What is Pericarditis? | American Heart Association

Pericarditis is inflammation of the pericardium, a sac-like structure with two thin layers of tissue that surround the heart to hold it in place and help it work. A small amount of fluid keeps the layers separate so there’s less friction between them as the heart beats.

A common symptom of pericarditis is chest pain, caused by the sac’s layers becoming inflamed and possibly rubbing against the heart. It may feel like pain from a heart attack.

If you have chest pain, call 911 right away because you may be having a heart attack. Learn about warning signs for a heart attack.

Overview

Pericarditis can be attributed to several factors, including viral, bacterial, fungal and other infections. Other possible causes of pericarditis include heart attack or heart surgery, other medical conditions, injuries and medications.

Pericarditis can be acute, meaning it happens suddenly and typically doesn’t last long. Or the condition may be “chronic,” meaning that it develops over time and may take longer to treat.

Both types of pericarditis can disrupt your heart’s normal function. In rare cases, pericarditis can have very serious consequences, possibly leading to abnormal heart rhythm and death.

Outlook

Pericarditis is frequently mild and may clear up on its own with rest or simple treatment. Sometimes, more intense treatment is needed to prevent complications.

Recovery time from pericarditis may vary depending on the type of condition and the patient’s health. Consultation with a health care professional can determine this.

Other names for pericarditis

• Idiopathic pericarditis (no known cause)
• Acute fibrinous pericarditis and acute purulent pericarditis (forms of acute pericarditis)
• Chronic effusive pericarditis and chronic constrictive pericarditis (forms of chronic pericarditis)
• Recurrent pericarditis

Causes of pericarditis

The cause of pericarditis is often unknown, though viral infections are a common reason. Pericarditis may occur after a respiratory or digestive system infection.

Chronic and recurring pericarditis may be caused by autoimmune disorders such as lupus, scleroderma and rheumatoid arthritis. These are disorders in which the body’s immune system makes antibodies that mistakenly attack the body’s tissues or cells.

Other possible causes of pericarditis are:

• Heart attack and heart surgery
• Kidney failure, HIV/AIDS, cancer, tuberculosis and other health problems
• Injuries from accidents or radiation therapy
• Certain medicines, such as phenytoin (an anti-seizure medicine), warfarin and heparin (both blood-thinning medicines), and procainamide (a medicine to treat irregular heartbeats)

Who is at risk for pericarditis?

Pericarditis affects people of all ages, but men ages 16 to 65 are more likely to develop it.

Among those treated for acute pericarditis, up to 30% may experience the condition again, with a small number eventually developing chronic pericarditis.  

Written by American Heart Association editorial staff and reviewed by science and medicine advisors. See our editorial policies and staff.

Last Reviewed: Apr 8, 2021

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Continuing Education Activity

The pericardium is a rigid, avascular, fibrous sac. Its primary functions are anchoring, lubricating, preventing overdistention of cardiac chambers, and optimizing diastolic filling. Normally the pericardium does not contain calcium deposits; calcification may be a sign of underlying inflammation or a more sinister etiology. Pericardial calcification alone is generally asymptomatic; however, signs and symptoms usually result secondary to constrictive physiology caused by the rigid pericardium. This activity reviews the etiologies, evaluation, and treatment of pericardial calcification and addresses the role of the interprofessional team in the care of patients with this condition.

Objectives:

• Describe the etiologies of pericardial calcification.

• Review the management options for pericardial calcification.

• Identify the etiology-specific treatment options for pericardial calcification.

• Explain modalities to improve care coordination among interprofessional team members to improve patient outcomes for pericardial calcification.

Access free multiple choice questions on this topic.

Introduction

The normal pericardium is 1 to 2 mm thick and is comprised of an outer fibrous layer and an inner serous layer (which further subdivides into a visceral layer, or epicardium, and a parietal layer). A potential space that contains approximately 15 to 35 ml of lubrication fluid separates the visceral and parietal layers. The pericardium is a rigid, avascular, fibrous sac and its primary function is minor anchoring, lubrication, preventing distention of cardiac chambers, and optimizing diastolic filling.[1] Normally the pericardium lacks any calcium deposits, and calcification may be a sign of underlying inflammation or a more sinister etiology. Pericardial calcification is a radiological finding most often discovered while performing chest computed tomography (CT).[2] 

Often pericardial calcification is an incidental finding noted during chest or cardiac CT and as such remains asymptomatic. However, signs and symptoms associated with pericardial calcification usually develop secondary to constrictive physiology caused by the rigid pericardium. However, a significant point to keep in mind is that pericardial calcification may not be present in up to 20% of cases of constrictive pericarditis (CP), and it may be present in the absence of constrictive physiology. Interestingly, there have been recent reports of the development of CP after cardiac transplantation, an unusual presentation, as the transplanted heart is believed to be free of any pericardial tissue. [3][4]

Etiology

Pericardial calcification is the sequelae of inflammation, fibrosis, and necrosis. The most common etiologies of such inflammation include viral infections, chest radiation exposure, and following cardiac surgery.[5] Historically, tuberculosis had been a significant cause of CP in the US and accounted for almost half of the cases, but now it is found more commonly in developing nations.[2] Other known etiologies include uremic pericarditis, trauma, malignancy, rheumatologic and connective tissue diseases.[5] Pericardial calcification has also been reported following asbestos exposure.[6] Despite these known associations, however, more than 50% of cases of pericardial calcification are thought to be idiopathic.[7]  

Epidemiology

Given that pericardial calcification can be an asymptomatic incidental finding, its true prevalence is unknown. However, it is found to be more likely to occur following trauma, purulent pericarditis, and when acute pericarditis or pericardial effusions are associated with malignancy and connective tissue diseases. [8]

For idiopathic and viral constrictive pericarditis, an incidence of 0.76 cases per 1000 person-years has been reported in a series of 500 consecutive patients; this contrasts with the connective tissue disease, neoplasms, tuberculosis, and purulent CP in which the incidence was noted to be 4.40, 6.33, 31.65, and 52.74 cases per 1000 person-years, respectively.[9]

Tuberculous pericarditis, caused by Mycobacterium tuberculosis, is found in approximately 1% of all autopsied cases of TB and 1 to 2% of instances of pulmonary TB. Most constrictive tuberculosis caused by TB is subacute so calcifications of the pericardium are rare.[10] 

A study by Ling et al. suggests that calcified CP is more common in males.[6]

Pathophysiology

Varying degrees of pericardial calcification are present in about 80 to 98% of the cases of CP.[2][6] These changes typically occur over a period of months to years after the initial insult. Pericardial calcification in CP may or may not be associated with pericardial thickening, and about 18% of CP cases have no pericardial thickening. [8] Constrictive pericarditis occurs when a normal thin compliant pericardium is replaced by a thick, calcified, non-compliant pericardium that interferes with ventricular filling, which in turn lowers cardiac output.[6]

History and Physical

Pericardial calcification is generally asymptomatic; symptoms usually develop with cardiac hemodynamic compromise such as in the setting of constrictive pericarditis. Symptoms of right heart failure or low output states such as dyspnea on exertion, orthopnea, bendopnea, and fatigue may develop. Of these, exertional dyspnea and lower extremity edema are the most common findings.[11] Physical examination findings include hepatomegaly, ascites, peripheral edema (usually bilateral), hepatojugular reflux, Kussmaul’s sign, jugular venous distention (with prominent x and y descents), and pulsus paradoxus. In advanced disease, there can be cachexia.[8] Atrial arrhythmias namely atrial fibrillation and flutter have been noted to be more common in CP with pericardial calcification.  [6]

Evaluation

Pericardial calcification is an anatomical finding with significance in the presence of constrictive physiology. This is why imaging for the same is two-pronged, involving the identification of calcification in the pericardium and hemodynamic consequences of the same.[12] Chest radiography may identify pericardial calcification; however, it suffers from low sensitivity. The laboratory workup may show an elevation of liver enzymes (especially alkaline phosphatase) and serum creatinine. In addition, B-type natriuretic peptide and N terminal pro-brain natriuretic peptide are frequently used to differentiate CP from restrictive cardiomyopathy.[13] 

CT provides excellent anatomic details of the heart and the pericardium. The normal pericardial thickness on the CT scan should be less than 2 mm.[14] Although the pericardial thickness of more than 4 mm on CT scan is predictive of constrictive pericarditis, up to 20% of patients with normal pericardial thickness can have evidence of pericardial calcification and develop CP. [15] In a cohort of patients with CP and pericardial calcification, calcification was noted to more commonly involve the diaphragmatic and anterior portions of the pericardium, with apical and left atrial involvement being found less commonly.[6] However, CT suffers from lower temporal resolution compared to echocardiography and cardiac magnetic resonance imaging (MRI) and cannot reliably demonstrate constrictive physiology.[16] In addition, pericardial calcification has also been demonstrated on 99m Tc-methylene diphosphonate bone scintigraphy, which can be another useful imaging option to help visualize pericardial calcification where CT imaging may be challenging.[17]  

To evaluate the functional significance of pericardial calcification, two-dimensional (2-D) echocardiography and tissue Doppler imaging are probably the essential tools for diagnosing CP. 2-D echocardiography demonstrates septal motion abnormalities, enhanced interventricular interdependence, and plethoric inferior vena cava. Tissue Doppler of the mitral annulus reveals increased medial early diastolic velocities (e’).[7] In healthy individuals, mitral lateral e’ velocity is usually greater than the medial e’ velocity, but in CP, due to the tethering effect of the lateral annulus to the surrounding calcified pericardium, this velocity becomes lower, a phenomenon called ‘annulus reversus’ and a hallmark feature of CP.[7] Cardiac MRI can be utilized not only to delineate cardiac anatomy and pericardial thickness, but it can also provide information on ventricular septal motion demonstrating the so-called ‘septal bounce’ and ‘septal shudder.'[7] 

While cardiac MRI is not as sensitive as CT in identifying pericardial calcification, it is a useful tool in assessing the extent of edema and scarring, which helps understand the progression of the disease and predict which patients are more likely to develop CP.[18] An invasive hemodynamic assessment with cardiac catheterization is possible in some cases where the diagnosis is unclear. Typically, an equalization of end-diastolic pressures in all four cardiac chambers is noted. Furthermore, with the ‘match-up’ study, by placing high fidelity catheters in the right and left ventricle and recording pressures simultaneously, discordant respirophasic changes in the ventricular filling patterns are noted (a catheterization equivalent of ventricular interdependence, in which one ventricle fills at the expense of the other depending on the respiratory cycle.[19]

In the past, an endomyocardial biopsy was also used to differentiate CP from restrictive cardiomyopathy, although it was found to have a low yield.[8] 

Treatment / Management

Pericardial calcification in the absence of symptoms does not require any treatment. Subacute cases of constrictive pericarditis with underlying inflammation may respond to anti-inflammatory therapy (such as colchicine, corticosteroids, and non-steroidal anti-inflammatory drugs).[4][20] However, anti-inflammatory agents are usually unable to reverse fibrosis and calcification but are mainly meant to decrease the progression of inflammation and further scarring. Cardiac MRI can often help concurrently visualize scar and edema to determine which patients are more likely to benefit from anti-inflammatory therapy.[21] 

Diuretics have been used in the early stages of constrictive pericarditis to improve pulmonary and systemic congestion. These must be used cautiously because any drop in intravascular volume may cause a corresponding drop in cardiac output.[22] Surgical pericardiectomy is the gold standard for CP and is potentially curative.[20] Pericardiectomy usually has a high success rate (70-80%) but also has a high perioperative mortality rate of 5 to 10%. Perioperative cardiovascular outcomes are usually influenced by New York Heart Association heart failure class, with NYHA Class I and II heart failure patients having better postoperative outcomes than NYHA Class III and IV heart failure. The presence and extent of pericardial calcification usually do not affect perioperative outcomes.[23] 

Differential Diagnosis

Constrictive pericarditis must be ruled out when pericardial calcification is present on imaging modalities. Unusual forms of CP such as transient constriction (which responds to anti-inflammatory therapy) and effusive constrictive pericarditis (developing in patients with cardiac tamponade and developing immediately after pericardiocentesis), or occult constrictive pericarditis (which manifests after a fluid challenge) must be ruled out as well [7]. Other differentials of calcification of pericardium include pericardial scarring due to recurrent pericarditis. Additionally, mimickers of CP include restrictive cardiomyopathy (which occurs due to abnormal myocardium), and severe tricuspid regurgitation must be ruled out.[8] 

Prognosis

The presence of pericardial calcification and its extent by itself does not affect prognosis. Patients with poor cardiovascular outcomes after pericardectomy were those that developed long-term sequelae of constrictive physiology, including myocardial atrophy, or had concurrent cardiomyopathy. Prognosis is worse for patients diagnosed with NYHA Class III and IV heart failure as they have higher operative mortality. [23]

Long-term survival after pericardiectomy for constrictive pericarditis depends on the underlying etiology. In a cohort of 163 patients, idiopathic CP had the best 7-year survival (88%), followed by postsurgical (66%) and post-radiation CP (27%).[24] Patients with advanced age, prior radiation exposure, pulmonary hypertension, renal dysfunction, and liver failure also had poor outcomes despite pericardectomy.[8][25] 

Complications

Pericardial calcification in the presence of symptoms should be evaluated further as constrictive pericarditis, if present, could be potentially curative. Without surgical pericardiectomy, constrictive pericarditis portends a very poor prognosis due to complications associated with heart failure and the low output state. Renal failure, organomegaly, shock, and death are other potential complications.[23]

Deterrence and Patient Education

Early recognition and diagnosis of pericardial calcification and ruling out constrictive pericarditis is imperative to ensure timely management and optimal clinical outcomes. This diagnosis is often an incidental finding. The increased use of cardiac CT for calcium scoring has led to the detection of pericardial calcifications in many asymptomatic patients.

Enhancing Healthcare Team Outcomes

Pericardial calcification in the absence of symptoms is a benign condition. However, constrictive pericarditis must be ruled out in such patients. Diagnosis of CP can be challenging and requires multimodal imaging with an interprofessional approach involving invasive and non-invasive cardiologists, radiologists, and cardiothoracic surgeons. The condition is often identified by primary care and emergency providers. CP carries a grave prognosis if missed, and most patients die with medical management alone. Pericardiectomy is potentially curative and carries acceptable mid and long-term outcomes depending on the etiology. Coordination of evaluation by the team will lead to improved outcomes. [Level 5]

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Figure

S/P CABG pericardial calcification. Image courtesy S Bhimji MD

Figure

Pericardial calcification and pericardial thickening (maximum anterior thickness 2.2 cm) in a patient with constrictive pericarditis. Contributed by Kifah Hussain, MD

References

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Umer A, Khalid N, Chhabra L, Spodick DH. Role of Pericardiectomy in Postcardiac Transplant Constrictive Pericarditis. Ann Thorac Surg. 2015 Dec;100(6):2420. [PubMed: 26652564]

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Umer A, Khalid N, Chhabra L, Memon S, Spodick DH. Constrictive pericarditis complicating cardiac transplantation. J Cardiothorac Surg. 2015 Aug 25;10:109. [PMC free article: PMC4549104] [PubMed: 26302865]

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Cameron J, Oesterle SN, Baldwin JC, Hancock EW. The etiologic spectrum of constrictive pericarditis. Am Heart J. 1987 Feb;113(2 Pt 1):354-60. [PubMed: 3812191]

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Ling LH, Oh JK, Breen JF, Schaff HV, Danielson GK, Mahoney DW, Seward JB, Tajik AJ. Calcific constrictive pericarditis: is it still with us? Ann Intern Med. 2000 Mar 21;132(6):444-50. [PubMed: 10733443]

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Miranda WR, Oh JK. Constrictive Pericarditis: A Practical Clinical Approach. Prog Cardiovasc Dis. 2017 Jan-Feb;59(4):369-379. [PubMed: 28062267]

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Welch TD, Oh JK. Constrictive Pericarditis. Cardiol Clin. 2017 Nov;35(4):539-549. [PubMed: 29025545]

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Imazio M, Brucato A, Maestroni S, Cumetti D, Belli R, Trinchero R, Adler Y. Risk of constrictive pericarditis after acute pericarditis. Circulation. 2011 Sep 13;124(11):1270-5. [PubMed: 21844077]

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Strang JI, Kakaza HH, Gibson DG, Girling DJ, Nunn AJ, Fox W. Controlled trial of prednisolone as adjuvant in treatment of tuberculous constrictive pericarditis in Transkei. Lancet. 1987 Dec 19;2(8573):1418-22. [PubMed: 2891992]

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Ling LH, Oh JK, Schaff HV, Danielson GK, Mahoney DW, Seward JB, Tajik AJ. Constrictive pericarditis in the modern era: evolving clinical spectrum and impact on outcome after pericardiectomy. Circulation. 1999 Sep 28;100(13):1380-6. [PubMed: 10500037]

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Kim JS, Kim HH, Yoon Y. Imaging of pericardial diseases. Clin Radiol. 2007 Jul;62(7):626-31. [PubMed: 17556030]

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Diaz-Arocutipa C, Saucedo-Chinchay J, Imazio M, Argulian E. Natriuretic peptides to differentiate constrictive pericarditis and restrictive cardiomyopathy: A systematic review and meta-analysis. Clin Cardiol. 2022 Mar;45(3):251-257. [PMC free article: PMC8922532] [PubMed: 34967020]

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Bull RK, Edwards PD, Dixon AK. CT dimensions of the normal pericardium. Br J Radiol. 1998 Sep;71(849):923-5. [PubMed: 10195005]

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Talreja DR, Edwards WD, Danielson GK, Schaff HV, Tajik AJ, Tazelaar HD, Breen JF, Oh JK. Constrictive pericarditis in 26 patients with histologically normal pericardial thickness. Circulation. 2003 Oct 14;108(15):1852-7. [PubMed: 14517161]

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Wang ZJ, Reddy GP, Gotway MB, Yeh BM, Hetts SW, Higgins CB. CT and MR imaging of pericardial disease. Radiographics. 2003 Oct;23 Spec No:S167-80. [PubMed: 14557510]

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Burns N, Shriki JE, Farvid AM, El-Sherief KE, Cunningham MJ, Colletti PM, Shinbane J. Calcific constrictive pericarditis demonstrated on 99mTc-MDP bone scintigraphy. J Radiol Case Rep. 2009;3(5):11-5. [PMC free article: PMC3303308] [PubMed: 22470658]

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Al-Mallah MH, Almasoudi F, Ebid M, Ahmed AM, Jamiel A. Multimodality Imaging of Pericardial Diseases. Curr Treat Options Cardiovasc Med. 2017 Oct 12;19(12):89. [PubMed: 29027095]

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Talreja DR, Nishimura RA, Oh JK, Holmes DR. Constrictive pericarditis in the modern era: novel criteria for diagnosis in the cardiac catheterization laboratory. J Am Coll Cardiol. 2008 Jan 22;51(3):315-9. [PubMed: 18206742]

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Haley JH, Tajik AJ, Danielson GK, Schaff HV, Mulvagh SL, Oh JK. Transient constrictive pericarditis: causes and natural history. J Am Coll Cardiol. 2004 Jan 21;43(2):271-5. [PubMed: 14736448]

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Inglessis I, Dec GW. Constrictive Pericarditis. Curr Treat Options Cardiovasc Med. 1999 Jun;1(1):63-71. [PubMed: 11096470]

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Depboylu BC, Mootoosamy P, Vistarini N, Testuz A, El-Hamamsy I, Cikirikcioglu M. Surgical Treatment of Constrictive Pericarditis. Tex Heart Inst J. 2017 Apr;44(2):101-106. [PMC free article: PMC5408622] [PubMed: 28461794]

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Bertog SC, Thambidorai SK, Parakh K, Schoenhagen P, Ozduran V, Houghtaling PL, Lytle BW, Blackstone EH, Lauer MS, Klein AL. Constrictive pericarditis: etiology and cause-specific survival after pericardiectomy. J Am Coll Cardiol. 2004 Apr 21;43(8):1445-52. [PubMed: 15093882]

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Disclosure: Nauman Khalid declares no relevant financial relationships with ineligible companies.

Disclosure: Kifah Hussain declares no relevant financial relationships with ineligible companies.

Disclosure: Evan Shlofmitz declares no relevant financial relationships with ineligible companies.

Primary synovial pericardial sarcoma

Primary synovial pericardial sarcoma is an extremely rare disease and accounts for about 13% of all primary cardiac sarcomas. Among them, the most common histological form is angiosarcoma, followed by undifferentiated and leiomyosarcoma. The most common benign lesions are cysts and lipomas of the pericardium, while mesothelioma is the most common primary malignant pericardial tumor. Currently, including clinical observation, no more than 20 cases of primary synovial pericardial sarcoma can be found in the world English-language literature [2–5]. This pathology is most often described in men in the age group from 30 to 50 years [6]. In most cases, the prognosis in patients with primary synovial pericardial sarcoma is extremely unfavorable [1, 7], although in 2013 X. Wu et al. [8] described a clinical observation of a patient who survived 32 months after surgical treatment followed by adjuvant chemotherapy. The optimal treatment strategy for this group of patients has not yet been determined due to the extreme rarity of the disease and its extremely aggressive course. Median survival is 25 months [9, 10]. According to published works, total or subtotal excision of the tumor is possible only in 17-22.7% of cases. In the adjuvant regimen, it is recommended to perform chemotherapy or radiation therapy on the area of ​​residual tumor or newly occurring recurrence [9–12].

The most common clinical picture of primary pericardial sarcoma includes: shortness of breath, signs of cardiac tamponade and / or systemic manifestations (fever, chills, general weakness and weight loss) [1, 13]. For the purpose of diagnosis, radiography, echocardiography, MSCT of the chest organs are used, with the help of which cardiomegaly is visualized due to pericardial effusion with the presence or absence of a solid tumor component of various sizes.

Here is a clinical observation

Patient F ., 44 years old, in December 2015 for the first time noted difficulty in breathing and pain behind the sternum, and therefore turned to the clinic at the place of residence. The neuropathologist regarded these phenomena as osteochondrosis and recommended physiotherapy. In February 2016, due to syncope, he was urgently hospitalized at the hospital at the place of residence. Upon admission, a plain chest X-ray was performed, which revealed a significant expansion of the shadow of the heart (Fig. 1). Rice. 1. Plain radiograph of the chest of the patient in the supine position upon admission to the hospital.

Echocardiography revealed a significant amount of fluid in the pericardial cavity (Fig. 2). Rice. 2. Echocardiography upon admission to the hospital at the place of residence.

Pericardiocentesis was performed under the control of an ultrasound sensor, a catheter was placed in the pericardial cavity, about 1000 ml of serous hemorrhagic fluid was evacuated. Cytological examination identified mesothelial cells, tumor elements were not detected. After stabilization of the patient’s condition, as an additional examination, MSCT of the chest organs was performed with intravenous contrast, which revealed an additional volumetric soft tissue formation in the anterior pericardium on the right with a density of up to 48 units. Hu, transverse dimension up to 7.92 × 4.16 cm, length 8.86 cm, poorly accumulating contrast – in the arterial phase up to 53 units. Hu, in the venous up to 63 units. Hu, delayed up to 50 units. Hu. In the thickness of the pericardium, along the periphery of the formation, air bubbles are visualized. A catheter is visualized in the anterior pericardium. The fiber of the mediastinum is heterogeneously compacted, contains multiple dense lymph nodes (para-aortic, paratracheal and bifurcation groups) up to 10 mm in size in a short diameter. The heart is somewhat displaced posteriorly and to the left (volumetric formation of the pericardium), its configuration is not changed. The chambers of the heart are not dilated (Fig. 3). Rice. 3. Computed tomogram. Formation of the anterior mediastinum.

After stabilization of the patient’s condition, he was referred to the thoracic surgical department of the MNII named after. P.A. Herzen. He was additionally examined at the institute: no other focal pathology in organs and systems was obtained. Ultrasound of the chest organs (in the anterior mediastinum, more to the right, the formation of a soft elastic consistency of 75 mm (length) × 84 mm (transverse size) × 47 mm (thickness) is determined. The formation has intra- and extrapericardial components. In the pericardial cavity and in the pleural fluid is not visualized in the cavities.In the central part of the formation there is a defect in the pericardium up to 45 × 50 mm.Other structures of the mediastinum are not involved in the process.Conclusion: an echographic picture of a mediastinal neoplasm involving the pericardium (originating from the pericardium?) with the presence of intra- and extrapericardial components ( fatty formation? formation of a different nature?).

At the consultation, taking into account the CT picture of the disease, the absence of morphological verification and distant metastases, as well as the satisfactory condition of the cardiorespiratory system, surgical treatment was recommended.

According to the decision of the council, a complete longitudinal sternotomy was performed, the tumor of the anterior mediastinum was removed with resection of the pericardium (Fig. 4). Rice. 4. Intraoperative view after full longitudinal sternotomy.

Intraoperative revision: in the anterior mediastinum, mainly in the pericardial cavity, a tumor-like formation of a soft consistency, 6 × 7 × 6 cm in size, is determined. There are no signs of invasion of the main vessels and lung tissue. The pericardium was opened (Fig. 5, a). Rice. 5. Stages of the operation. a — intraoperative view after opening the pericardium; b — tumor tissue on the resected fragment of the pericardium; c — macropreparation. A fragment of the tumor tissue was sent for urgent morphological examination — a spindle cell formation. In a blunt and sharp way, the tumor tissue with the surrounding tissue of the anterior mediastinum and the anterior wall of the pericardium in the area of ​​10×10 cm is separated from the superior vena cava and brachiocephalic veins, the brachiocephalic trunk; at the same time, both pleural cavities were opened, the phrenic nerves were traced and preserved on both sides (see Fig. 5, b). Highlighted, ligated and crossed the upper horns of the thymus. The drug was removed as a single block (see Fig. 5c; Fig. 6). Rice. 6. The final view of the surgical wound after removal of the tumor preparation. One drainage was left in the pleural cavities, the anterior mediastinum was drained separately.

Postoperative period without complications. The patient was transferred from the intensive care unit and intensive care on the 1st day after surgery. The drains were removed on the 2nd day after the operation. At the control radiography on the 3rd postoperative day there are no infiltrative shadows, air, fluid in the pleural cavities.

According to the data of a planned histological examination of the surgical material, the macropreparation is described as a fragment of adipose tissue measuring 15 × 7 × 3.5 cm, with areas of pericardial tissue measuring 7 × 2.5 cm. color, 7 × 6 × 3 cm in size, on the section, a tumor node in its own capsule (torn) of a variegated appearance, lobed structure, yellowish-red-gray in color, 6. 5 × 5.2 × 3 cm in size. Microscopic picture: mesenchymal tumor, consisting of spindle-shaped cells that form bundles going in different directions, with foci of necrosis, hemorrhages, the formation of cystic cavities lined with a flattened cuboidal epithelium, deposition of brown pigment in a thickened fibrous tumor capsule. Mitotic activity 11 mitoses per 10 fields of view (×400). The tumor capsule is intimately soldered to the pericardium and thymus tissue. There are no metastases in the lymph nodes, lymphoid hyperplasia, epithelial giant cell granuloma with deposition of brown pigment. Conclusion: poorly differentiated spindle cell sarcoma (Fig. 7). Rice. 7. Poorly differentiated spindle cell sarcoma. Stained with hematoxylin and eosin. ×100.

Immunohistochemical examination of tumor cells expresses: Vimentin – positive expression in tumor cells; ЕМА — positive expression in some cells; SMA, positive expression in some cells; Desmin, negative expression in tumor cells; CD68 – positive expression in some cells; S100, negative expression in tumor cells; BCC — positive expression in some cells and cyst lining; p63, negative expression in tumor cells; CD45 positive expression in some cells (lymphoid cells) (Fig. 8). Rice. 8. IHC micropreparation of the tumor. a — Vimentin, b — BCC, c — CD99, d — CD45. ×100.

Conclusion: the morphological and immunohistochemical picture does not exclude synovial sarcoma, it is necessary to conduct a molecular genetic study.

To confirm the diagnosis of primary synovial pericardial sarcoma, a FISH reaction was performed on histological slides of the tumor preparation: a rearrangement of the SS18 (18q11.2) gene was detected, which clearly indicates synovial sarcoma (Fig. 9). Rice. 9. FISH reaction on histological slides of the tumor preparation, confirming the diagnosis of primary synovial pericardial sarcoma.

At the postoperative consultation, taking into account the unfavorable prognosis of the disease, chemotherapy was recommended according to the scheme: doxorubicin 75 mg/m ² as a 72-hour infusion + ifosfamide 2.5 g/m ² as a 3-hour infusion 1, 2 , 3, 4 days + mesna uromitexan 500 mg/m ² on day 1 together with ifosfamide, then 1500 mg/m ² as a 24-hour infusion for 4 days, 3 weeks interval. After 3 courses of polychemotherapy, external radiation therapy is recommended, followed by 3 courses of PCT according to the previous scheme.

Polychemotherapy according to the recommended scheme was carried out in the conditions of the oncological dispensary at the place of residence. Despite the ongoing treatment, the progression of the disease in the form of a local recurrence was noted, followed by a lethal outcome, which was recorded 10 months after surgical treatment.

The name “synovial sarcoma” is historical, since the association of the tumor with the synovial membrane is rejected. Synovial sarcoma is a tumor that is defined in terms of genetic disorders, in particular a specific translocation between chromosome 18 and the female sex chromosome t (X;18) (p11;q11). Gene 9 is involved in the translocation0013 SYT , localized on the long arm of chromosome 18, and genes SSX1 (variants SSX2, SSX4 ), which are representatives of the family of genes located on the X chromosome and differ from each other by the break point. The fused chimeric gene formed during translocation encodes a protein that is a transcriptional regulator and activates proto-oncogenes or inhibits suppressors. Clinical and biological properties of synovial sarcoma are determined by the product of the newly formed chimeric gene. To determine the gene translocation characteristic of synovial sarcoma SYT , the FISH reaction was carried out using a fluorescent probe from Abbott-Vysis (Fig. 10, a,). Rice. 10. FISH reaction. a — fluorescent probe from Abbott-Vysis; b — SYT gene translocation. The reaction was carried out on thin sections. A positive result was defined as one continuous yellow signal and two separate (red and green) signals (Fig. 10b).

For morphological verification, a biopsy of the neoplasm of the pericardium is indicated. To confirm the diagnosis of primary synovial pericardial sarcoma, a FISH reaction is mandatory [14, 15].

If idiopathic pericarditis is suspected as the cause of recurrent non-hemorrhagic pericardial effusion with a rapid rate of accumulation, the rare malignant pericardial pathology described in this paper should be included in the differential diagnosis without fail.

The authors declare no conflict of interest.

Information about authors

Barmin Vitaliy Valerievich collaborator thoracic department; e-mail: [email protected];

Diseases of the heart of animals. Diagnosis of heart disease in dogs and cats.

Heart disease occurs not only in humans, but also in animals, especially in dogs and cats. Which heart conditions are most common in dogs and which are most common in cats? What symptoms are alarming because they can show the pathology of your pet’s heart? What should pet owners pay attention to, and what examination should be done in order to diagnose heart disease in time?

After all, the sooner you take action, the greater the chance of restoring your pet’s health. In this article we will talk about it, and if you decide to examine your animal in Almaty, we will be ready to help.

Heart disease can occur during the life of an animal at any age! Also, cardiac pathologies can be congenital.

Congenital heart diseases (malformations) are most often diagnosed in young animals. Here is a list of such heart diseases:

• Aortic stenosis (narrowing of the vessel lumen).
• Stenosis of the mouth of the pulmonary artery.
• Non-occlusion of the Botal’s duct (open ductus arteriosus).
• Ventricular septal defects (VSD).
• Atrial septal defects.
• Non-occlusion of the right aortic arch.
• Tetralogy of Fallot (malformed structures of the heart).
• Dysplasia (tissue growth) of the mitral valve.

Characteristic symptoms of these pathologies are slow growth, fatigue, poor appetite, shortness of breath, cough, pallor or blueness of the mucous membranes.

Acquired diseases are very diverse. The pathological process may involve the heart muscle (myocardium), the connective tissue membrane of the heart (pericardium), the inner lining of the heart (endocardium), and heart valves. Not uncommon – the formation of tumors of various parts of the heart.

The incidence of heart disease is not the same in dogs and cats. In dogs, common heart conditions are :

• Endocardiosis (valvular heart disease common in small breed dogs).
• Dilated cardiomyopathy (DCM – a group of diseases of the heart muscle, in which the size of the ventricles of the heart increase (expand), but at the same time they lose the ability to pump the necessary volume of blood).
• Myocarditis (inflammation of the muscle tissue of the heart – myocardium).
• Pericarditis (inflammation of the outer lining of the heart).

One of the most common heart diseases in dogs is endocardiosis . This disease usually develops in the second half of a dog’s life and is almost never found in young animals. With endocardiosis, the valves of the heart gradually thicken and deform, cease to close tightly and begin to pass blood in the opposite direction.

Most often, this disease affects dogs of the so-called risk group, these are dwarf dog breeds: Pekingese, Toy Terriers, Chihuahuas, Spitz, Yorkshire Terriers, Poodles, Dachshunds, Spaniels, Pugs, French Bulldogs. Less commonly, valve disease occurs in large dogs, especially the terrier group, but, as a rule, the disease is not aggressive and rarely leads to serious consequences. With regard to dwarf breeds, there is a definite trend: the smaller the weight of the dog, the more common endocardiosis, and as a rule, it is more severe. This must be taken into account if you decide to have a small dog, you must definitely pay attention to her health.

Dilated cardiomyopathy is a pathology of the heart muscle, which in most cases has a hereditary nature. Dogs of the Doberman Pinscher, Great Dane, Boxer, Irish Wolfhound, Newfoundland, American and English Cocker Spaniel breeds are most predisposed to this disease, although it can also be detected in dogs of other breeds (often giants). This disease is very rare in cats.

This disease has a poor prognosis, and early diagnosis is especially important here! Examination of the dog’s heart and early detection of dilated cardiomyopathy allows timely treatment and observation of the animal’s condition. In addition, the earliest possible diagnosis allows you to exclude a sick animal from pedigree breeding.

Myocarditis – inflammation of the heart muscle (myocardium), which leads to the death of muscle fiber cells, increased excitability and decreased contractility of the myocardium, the development of extrasystoles – heartbeats outside the healthy rhythm of the heart (arrhythmias). Myocarditis can be focal (inflammation in specific foci of the heart muscle) and diffuse (throughout myocardial tissue), acute and chronic.

Myocarditis is most often a complication of infectious and parasitic diseases in animals. Also, myocarditis can have an autoimmune origin (associated with the pathology of the immune system), and occur with poisoning with arsenic, phosphorus and organochlorine compounds, with blood poisoning, rheumatic diseases, and metabolic disorders.

Pericarditis is an inflammation of the outer shell of the heart (pericardium, cardiac sac), it can be non-traumatic and traumatic. Non-traumatic pericarditis occurs as a complication of infectious diseases due to subcutaneous abscesses and blood poisoning, the transfer of inflammation from the heart muscle, lungs, pleura, etc. Dirofilariasis is transmitted from mosquitoes to animals and humans. Dirofilaria live, multiply and circulate constantly in the bloodstream, heart, subcutaneous tissue and muscles. These are viviparous organisms, their larvae – microfilariae of the 1st stage, can circulate in the blood of an infected dog for up to 2.5 years without causing any symptoms. If such a dog is bitten by a mosquito, the larva in it goes through the next stages of development in 2-3 weeks and becomes capable of infecting the next organism. When bitten by an infected mosquito, such larvae enter the subcutaneous tissue of the dog, where they mature further. Larvae of the 5th stage penetrate into the blood vessels, are brought into the heart and large vessels by the blood flow, and after 6 months become adult sexually mature helminths, can reach 35 cm in length, causing harm to organs and tissues.

The danger of this disease lies in the acute blockage of the heart vessels by adult worms and the instant death of the animal!

How can you protect your pet from this dangerous disease? Mosquito and other insect repellents and regular heart checks are recommended for giant and large breed dogs after 3 years of age, and for small breed dogs after 6 years of age.

Unlike cats, symptoms of heart disease in dogs are clearly symptomatic and are expressed by:

• Shortness of breath, heavy breathing.
• Cough (after exertion).
• Fatigue.
• Enlargement of the abdomen (ascites).
• Edema in the dewlap, front paws.
• Paleness or bluishness of the mucous membranes.

Dog owners, if their pets show these symptoms, are strongly advised to examine the animals! Ultrasound of the heart, as well as examination for the presence of parasites and common infections, you can get in our Diagnostic Center for Animals VIVAT. The earlier the disease is detected, the greater the chance of curing the animal!

What about heart disease in cats? Please note that heart disease is different in dogs and cats. Signs of heart disease in cats may appear with severe stress or exercise. Their breathing quickens, sometimes shortness of breath with an open mouth is manifested. But more often in cats, heart disease occurs without symptoms, due to an inactive lifestyle, since a cat spends most of the day in a dream, and sudden death can occur.

Cats with heart disease become lethargic and passive. Certain feline heart conditions can cause complications such as pelvic palsy and blindness. Such acquired diseases, for example, include:

• Hypertrophic cardiomyopathy (HCM) is a thickening of the serucius muscle.
• Dilated cardiomyopathy (DCM).
• Restrictive cardiomyopathy (RCMP).
• Arrhythmogenic dysplasia/cardiomyopathy of the right ventricle (ARVC).
• Pericarditis and myocarditis can also occur in cats.

In cats, the most common hypertrophic cardiomyopathy is a disease in which the heart muscle (myocardium) becomes abnormally thick or hypertrophied. This thickening of the heart muscle greatly complicates the work of the heart to pump blood. Hypertrophic cardiomyopathy can also negatively affect the electrical system of the heart. This disease often goes undiagnosed as many cats are asymptomatic.

Some pedigree cats have a predisposition to hypertrophic cardiomyopathy (HCM), this applies to breeds such as: British Shorthair, Scottish Fold, Maine Coon, Ragdoll, Sphynx, Abyssinian, Oriental cat. Now in many veterinary clinics, these breeds are examined (including preoperative) in order to detect heart diseases early.

It is worth noting that coughing in cats is not a symptom of heart disease, in this case, one should rather think of diseases of the upper respiratory tract, stomach stones from wool, or less often – inflammation of the lungs. But if a cat breathes with its mouth open like a dog, heart disease is more likely to be present .

Other heart diseases are rare in cats.

Important for cat owners! Thromboembolism is a syndrome of acute circulatory disorders as a result of blockage of an artery by a thrombus. Most often, arterial thromboembolism occurs in an apparently healthy animal with chronic heart disease as a result of cardiomyopathy. However, in some cases, the underlying condition of thromboembolism cannot be established.

The diagnosis is usually made on the basis of a medical history and clinical examination, but the presence of a blood clot itself can be confirmed directly with the help of special equipment. Clinical signs are usually seen in predisposed cats around 5 years of age, and in others over 10 years of age. Cats are much more likely to get sick than cats and may show symptoms as early as 1 year of age.

Thromboembolism appears suddenly and develops very quickly. This is due to the bleeding of tissues near the site of thrombosis and depends on the localization of the thrombus and the degree of blockage of the vessels. The most common blockage of the largest artery in the body is aortic thromboembolism (more than 90% of cases), but blockage of other areas of the aorta and arteries may also occur. With classical thrombosis in the area of ​​division of the abdominal aorta into 2 vessels, the diagnosis can be established only on the basis of 5 clinical signs: pain, paralysis, absence of pulse, cold and pale limb.

Restrictive cardiomyopathy – damage to the heart muscle (myocardium) with the formation of infiltrates (accumulations of cellular material mixed with blood and lymph) or scar tissue, which is characterized by hardened, unyielding walls of the ventricles of the heart, a decrease in filling and diastolic volume (between heartbeats) of one or both ventricles with normal or almost unchanged systolic (during heart contraction) function and wall thickness. The disease is based on widespread interstitial fibrosis (pulmonary fibrosis).

Arrhythmogenic right ventricular cardiomyopathy . A feature of this disease is the development of life-threatening arrhythmias. This disease is characterized by the replacement of myocardial muscle tissue with fat cells and connective (fibrous) tissue cells. This pathology refers to genetically inherited diseases.

Diagnosis in cardiac pathology is a multi-stage process and is based on the results of a comprehensive examination. The first stage of diagnostic work is the collection of anamnesis (history of life and illness), examination of the animal and listening to its heart. None of even the most modern apparatus can replace the eyes, hands and head of a doctor. This is followed by an x-ray of the chest, which provides invaluable information about the structure of the airways, lungs, blood vessels and heart. This is a priority method for assessing the nature of the pathological process of the lungs and pulmonary circulation (detection and dynamic monitoring of the degree of pulmonary edema).