Chronic pericarditis

Chronic pericarditis is an inflammatory disease of the pericardium lasting more than 6 months, arising as primary-chronic processes or as a result of chronic or recurrent course of acute pericarditis; include exudative, adhesive, exudative-constrictive and constrictive forms.

ICD-10 code

• 131.0. Chronic adhesive pericarditis,
• 131.1 Chronic constrictive pericarditis,
• 131.8. Other specified diseases of the pericardium,
• 131.9. Pericardial diseases, unspecified.

Epidemiology of chronic pericarditis

The disease is rare, perhaps at any age.

[1], [2], [3], [4], [5]

Causes of chronic pericarditis

Constriction of the pericardium usually occurs due to a long-term inflammation leading to fibrosis, thickening and calcification of the pericardium. Pericarditis of any etiology in the outcome can lead to the construction of the heart.

Typical causes of constrictive pericarditis:

• Idiopathic: in 50-60% of cases, no underlying disease is found (it can be assumed that previously unrecognized viral pericarditis was transferred).
• Infectious (bacterial): tuberculous pericarditis, bacterial infections leading to purulent pericarditis (3-6%).
• Radiation: long-term effects (after 5-10 years) of mediastinal and thoracic irradiation (10-30%).
• Post-surgical: any operative or invasive interventions, in which the pericardium was damaged (11-37%).

Less frequent causes of chronic pericarditis:

• Fungal infections (Aspergillus, Candida, Coccidioides) in patients with immunodeficiency.
• Tumors: Malignant proliferation (most typical metastases of lung, breast and lymphoma cancer) can manifest as a palpable heart with a thickening of the visceral and parietal pericardial sheets.
• Diseases of connective tissue (rheumatoid arthritis, SLE, systemic scleroderma, dermatomyositis) (3-7%).
• Medicinal: procainamide, hydralazine (drug-induced lupus syndrome), metisergid, cabergoline.
• Trauma of the chest wall (dull and penetrating).
• Chronic renal failure.

Rare causes of chronic pericarditis:

• Sarcoidosis.
• Myocardial infarction: cases of CP after myocardial infarction in the case of Dressler's history in the history or hemopericard after thrombolytic treatment are described.
• Percutaneous interventions on coronary vessels and pacemakers.
• Hereditary family pericarditis (Nanism Malibrea).
• Disease gyer-IgG4 (in the literature single cases are described).

In developed countries, most cases of constrictive pericarditis are idiopathic or, presumably, viral or associated with thoracic surgery. In developing countries, infectious causes predominate, especially tuberculosis.

[6], [7]

Pathogenesis of chronic pericarditis

Pericardial constriction usually occurs when a dense, sclerotized thickened and, often, calcified pericardium limits the filling of the heart, causing a decrease in heart volume. Early diastolic filling becomes rapid due to high venous pressure, however, once the volume is limited to the pericardium, further diastolic filling is stopped. Restriction of the late filling phase leads to a characteristic diastolic "westing and plateau" on the pressure curve in the right and / or left ventricle and a decrease in the final diastolic volume of the ventricles. The pathophysiological marker of constriction of the heart by the pericardium is the equalization of the final diastolic pressure in all chambers of the heart (including pressure in the right and left atria, so the emerging venous stasis over the large circulation is much more pronounced than stagnation in a small circle). The dense pericardium reduces the influence of fluctuations in intrathoracic pressure associated with breathing on the filling of the heart chambers, leading to the Kussmaul symptom (no reduction in systemic venous pressure during inspiration), as well as a decrease in the filling of the left heart with inspiration. All this leads to chronic venous stasis and a decrease in cardiac output.

Constriction of the pericardium can occur without deposition of calcium in it, and in some cases even without a thickening of the pericardium (up to 25% of cases).

Chronic exudative pericarditis

Chronic exudative pericarditis is an inflammatory pericardial effusion that persists from several months to several years. Etiology is similar to acute pericarditis, but with a higher incidence of tuberculosis, tumor and immune-related diseases. Clinical symptoms and diagnosis of pericardial effusion are described above, slow-growing chronic effusions, as a rule, are slightly asymptomatic. With large asymptomatic chronic pericardial effusions, an unexpected deterioration with the development of a tamponade of the heart is often possible. To this predispose hypovolemia, paroxysms of tachyarrhythmias, relapses of acute pericarditis. It is important to diagnose a potentially curable form of the disease that is potentially curable or requires specific etiotropic treatment (tuberculosis, autoimmune and diffuse connective tissue diseases, toxoplasmosis). Symptomatic treatment and indications for pericardiocentesis and pericardial drainage are the same as in acute pericarditis. With frequent relapses of effusion with cardiac tamponade, surgical treatment (pericardiotomy, pericardectomy) can be indicated.

[8], [9], [10], [11], [12], [13], [14]

Chronic exudative-constrictive pericarditis

This is a rare clinical syndrome that is characterized by a combination of pericardial effusion and pericardial constriction with preservation of the design after removal of effusion. Any form of chronic pericardial effusion can be organized in a constrictive-exudative state, the most common cause of exudative-constrictive pericarditis is tuberculosis. The pericardial effusion in this disease is distinguished by the size and duration of existence, upon detection of effusion it is necessary to evaluate it for the purpose of determining etiology and hemodynamic significance. The mechanism of constriction of the heart is compression of the visceral pericardium. The thickening of the parietal and visceral pericardium can be established by echocardiography or MRI. Hemodynamic characteristics - a prolonged increase in the final diastolic pressure in the right and left ventricles after removal of the pericardial fluid returns the pressure in the pericardium to zero or close to zero. Not all cases of exudative-constrictive pericarditis progress to chronic constrictive pericarditis. Treatment with pericardiocentesis may be inadequate, visceral pericardectomy is indicated with confirmation of the persistent constriction of the visceral pericardium.

[15], [16], [17], [18]

Chronic constrictive pericarditis

Chronic constrictive pericarditis is a distant consequence of acute or chronic pericarditis, in which fibrous thickening, compaction and / or calcification of the parietal and, more rarely, visceral pericardium interfere with normal diastolic filling of the heart, leading to chronic venous congestion and a decrease in cardiac output, as well as compensatory sodium retention and liquid.

[19], [20], [21],

Symptoms of chronic pericarditis

Constrictive chronic pericarditis manifests itself in a variety of symptoms caused by increased systemic venous pressure and low cardiac output, which progresses, as a rule, for several years. The most characteristic of Bek's triad is high venous pressure, ascites, a "small quiet heart". The diagnosis of "constrictive pericarditis" should be suspected in patients with right-sided congestive heart failure with normal systolic function of the ventricles, with swelling of the jugular veins, pleural effusion, hepatosplenomegaly, ascites not explainable by other causes. At laboratory research of blood at patients KP often diagnose an anemia and increase in activity of hepatic enzymes.

To assess the etiology of the disease, the history of the disease (diseases, operations, cardiac trauma, radiation exposure) is important.

Thickening of the pericardium is not equivalent to a constrictive pathology, with a combination of clinical symptoms, echocardiographic and hemodynamic signs of cardiac constriction, the normal thickness of the pericardium does not exclude CP.

Clinical symptoms of chronic constrictive pericarditis

Complaints of the patient and the history of the disease:

• shortness of breath when exercising, coughing (not growing in prone position);
• augmentation of the abdomen, later - swelling of the lower extremities;
• weakness during exercise;
• chest pain (rarely);
• nausea, vomiting, diarrhea, bloating, pain and heaviness in the right upper quadrant (manifestations of venous circulation in the liver, intestines);
• quite often - the initial erroneous diagnosis of cryptogenic cirrhosis of the liver.

Inspection data and physical research methods.

General inspection:

• acrocyanosis, cyanosis of the face, increasing in prone position, puffiness of face, neck (Stokes collar);
• peripheral edema;
• in the expanded stages there can be a loss of muscle mass, cachexia and jaundice.

The cardiovascular system:

• swelling of the cervical veins (examination of patients in a vertical position and lying down), high venous pressure, a symptom of Kussmaul (increased or no decrease in systemic venous pressure during inspiration), swelling of the cervical veins increases with pressure on the right hypochondrium, pulsation of veins, their diastolic collapse (symptom Friedreich);
• apical impulse usually not palpable;
• the boundaries of cardiac dullness are usually little changed;
• tachycardia with exercise and at rest;
• heart sounds can be muffled, "pericardial tone" - an additional tone in the high-pitched proto diastole (corresponding to a sudden cessation of ventricular filling in early diastole) - occurs in almost half of the patients. This is a specific but not sensitive sign of the KP; at the beginning of the inhalation, a bifurcation of the second tone over the pulmonary artery is heard; sometimes - tricuspid insufficiency;
• paradoxical pulse (rarely exceeds 10 mm Hg, if there is no concomitant pericardial effusion with abnormally increased pressure), the pulse is weak, during deep inspiration may disappear (with a sign of Rigel);
• blood pressure is normal or low, it is possible to reduce the pulse pressure.

Systems of the digestive, respiratory, and other organs:

• Hepatomegaly with liver pulsation can be found in 70% of patients; splenomegaly, pseudocirrhosis of the liver of the peak;
• Other symptoms caused by chronic congestion in the liver; ascites, vascular asterisks, erythema of the palms;
• Pleural effusion (bowl is left-sided or bilateral).

Instrumental diagnosis of constrictive pericarditis (Guidelines for diagnosis and treatment of pericardial diseases of the European Society of Cardiology, 2004)

Methodology	Typical results
ECG	It may be normal, or a low QRS voltage, a generalized inversion or flattening of the T wave, an extended high P tooth (high P contrasts with a low QRS voltage), atrial fibrillation (in a third of patients), atrial flutter, atrioventricular blockage, intraventricular conduction disorders
Chest X-ray	A small, sometimes altered form, a heart, calcification of the pericardium, a "fixed" heart when the position changes, often - pleural effusion or pleural adhesions, pulmonary venous hypertension
Echocardiography	Thickening (more than 2 mm) and calcification of the pericardium, as well as indirect signs: constriction, atrial enlargement in normal form and normal systolic function of the ventricles (according to PV); paradoxical "pendular" movement of the interventricular septum in the early phase of diastole; flattening of systolic-diastolic movement of the posterior wall of the left ventricle; the diameter of the left ventricle does not increase after the early filling phase; lower hollow vein and hepatic veins dilated with limited respiratory vibrations
Doppler echocardiography	Restriction of filling of both ventricles (with differences in transmittral rate of filling, associated with respiration, more than 25%)
Transesophageal echocardiography	Evaluation of pericardial thickness
Computed tomography or MRI	Thickening (> 4 mm) and / or calcification of the pericardium, narrowed configuration of the right or both ventricles, an increase in one or both atria. Stretching of the hollow veins
Cardiac catheterization	"Diastolic wiggling and lazy" (or "square root") on the pressure curve in the right and / or left ventricles, equalizing the final diastolic pressure in the chambers of the heart (the difference between the end diastolic pressure in the left and right ventricles does not exceed 5 mm Hg. ); The decline of X is preserved and the Y drop of the pressure curve in the right atrium is expressed
Angiography of the ventricles	Decrease of ventricles and an increase in head injuries; rapid filling in the early phase of diastole with the cessation of further increase
Coroparography	Shown to patients older than 35 years

[22], [23], [24]

Indications for consultation of other specialists

Cardiologist (interpretation of the results of echocardiography, pericardiocentesis and invasive hemodynamic research).

Cardiosurgeon (assessment of indications for surgical treatment).

Differential diagnosis of chronic pericarditis

Includes:

• Restrictive cardiomyopathy (with arch dose, amyloidosis, hemochromatosis, endocarditis Leffler);
• congestive right ventricular heart failure of another etiology, including pulmonary heart, right ventricular infarction, tricuspid deformities;
• cardiac tamponade (with tamponade more often than with constriction, a paradoxical pulse is detected, there is no Y-fall of systemic venous pressure, expressed at constriction.) Systemic venous pressure decreases with inspiratory tamponade, while at constriction the venous inspiratory pressure does not decrease or increases);
• heart tumor - a mix of the right atrium, primary heart tumors (lymphoma, sarcoma);
• mediastinal tumors;
• exudative-constrictive pericarditis;
• cirrhosis of the liver (systemic venous pressure not increased);
• syndrome of the inferior vena cava, nephrotic syndrome and other hypo-oncotic conditions causing severe edema and ascites (eg, hypoalbuminemia in primary intestinal lymphangiectasia, intestinal lymphoma, Whipple's disease);
• ovarian carcinoma should be suspected in patients with ascites and edema;
• isolated calcification of the apex or posterior wall of the left ventricle is most likely associated with an aneurysm of the left ventricle than with calcification of the pericardium.

[25], [26], [27], [28], [29]

Treatment of chronic pericarditis

The goals of chronic pericarditis treatment are surgical correction of heart constriction and treatment of congestive heart failure

Indications for hospitalization

Hospitalization is indicated if necessary for invasive examinations and for surgical treatment.

Conservative treatment of chronic pericarditis

Conservative treatment of chronic pericarditis is performed with a minor degree of constriction, during the preparation for surgery or inoperable patients. Moreover, in some patients with a relatively acute recent appearance of pericardial constriction, the disappearance or reduction of symptoms and signs of constriction in the treatment with anti-inflammatory drugs, colchicine and / or glucocorticoids is described.

[30], [31], [32]

Non-pharmacological treatment of chronic pericarditis

• restriction of physical and emotional load;
• restriction of salt (optimally less than 100 mg / day) and fluid in the diet, alcohol consumption;
• annual vaccination against influenza;
• it is recommended to avoid the use of drugs that promote sodium retention (NSAIDs, glucocorticoids, licorice preparations).

Drug treatment of chronic pericarditis

Diuretics (loop) with swelling and ascites should be used in the lowest effective doses. It is necessary to avoid hypovolemia, arterial hypotension and kidney hypoperfusion. Additionally, potassium-sparing diuretics (under the control of kidney function and potassium plasma level) are used. Ultrafiltration of plasma can improve the condition of patients with severe volume overload.

It is necessary to avoid the appointment of beta-adrenoblockers or blockers of slow calcium channels, which reduce the compensatory sinus tachycardia. It is advisable not to reduce the heart rate below 80-90 per minute.

Angiotensin-converting enzyme inhibitors or angiotensin receptor blockers that can reduce blood pressure and induce renal hypoperfusion should be used with caution under the control of kidney function.

[33], [34], [35], [36]

Surgical treatment of chronic pericarditis

Pericardectomy with a wide removal of the visceral and parietal pericardium is the main method of treatment with pronounced chronic constriction. The complete disappearance of constrictive hemodynamic disorders after this operation is described in approximately 60% of patients. The operation is indicated for patients with COP with circulatory failure of the 2nd or 3rd functional class (MUNA). The operation is usually performed through median sternotomy access, in some cases, thoracoscopic access is appropriate. With purulent pericarditis, primary access with lateral thoracotomy. This operation with a significant operational risk is not indicated with weak manifestations of constriction, expressed by calcification of the pericardium or severe injury, marked fibrosis of the myocardium. Operational risk is highest in elderly patients, in cases of radiation related illness, with severe manifestations of constriction, severe renal dysfunction, and myocardial dysfunction.

Approximate terms of incapacity for work

With constrictive chronic pericarditis, work capacity, as a rule, is steadily reduced.

[37], [38]

Prognosis for chronic pericarditis

Operational mortality during pericardectomy at COP is 5-19% even in specialized institutions. The long-term prognosis after pericardectomy depends on the etiology of CP (a better prognosis for idiopathic constrictive chronic pericarditis). If indications for surgical treatment were established early, the long-term mortality after pericardectomy corresponds to mortality in the general population. Mortality in pericardectomy is most closely related to the unrecognized fibrosis of the myocardium before surgery.