes calcified pericardium. In less developed countries, tuberculosis is the most common cause of chronic constrictive pericarditis, whereas in the United States, tuberculosis is infrequently the culprit. Constriction may be associated with malignant disease (lung cancer, breast cancer, lymphoma), histoplasmosis, mediastinal irradiation, purulent or recurrent viral pericarditis, rheumatoid arthritis, uremia, chest trauma or hemopericardium, and cardiac surgery. Constriction may follow cardiac surgery by several weeks to months and may occur decades after chest wall irradiation. The “cause” may not be identified in many patients.

Pathobiology

The normal pericardium is 3 mm or less thick. With chronic constriction, especially from tuberculosis, the pericardium may thicken to 6 mm or more, calcify, and intimately involve the epicardium. In subacute constriction, calcification is less prominent, and the pericardium may be only minimally thickened. As with cardiac tamponade, the pathophysiologic process of constriction includes impaired diastolic ventricular filling, which leads to elevated venous pressure. Tamponade and constriction have many important differences (see Table 77-3 ), however. With constriction, the impairment in ventricular filling is minimal in early diastole, and a prominent y descent is present ( Fig. 77-11 ). Subsequently, diastolic pressure rises abruptly when cardiac volume reaches the anatomic limit set by the noncompliant pericardium; by comparison, in tamponade, ventricular filling is impaired throughout diastole. Diastolic pressure remains elevated until the onset of systole. This prominent y descent with an elevated plateau of ventricular pressure has been termed the “dip and plateau” or “square root” sign ( Fig. 77-12 ); by comparison, in tamponade, the y descent is absent. Stroke volume and cardiac output are reduced because of impaired filling, whereas intrinsic systolic function of the ventricles may be normal or only minimally impaired.

FIGURE 77-11  Right atrial (RA) pressure recording from a patient with constrictive pericarditis. Note the elevation in pressure and prominent y descent corresponding to rapid early diastolic right atrial emptying.  (From Lorell BH: Profiles in constriction, restriction and tamponade. In Baim DS, Grossman W [eds]: Cardiac Catheterization, Angiography, and Intervention, 6th ed. Philadelphia, Williams & Wilkins, 2000, p 832.)

FIGURE 77-12  Simultaneous left ventricular (LV) and right ventricular (RV) pressure recordings in a patient with constrictive pericarditis. Note the equilibration of LV and RV diastolic pressures and the “dip and plateau” most apparent with the prolonged diastole.  (From Lorell BH: Profiles in constriction, restriction and tamponade. In Baim DS, Grossman W [eds]: Grossman’s Cardiac Catheterization, Angiography, and Intervention, 6th ed. Philadelphia, Lippincott Williams & Wilkins, 2000, p 832.)

Clinical Manifestations

In constriction, the most prominent physical finding is an abnormal jugular venous pulse ( Chapter 48 ). Central venous pressure is elevated and displays prominent x and y descents. For patients in sinus rhythm, the x descent is coincident with the carotid pulse. The y descent, which is absent or diminished in tamponade, is most prominent and abbreviated because of a rapid rise in pressure in mid-diastole. A diagnosis of constriction always should be suspected in patients with a prominent y descent with dyspnea, weakness, anorexia, peripheral edema, hepatomegaly, splenomegaly, and ascites. The pulse pressure is often narrowed, but pulsus paradoxus is usually absent. Pleural effusions are common. The clinical picture may mimic hepatic cirrhosis, but with distended neck veins. Venous pressure often fails to fall with inspiration (Kussmaul’s sign), and arterial pulse pressure is normal or reduced. The apical pulse is often poorly defined, and heart sounds may be distant. A loud S3, the pericardial knock, may be audible early after aortic valve closure because of the sudden deceleration in ventricular filling.

Diagnosis

The electrocardiogram of patients with constriction is often abnormal and displays low QRS voltage (especially in the limb leads), P mitrale, and nonspecific ST-T wave changes. Atrial fibrillation may be present in one third of patients. The chest radiograph may show pericardial calcification in tuberculous constriction. Though suggestive, the finding of pericar-dial calcification is not diagnostic of constriction. Cardiac size may be small, normal, or enlarged. Transthoracic echocardiography is less helpful than with cardiac tamponade, but it may display pericardial thickening or calcification, abrupt posterior deflection of the interventricular septum at end diastole, and M-mode posterior wall “flat tiring.” Enhanced transmitral and transtricuspid Doppler E wave variation with respiration may be particularly helpful in establishing the diagnosis. The inferior vena cava and hepatic veins often are markedly dilated with blunted respiratory variability in caval diameter. Newer tissue Doppler imaging is also helpful to distinguish constrictive pericarditis from restrictive cardiomyopathy ( Chapter 59 ); constrictive pericarditis displays normal or enhanced early diastolic indexes.

Increased pericardial thickness is diagnosed most reliably by CT or MRI (see Fig. 77-10 ). CT is more helpful for the identification of pericardial calcification. Right atrial, inferior vena cava, and hepatic vein distention also are seen commonly with CT and MRI. Like chest radiography, CT and MRI do not indicate the physiologic significance of these anatomic findings and need to be interpreted in the context of the clinical findings.

At cardiac catheterization, patients with chronic constrictive pericarditis usually have elevation (>15 mm Hg) and equalization (within 5 mm Hg) of right atrial, right ventricular diastolic, pulmonary capillary wedge, and left ventricular diastolic pressures. Right ventricular end-diastolic pressure is often one third of systolic pressure, and pulmonary artery hypertension is mild. Cardiac output usually is depressed. Right atrial pressure is characterized by a preserved x descent with a prominent early diastolic y descent. The right atrial pressure fails to decrease appropriately or may rise during inspiration. Right and left ventricular diastolic pressures display an early diastolic dip followed by a plateau (see Fig. 77-12 ), although this finding may be difficult to appreciate if the patient is tachycardic or in atrial fibrillation.

Treatment

Constrictive pericarditis occasionally may reverse spontaneously when it develops in acute pericarditis. More commonly, the natural history of this disease is one of progression with declining cardiac output and progressive renal and hepatic failure. Surgical stripping or removal of both layers of the adherent pericardium is the definitive therapy. The benefits of pericardial stripping may be modest initially but continue to be manifested in the ensuing months. Operative mortality is generally low but may exceed 5 to 15% in the most advanced cases. The surgical risk is related to the extent of myocardial involvement and the severity of secondary hepatic or renal dysfunction. For patients with suspected tuberculous constriction, antituberculosis therapy should be administered before and after pericardial surgery. In addition to advanced age and systolic dysfunction, postirradiation constriction is a predictor of worse prognosis.

   Effusive-Constrictive Pericarditis

Effusive-constrictive pericarditis is a rare disorder occurring in about 1% of patients who have pericarditis and approximately 7% of patients with tamponade. It is characterized by the combination of a tense pericardial effusion in the presence of visceral pericardial constriction and may represent an intermediate stage in the development of constrictive pericarditis. Causes of effusive-constrictive pericarditis are the same as those associated with constriction, and the clinical features resemble those of tamponade and constriction. Physical examination shows pulsus paradoxus and a prominent x descent in the absence of a y descent. The cardiac silhouette is generally enlarged because of the associated pericardial effusion, whereas the electrocardiogram displays low QRS voltage and nonspecific ST-T wave changes. Surface echocardiography may show an echo-filled pericardial effusion with thickened pericardium and fibrinous pericardial bands. Although this echocardiographic appearance should heighten suspicion, the diagnosis generally is made after successful pericardiocentesis. Rather than normalizing after pericardiocentesis, intracardiac pressures remain elevated with a square root sign in the ventricular tracings and development of a prominent y descent in the atrial and jugular venous pressure pulses. Kussmaul’s sign also may be evident. Treatment by excision of visceral and parietal pericardium is usually effective. A transient, self-limited form of effusive-constrictive pericarditis has also been reported.

Future Directions

Access to the pericardial space may provide a new means to deliver novel gene or pharmacologic therapies for myocardial (angiogenesis, antiarrhythmics) or pericardial disease.

Email to Colleague Print Version

Copyright © 2007 Elsevier Inc. All rights reserved. –

Leave a Reply


Time limit is exhausted. Please reload the CAPTCHA.

Categories

apply_now Pepperstone Group Limited