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 chronicization or recurrent course of acute pericarditis; includes 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 pericardium,
• 131.9. Diseases of pericardium, unspecified.

Epidemiology of chronic pericarditis

The disease is rare and can occur at any age.

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Causes of Chronic Pericarditis

Pericardial constriction usually occurs as a result of long-standing inflammation, leading to fibrosis, thickening and calcification of the pericardium. Pericarditis of any etiology can ultimately lead to cardiac constriction.

Typical causes of constrictive pericarditis:

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

Less common causes of chronic pericarditis:

• Fungal infections (Aspergillus, Candida, Coccidioides) in immunocompromised patients.
• Tumors: Malignant spread (most typical metastases from lung, breast cancer, and lymphoma) may manifest as an armored heart with thickening of the visceral and parietal pericardium.
• Connective tissue diseases (rheumatoid arthritis, SLE, systemic scleroderma, dermatomyositis) (3-7%).
• Medicinal: procainamide, hydralazine (drug-induced lupus syndrome), methysergide, cabergoline.
• Chest wall trauma (blunt and penetrating).
• Chronic renal failure.

Rare causes of chronic pericarditis:

• Sarcoidosis.
• Myocardial infarction: Cases of CP after myocardial infarction have been described in patients with a history of Dressler syndrome or hemopericardium after thrombolytic treatment.
• Percutaneous coronary interventions and pacemakers.
• Hereditary familial pericarditis (Malibrey's dwarfism).
• Hypertension-IgG4 disease (isolated cases are described in the literature).

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

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Pathogenesis of chronic pericarditis

Pericardial constriction typically occurs when dense, sclerotic, thickened, and often calcified pericardium restricts cardiac filling, causing a decrease in cardiac volume. Early diastolic filling is rapid due to high venous pressure, but once the pericardial-limited volume is reached, further diastolic filling ceases. Restriction of the late filling phase results in a characteristic diastolic "trough and plateau" in the right and/or left ventricular pressure curve and a decrease in ventricular end-diastolic volume. The pathophysiological marker of cardiac constriction by the pericardium is equalization of end-diastolic pressure in all cardiac chambers (including pressure in the right and left atria, so that the resulting venous congestion in the systemic circulation is much more pronounced than congestion in the pulmonary circulation). A dense pericardium reduces the effect of intrathoracic pressure fluctuations associated with breathing on the filling of the heart chambers, leading to Kussmaul's sign (lack of decrease in systemic venous pressure during inspiration) and a decrease in the filling of the left chambers of the heart during inspiration. All this leads to chronic venous congestion and a decrease in cardiac output.

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

Chronic exudative pericarditis

Chronic exudative pericarditis is an inflammatory pericardial effusion that persists for several months to several years. The etiology is similar to acute pericarditis, but with a higher frequency of tuberculosis, tumors, and diseases associated with immune inflammation. Clinical symptoms and diagnostics of pericardial effusion are described above; slowly increasing chronic effusions are usually asymptomatic. In large asymptomatic chronic pericardial effusions, an unexpected deterioration with the development of cardiac tamponade is often possible. Hypovolemia, paroxysms of tachyarrhythmia, and relapses of acute pericarditis predispose to this. It is important to diagnose potentially curable forms of the disease or those requiring specific etiotropic treatment (tuberculosis, autoimmune and diffuse connective tissue diseases, toxoplasmosis). Symptomatic treatment and indications for pericardiocentesis and pericardial drainage are the same as for acute pericarditis. In case of frequent recurrence of effusion with cardiac tamponade, surgical treatment (pericardiotomy, pericardiectomy) may be indicated.

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Chronic exudative-constrictive pericarditis

This is a rare clinical syndrome characterized by a combination of pericardial effusion and pericardial constriction with preservation of the structure after removal of the effusion. Any form of chronic pericardial effusion can organize into a constrictive-exudative condition, the most common cause of exudative-constrictive pericarditis is tuberculosis. Pericardial effusion in this disease is differentiated by size and duration of existence, if effusion is detected, it must be assessed to determine the etiology and hemodynamic significance. The mechanism of cardiac constriction is compression by visceral pericardium. Thickening of the parietal and visceral pericardium can be established using echocardiography or MRI. Hemodynamic characteristic - prolonged increase in end-diastolic pressure in the right and left ventricles after removal of pericardial fluid returns the pressure in the pericardium to zero or close to zero. Not all cases of effusive-constrictive pericarditis progress to chronic constrictive pericarditis. Treatment with pericardiocentesis may not be sufficient; visceral pericardiectomy is indicated when persistent visceral pericardial constriction is confirmed.

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Chronic constrictive pericarditis

Chronic constrictive pericarditis is a late sequelae of acute or chronic pericarditis in which fibrous thickening, induration, and/or calcification of the parietal and, less commonly, visceral pericardium interfere with normal diastolic filling of the heart, leading to chronic venous congestion and decreased cardiac output, as well as compensatory sodium and fluid retention.

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Symptoms of Chronic Pericarditis

Constrictive chronic pericarditis presents with a variety of symptoms due to elevated systemic venous pressure and low cardiac output, which usually progress over several years. The most characteristic is Beck's triad - high venous pressure, ascites, "small quiet heart". The diagnosis of "constrictive pericarditis" should be suspected in patients with right-sided congestive heart failure with normal ventricular systolic function, with jugular vein distension, pleural effusion, hepatosplenomegaly, ascites, not explained by other causes. Laboratory blood tests in patients with CP often reveal anemia and increased liver enzyme activity.

To assess the etiology of the disease, anamnesis data (previous illnesses, surgeries, heart injuries, radiation exposure) are important.

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

Clinical symptoms of chronic constrictive pericarditis

Patient's complaints and medical history:

• shortness of breath during exertion, cough (does not worsen when lying down);
• abdominal enlargement, later - swelling of the lower extremities;
• weakness during physical exertion;
• chest pain (rare);
• nausea, vomiting, diarrhea, bloating, pain and heaviness in the right hypochondrium (manifestations of impaired venous circulation in the liver and intestines);
• often - the initial misdiagnosis of cryptogenic liver cirrhosis.

Data from examination and physical research methods.

General inspection:

• acrocyanosis, cyanosis of the face, which increases in the lying position, puffiness of the face and neck (Stokes collar);
• peripheral edema;
• In advanced stages there may be loss of muscle mass, cachexia and jaundice.

Cardiovascular system:

• swelling of the jugular veins (examine patients in an upright and lying position), high venous pressure, Kussmaul's symptom (increase or absence of decrease in systemic venous pressure during inhalation), swelling of the jugular veins increases with pressure on the right hypochondrium, pulsation of the veins, their diastolic collapse (Friedreich's symptom);
• the apex beat is usually not palpable;
• the boundaries of cardiac dullness are usually little changed;
• tachycardia during exercise and at rest;
• heart sounds may be muffled, "pericardial sound" - an additional tone in the protodiastole of a high timbre (corresponding to a sudden cessation of filling of the ventricles in early diastole) - occurs in almost half of patients. This is a specific, but not sensitive sign of CP; at the beginning of inspiration, a bifurcation of the second tone is heard over the pulmonary artery; sometimes - tricuspid insufficiency noise;
• paradoxical pulse (rarely exceeds 10 mm Hg, if there is no concomitant pericardial effusion with abnormally high pressure), the pulse is weak, may disappear during deep inspiration (with Riegel's sign);
• blood pressure is normal or low, pulse pressure may decrease.

Digestive, respiratory, etc. systems:

• hepatomegaly with liver pulsation can be found in 70% of patients; splenomegaly, Pick's pseudocirrhosis of the liver;
• other symptoms caused by chronic congestion of the liver; ascites, spider veins, erythema of the palms;
• pleural effusion (usually left-sided or bilateral).

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

Methodology	Characteristic results
ECG	May be normal, or low QRS voltage, generalized inversion or flattening of T waves, widened, tall P wave (tall P contrasts with low QRS voltage), atrial fibrillation (in one third of patients), atrial flutter, atrioventricular block, intraventricular conduction abnormalities are diagnosed
Chest X-ray	Small, sometimes malformed heart, pericardial calcification, "fixed" heart when changing position, often pleural effusion or pleural adhesions, pulmonary venous hypertension
EchoCG	Thickening (more than 2 mm) and calcifications of the pericardium, as well as indirect signs: constriction, enlargement of the atria with a normal appearance and normal systolic function of the ventricles (according to EF); paradoxical "pendulum-like" movement of the interventricular septum in the early phase of diastole; flattening of the 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; the inferior vena cava and hepatic veins are dilated with limited respiratory oscillations
Doppler echocardiography	Biventricular filling limitation (with respiratory-related transmitral filling velocity differences greater than 25%)
Transesophageal echocardiography	Assessment of pericardial thickness
Computed tomography or MRI	Thickening (>4 mm) and/or calcification of the pericardium, narrowed configuration of the right or both ventricles, enlargement of one or both atria. Distension of the vena cava
Cardiac catheterization	"Diastolic dip and llago" (or "square root") on the pressure curve in the right and/or left ventricles, equalization of the end diastolic pressure in the heart chambers (the difference between the end diastolic pressure in the left and right ventricles does not exceed 5 mm Hg); the X decline is preserved and the Y decline is pronounced on the pressure curve in the right atrium
Ventricular angiography	Diminution of the ventricles and enlargement of the atria; rapid filling in the early phase of diastole with cessation of further enlargement
Coroparagraphy	Shown to patients over 35 years of age

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Indications for consultation with other specialists

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

Cardiac surgeon (assessment of indications for surgical treatment).

Differential diagnosis of chronic pericarditis

Includes:

• restrictive cardiomyopathy (with arc dosa, amyloidosis, hemochromatosis, Loeffler's endocarditis);
• congestive right ventricular heart failure of other etiologies, including pulmonary heart disease, right ventricular infarction, tricuspid valve defects;
• cardiac tamponade (with tamponade, paradoxical pulse is detected more often than with constriction, the Y-drop in systemic venous pressure that is expressed with constriction is absent. Systemic venous pressure with tamponade decreases on inspiration, whereas with constriction venous pressure with inspiration does not decrease or increases);
• cardiac tumors - myxoma of the right atrium, primary cardiac tumors (lymphoma, sarcoma);
• mediastinal tumors;
• exudative-constrictive pericarditis;
• liver cirrhosis (systemic venous pressure is not elevated);
• inferior vena cava syndrome, nephrotic syndrome and other hypooncotic 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 more likely to be due to left ventricular aneurysm than to pericardial calcification.

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Treatment of chronic pericarditis

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

Indications for hospitalization

Hospitalization is indicated when invasive examinations and surgical treatment are necessary.

Conservative treatment of chronic pericarditis

Conservative treatment of chronic pericarditis is performed in cases of mild constriction, during preparation for surgery, or in inoperable patients. In addition, in individual patients with relatively acute recent onset of pericardial constriction, disappearance or reduction of symptoms and signs of constriction has been described with treatment with anti-inflammatory drugs, colchicine, and/or glucocorticoids.

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Non-drug treatment of chronic pericarditis

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

Drug treatment of chronic pericarditis

Diuretics (loop) for edema and ascites should preferably be used in minimally effective doses. It is necessary to avoid hypovolemia, arterial hypotension and renal hypoperfusion. Potassium-sparing diuretics are additionally used (under control of renal function and plasma potassium levels). Plasma ultrafiltration can improve the condition of patients with severe volume overload.

It is necessary to avoid prescribing beta-blockers or slow calcium channel blockers that reduce 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, which can lower blood pressure and cause renal hypoperfusion, should be used with caution and under monitoring of renal function.

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Surgical treatment of chronic pericarditis

Pericardiectomy with wide removal of visceral and parietal pericardium is the main method of treatment for severe chronic constriction. Complete disappearance of constrictive hemodynamic disorders after this operation is described in approximately 60% of patients. The operation is indicated for patients with CP with circulatory failure of the 2nd or 3rd functional class (MUNA). The operation is usually performed through a median sternotomy approach, in some cases a thoracoscopic approach is suitable. In purulent pericarditis, the preferred approach is through lateral thoracotomy. This operation with a significant surgical risk is not indicated for mild manifestations of constriction, severe calcification of the pericardium or its severe damage, severe myocardial fibrosis. The surgical risk is highest in elderly patients, in cases of disease associated with radiation, with severe manifestations of constriction, severe renal dysfunction, the presence of myocardial dysfunction.

Approximate periods of incapacity for work

In constrictive chronic pericarditis, work capacity is usually persistently reduced.

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Prognosis for chronic pericarditis

Surgical mortality during pericardiectomy for CP reaches 5-19% even in specialized institutions. Remote prognosis after pericardiectomy depends on the etiology of CP (better prognosis in idiopathic constrictive chronic pericarditis). If the indications for surgical treatment were established early, remote mortality after pericardiectomy corresponds to mortality in the general population. Mortality during pericardiectomy is most associated with myocardial fibrosis that was not recognized before surgery.