Aetiology

The transmission is mostly autosomal dominant. Less frequently the transmission is autosomal recessive and associated with palmoplantar keratoderma and woolly hair (Naxos and Carvajal syndromes).

Currently 16 genes, whose mutations are responsible for the disease, have been identified. The most frequently detected pathogenetic mutations affect 5 genes encoding for desmosomes, structures involved in cell-to-cell adhesion.

Epidemiology

• The prevalence is reported as between 1 in 2000 and 1 in 5000.
• Males are most affected, with a 3:1 ratio.
• The onset of disease occurs at young age, between the second and fourth decade; it is one of the main causes of SCD in young adults and athletes.
• First-degree family members could be mutation carriers, therefore a clinical screening with ECG and echocardiogram starting from 10 to 12 years of age is recommended and, even if negative, must be repeated with individualized timing of re-evaluation.

Pathophysiology

Macroscopically, the typical structural changes in ARVC include RV dilatation with associated RV myocardial thinning and fibro-fatty infiltration; the focal fibro-fatty deposits are the arrhythmogenic substrate that is a hallmark of ARVC.

Left ventricular involvement can be a late feature or an isolated form of the disease.

Particular sites involved include the RV apex, inflow tract, and outflow tract. With disease progression, further involvement of the RV free wall leads to aneurysms and RV cavity dilatation.

Symptoms

Patients are often asymptomatic, but symptoms of palpitations, pre-syncope and syncope can occur and may be precipitated by exercise. 

Ventricular arrhythmias (i.e. premature ventricular contractions, non-sustained ventricular tachycardia) can occur, together with major and potentially fatal arrhythmias, also during exercise.

The most typical arrhythmias in ARVC have a left bundle branch block morphology, with negative QRS in the right precordial leads and vertical axis on the frontal plane.

The natural history of ARVC may be separated into a number of distinct phases with progressive development of symptoms and structural abnormalities:

• concealed phase: a subclinical asymptomatic phase with little or no structural RV abnormality. Sudden CD may still occur in this stage of disease;
• overt electrical disorder: typically, with symptomatic ventricular arrhythmias of RV origin. Arrhythmia may vary from premature ventricular beats to non-sustained ventricular tachycardia to ventricular fibrillation. Structurally, there is clear evidence of RV wall involvement;
• RV failure: progressive loss of RV myocardium due to fi bro-fatty replacement impairs RV function and may result in severe pump failure. Gross dilatation of the RV occurs;
• biventricular failure: an advanced stage with involvement of the interventricular septum and LV causing CCF and imitating DCM.

Physical examination

Accurate individual and familiar history, clinical examination, first/second level examinations (12-lead ECG, signal-averaged ECG, 24-hour Holter-ECG, stress test and transthoracic echocardiogram) and third level exams, such as cardiac MR which represents the gold standard for the evaluation.

Diagnosis

The current diagnostic criteria consider 6 categories, which reflect the main characteristics of the disease: structural and functional abnormalities of the RV, depolarization and repolarization abnormalities on the ECG, arrhythmias, histological tissue characterization, family history and genetics. Definite diagnosis is made in presence of 2 major criteria, 1 major and 3 minor or 4 minor criteria.

Diagnostic criteria for arithmogenic cardiomyopathy of the right ventricle new “padua criteria”

Diagnostic Criteria of Arrhythmogenic Right Ventricle Cardiomyopathy (DIAGNOSTIC CRITERIA ITF 2010).

I. Global and regional dysfunction and structural alterations

Major

Echocardiography

• Regional RV akinesia, dyskinesia, or aneurism and 1 of the following (end diastole):

– PLAX RVOT ≥ 32 mm (corrected for body size PLAX/BSA ≥ 19 mm/m²)

– PSAX RVOT ≥ 36 mm (corrected for body size PSAX/BSA ≥ 21 mm/m²)

or RVFAC ≤ 33%

Magnetic Resonance

• Regional RV akinesia or dyskinesia or dyssynchronous RV contraction and 1 of the following:

– Ratio of RV end-diastolic volume to BSA ≥ 110 ml/m² (male) or ≥ 100 ml/m² (female) or RV ejection fraction ≤ 40%

RV angiography

• Regional RV akinesia, dyskinesia or aneurysm

Minor

Echocardiography

• Regional RV akinesia or dyskinesia and 1 of the following (end diastole):

PLAX RVOT ≥ 29 to < 32 mm (corrected for body size PLAX/BSA ≥ 16 to < 19 mm/m²)

PSAX RVOT ≥ 32 to < 36 mm (corrected for body size PSAX/BSA ≥ 18 to < 21 mm/m²)

RVFAC >33 to ≤ 40%

Magnetic Resonance

• Regional RV akinesia or dyskinesia or dyssynchronous RV contraction and 1 of the following: Ratio of RV end-diastolic volume to BSA ≥ 100 to < 110 ml/m² (male) or ≥ 90 to < 100 ml/m² (female)

or RV ejection fraction >40 to ≤ 45%

II. Tissue characterization

Major

Endomyocardial biopsy

• Residual myocytes < 60% on morphometric analysis (or < 50% if estimated), with fibrous replacement of the RV free wall myocardium in ≥ 1 sample, with or without fatty replacement of tissue

Minor

Endomyocardial biopsy

• Residual myocytes 60% to 75% by morphometric analysis (or 50% to 65% if estimated), with fibrous replacement of the RV free wall myocardium in ≥ 1 sample, with or without fatty replacement of tissue

III. Depolarization abnormalities

Major

Epsilon wave in right precordial leads (V1-V3)

Minor

• Late potentials by SAECG in ≥ 1 of 3 parameters in the absence of a QRS duration of ≥ 110 ms on the standard ECG
• Filtered QRS duration (fQRS) 114 ms
• Duration of terminal QRS < 40 µV (low-amplitude signal duration) ≥ 38 ms
• Root-mean-square voltage of terminal 40 ms ≤ 20 µV
• Terminal activation duration of QRS ≥ 55 ms measured from the nadir of the S wave to the end of the QRS, including R’, in V1, V2, V3 in the absence of complete right bundle-branch block

IV. Repolarization abnomalies

Major

ECG

• Negative Twaves in right precordial leads or beyond in individuals > 14 years of age (in the absence of complete right bundle branch block QRS ≥ 120 ms)

Minor

ECG

• Inverted Twaves in leads V1 and V2 in individuals > 14 years of age (in the absence of complete right bundle-branch block) or in V4, V5, or V6
• Inverted Twaves in leads V1, V2, V3, and V4 in individuals > 14 years of age in the presence of complete right bundle-branch block

V. Ventricular arrhythmias

Major

• Non-sustained or sustained ventricular tachycardia of left bundle-branch morphology with superior axis

Minor

• Non-sustained or sustained ventricular tachycardia of RV outflow configuration, left bundle-branch block morphology with inferior axis or unknown axis

At least 500 ventricular extrasystoles per day at Holter

VI. Family history and genetics

Major

• Definitive diagnosis of ARVC in a first-degree family member (Task Force 2010)
• Autoptic diagnosis (or from transplanted heart analysis) of ARVC in a first-degree family member
• Identification of a pathogenic mutation categorized as associated or probably associated with ARVC in the patient under evaluation

Minor

• History of ARVC in a first-degree relative in whom it is not possible or practical to determine whether the family member meets current Task Force criteria
• Premature sudden death (35 years of age) due to suspected ARVC/D in a first-degree relative
• ARVC confirmed pathologically or by current Task Force Criteria in second-degree relative

* PLAX RVOT = parasternal long-axis right ventricle outflow tract, PSAX = parasternal short-axis, RVFAC = right ventricle fractional area change, RV = right ventricle, SAECG = signal-averaged ECG.

Algorithm for diagnostics of acmp according to new 2020 criteria.

1. Right-dominant arrhythmogenic cardiopathy
   • Availability of updated APLC criteria (based on modified ITF 2010) – 2 large, 1 large and 2 small, 4 small criteria; the presence of ≥ 1 major or minor criterion from category I or II is required.
   • Absence of morphological or structural criteria of LV lesion
2. Biventricular ACMP
   • Availability of updated criteria for AFA in 2020 based on the modified criteria for ITF 2010 (presence of signs of a definitely accurate or probable diagnosis of AFA)
   • The presence of morphological or structural criteria for LV lesion
3. Left-dominant arrhythmogenic cardiopathy
   • The presence of structural criteria for LV lesion (the presence of a large criterion from category II)
   • Verification of pathogenic mutations in ACMP-associated genes
   • Absence or minimal involvement of lifespan (according to updated ITF 2010 criteria)

Molecular genetics

• According to the general recommendations for molecular genetic testing in inherited cardiomyopathies, genotyping is indicated to identify a pathogenic or likely pathogenic mutation in a proband who already fulfils phenotypic diagnostic criteria for ARVC, and thus to apply mutation-specific cascade genetic testing for detection of gene carriers among family members.
• Mutation-specific genetic testing is recommended for family members following the identification of a pathogenic or likely pathogenic mutation in the proband with a clinical diagnosis of definite ARVC, to identify genetically affected individuals at a preclinical phase.

Differential diagnosis:

• Idiopathic Ventricular Tachycardia
• Dilated Cardiomyopathy
• Sarcoidosis and myocarditis (ruled out by MR)
• Congenital conditions with right ventricular involvement (Uhl’s disease, Fallot’s Tetralogy)
• Brugada syndrome
• Pulmonary artery hypertension
• Athlete’s heart
• Genetic neuromuscular disorders

Management

There are no established best treatment options for patients with ARVC, and, as the disease is progressive, antiarrhythmic options are used for symptomatic benefit in patients with haemodynamically well-tolerated ventricular arrhythmias.

Excessive training or participation in sport must be avoided.

Antiarrhythmics

B -blockers alone or in combination with class I and III antiarrhythmics. Sotalol and amiodarone have been shown to be most effective.

Radiofrequency ablation

Used to reduce episodes of recurrent monomorphic ventricular tachycardia and ICD shocks in patients who do not respond to medical therapy or if it is contraindicated.

Implantable cardioverter defibrillators

In patients with life-threatening or drug-refractory ventricular arrhythmias and widespread disease, ICDs offer the best protective measure against SCD.

Cardiac Transplantation

In rare, selected, cases there may be a need for heart transplantation due to refractory HF, while incessant ventricular arrhythmias are an infrequent indication.

ICD classification: ICD-11 BC43.6; ICD-10 I42.8

This article is for informational purposes only. Self-medication can be harmful to your health. The use of any drugs mentioned in this article is possible only as directed and under the supervision of a physician.

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References:

1. Gianfranco Sinagra, Chiara Cappelletto, Antonio DE Luca, Simona Romani, Alessia Paldino, Renata Korcova, Matteo Dal Ferro, Giancarlo Vitrella, Lorenzo Pagnan, Bruno Pinamonti, Focus on arrhythmogenic right ventricular cardiomyopathy, European Heart Journal Supplements, Volume 22, Issue Supplement_L, November 2020, Pages L129–L135, https://doi.org/10.1093/eurheartj/suaa152
2. (2012-02). Ramrakha, P., & Hill, J. (Eds.), Oxford Handbook of Cardiology. Oxford, UK: Oxford University Press. Retrieved 22 Aug. 2021, from https://oxfordmedicine.com/view/10.1093/med/9780199643219.001.0001/med-9780199643219.
3. Domenico Corrado, Peter J van Tintelen, William J McKenna, Richard N W Hauer, Aris Anastastakis, Angeliki Asimaki, Cristina Basso, Barbara Bauce, Corinna Brunckhorst, Chiara Bucciarelli-Ducci, Firat Duru, Perry Elliott, Robert M Hamilton, Kristina H Haugaa, Cynthia A James, Daniel Judge, Mark S Link, Francis E Marchlinski, Andrea Mazzanti, Luisa Mestroni, Antonis Pantazis, Antonio Pelliccia, Martina Perazzolo Marra, Kalliopi Pilichou, Pyotr G A Platonov, Alexandros Protonotarios, Alessandra Rampazzo, Jeffry E Saffitz, Ardan M Saguner, Christian Schmied, Sanjay Sharma, Hari Tandri, Anneline S J M Te Riele, Gaetano Thiene, Adalena Tsatsopoulou, Wojciech Zareba, Alessandro Zorzi, Thomas Wichter, Frank I Marcus, Hugh Calkins, International Experts, Arrhythmogenic right ventricular cardiomyopathy: evaluation of the current diagnostic criteria and differential diagnosis, European Heart Journal, Volume 41, Issue 14, 7 April 2020, Pages 1414–1429, https://doi.org/10.1093/eurheartj/ehz669
4. Ackerman MJ, Priori SG, Willems S, Berul C, Brugada R, Calkins H, Camm AJ, Ellinor PT, Gollob M, Hamilton R, Hershberger RE, Judge DP, Le Marec H, McKenna WJ, Schulze-Bahr E, Semsarian C, Towbin JA, Watkins H, Wilde A, Wolpert C, Zipes DP. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies: this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Europace 2011; 13: 1077–1109.
5. Corrado D, Perazzolo-Marra M, Zorzi A, et al. Diagnosis of arrhythmogenic cardiomyopathy: The Padua criteria. International Journal of Cardiology. 2020; doi:10.1016/j.ijcard.2020.06.005.

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Diagnosis of endocarditis

Key facts!

• Clinical features can be non-specific and diagnosis difficult.
• Maintain a high index of suspicion in patients presenting with unexplained fever, a predisposing cardiac lesion, bacteremia, and embolic phenomena.

Duke classification

• Developed in 1994 and modified in 2000 as a means of standardizing the diagnosis of IE.
• This classification has a sensitivity of approximately 80% overall when the criteria are evaluated at the end of patient follow-up in epidemiological studies.
• However, the modified Duke criteria show a lower diagnostic accuracy for early diagnosis in clinical practice, especially in the case of prosthetic valve endocarditis (PVE) and pacemaker or defibrillator lead IE.
• Cardiac/whole-body computer tomography (CT) scan, cerebral MRI, 18F-FDG PET/CT, and radiolabeled leukocyte SPECT/CT might improve the detection of silent vascular phenomena as well as endocardial lesions.
• The addition of the results of these imaging modalities may improve the sensitivity of the modified Duke criteria in difficult cases.

Definitions of the terms used in the European Society of Cardiology 2015 modified criteria for the diagnosis of infective endocarditis

Definition of infective endocarditis according to the modified Duke criteria (adapted from Li et al.)

Investigation of endocarditis

Literature:

1. Durack DT, Lukes AS, Bright DK. New criteria for diagnosis of infective endocarditis: utilization of specific echocardiographic findings. Duke Endocarditis Service. American Journal of Medicine. 96(3):200-9, 1994.
2. Li JS, Sexton DJ, Mick N, Nettles R, Fowler VG Jr, Ryan T, Bashore T, Corey GR. Proposed modifications to the Duke criteria for the diagnosis of infective endocarditis. Clin Infect Dis. 2000 Apr;30(4):633-8. doi: 10.1086/313753. Epub 2000 Apr 3. PMID: 10770721.
3. Yehuda Adler, Philippe Charron, Massimo Imazio, Luigi Badano, Gonzalo Barón-Esquivias, Jan Bogaert, Antonio Brucato, Pascal Gueret, Karin Klingel, Christos Lionis, Bernhard Maisch, Bongani Mayosi, Alain Pavie, Arsen D Ristić, Manel Sabaté Tenas, Petar Seferovic, Karl Swedberg, Witold Tomkowski, ESC Scientific Document Group, 2015 ESC Guidelines for the diagnosis and management of pericardial diseases: The Task Force for the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology (ESC) Endorsed by: The European Association for Cardio-Thoracic Surgery (EACTS), European Heart Journal, Volume 36, Issue 42, 7 November 2015, Pages 2921–2964, https://doi.org/10.1093/eurheartj/ehv318
4. Thornhill MH, Gibson TB, Cutler E, Dayer MJ, Chu VH, Lockhart PB, et al. Antibiotic Prophylaxis and Incidence of Endocarditis Before and After the 2007 AHA Recommendations. J Am Coll Cardiol. 2018 Nov 13. 72 (20):2443-2454. [Medline].
5. https://emedicine.medscape.com/article/216650-guidelines

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A 55-year-old woman presents with chest pain. She reports the chest pain started several days ago and describes it as sharp and brief. When she sits up, the pain improves. She has no significant illnesses in her past medical history but endorses a viral respiratory infection a few days ago.  On physical exam, she is in no acute distress. She has a low-grade fever. ECG shows widespread ST segment elevation and PR depression in the precordial leads.

Relevance

Pericarditis is the most common form of pericardial disease worldwide and is typically encountered in young and middle-aged people.

Main causes of acute pericarditis include:

Aetiology depends on the type of pericarditis:

Pathogenesis of the disease

• inflammation of the pericardium can cause chest pain;
• movement of the heart can cause friction between the 2 pericardial layers, producing a friction rub;
• inflammation may cause a pericardial effusion.

Diagnosis

The diagnosis is clinical and can be made based on two of the following criteria:

Clinical Findings:

• Chest pain (>85% to 90% of cases) that usually radiates to the trapezius ridge of the left shoulder or arm and resembles ischaemic pain. However, retrosternal pain in acute pericarditis is mainly sharp and pleuritic, which augments in the supine position with cough and deep inspiration. Conversely, it usually subsides in the upright position and by leaning forward due to decrease of the pressure on the parietal pericardium:
  • sharp pleuritic chest pain that is worsened by inhalation;
  • pain is also relieved by sitting up and leaning forward;
  • shoulder pain (referred pain) (pericarditis is innervated by phrenic nerve).

Physical exam

• May have a fever.
• Friction rub (pathognomonic) – Auscultation exposes a pericardial friction rub (pathognomonic sign for pericarditis) is best heard with the diaphragm of the stethoscope over the left sternal border, with the patient leaning forward. The auscultation of pericardial friction rub is highly specific for acute pericarditis (specificity approximately 100%).
• Kussmaul sign (seen in constrictive pericarditis).
• ↑ jugular venous distention on inspiration.
• Hypotension, tachycardia, pulsus paradoxus, and impaired diastolic filling in constrictive pericarditis.

ECG Findings

• the occurrence of ST-elevation less than 5 mm;
• ST-segment concavity;
• widespread ST segment elevation;
• more extensive lead involvement;
• less prominent reciprocal ST-segment depression;
• PR segment depression, especially in lead II and in all leads except aVR;
• PR-segment elevation in aVR, with reciprocal PR-segment depression in other leads;
• the absence of abnormal Q-waves;
• variability in the time of T-wave inversion occurrence following ST-segment elevation;
• the lack of QRS widening and QT interval shortening in leads with ST elevation;
• upright T waves;
• weeks after pericarditis, this will become inverted T waves.

NB! classic ECG signs may be absent in uremic pericarditis

Laboratory Tests

Elevation of inflammatory markers is common in patients presenting with acute pericarditis such as:

• white blood cells (WBCs) count;
• ↑ erythrocyte sedimentation rate (ESR);
• ↑ C-reactive protein (CRP);
• may have ↑ troponin I, T, creatine phosphokinase.

* The evaluation of troponins does not seem to exhibit prognostic value on acute pericarditis.
* Interestingly, pericarditis patients with elevated biomarkers of myocardial injury almost always exhibit ECG changes characteristic of ST-segment elevation.

The prognostic role is promising for carcinoembryonic antigen cell adhesion molecule 1 (CEACAM1), which is an immune inhibitory protein, and for MHC class I chain related protein A (MICA), which exhibits an immune stimulating function in patients with acute and recurrent pericarditis. CEACAM1 and MICA are serum proteins that can be released from damaged or inflamed tissues in patients with pericarditis are found to be increased as well.

A routine laboratory evaluation should include markers of renal function (urea, creatinine), electrolytes, LDH and transaminases (SGOT, SGPT). If there is a clinical indication, more specific tests may be ordered, such as blood cultures, thyroid function hormones, antinuclear antibodies (ANA), anti-neutrophil cytoplasm antibodies (ANCA), anti-extractable nuclear antigens (ENA), HIV testing and even pericardial biopsy.

Chest X-ray

Usually within normal limits since an increased cardiothoracic index is exhibited when pericardial effusion is more than 300 ml. Signs of pleuropericardial involvement may be found in the background of pleuropulmonary disease in the chest X-rays of patients with acute pericarditis. Chest X-rays may reveal malignancies, sarcoidosis, or several infections, which may be the cause of acute pericarditis. 

N.B.! A patient presenting with new and otherwise unexplained cardiomegaly, especially in the setting of clear lung fields, should be evaluated for acute pericarditis

Imaging

Echocardiography

Pericardial effusion, either new appearing or worsening, is typically mild and is evident in 60% of cases. Pericardial effusion, based on the size at diastole, is characterised as mild (<10 mm), moderate (10-20 mm), or large (>20 mm). The hallmark of benign, idiopathic effusion is a clear ECHO-free space, whereas malignancy, bacterial infection, and haemorrhagic effusions are more likely to have solid components or stranding.

CT/CMR

Cardiac CT aids in the assessment of pericardial calcifications and thickening and the evaluation of concomitant diseases. CMR can provide the characterisation of cardiac tissue, the assessment of thickening and constriction, and the evaluation of myocardial involvement. CMR does not require radiation or nephrotoxic contrast agents. Furthermore, baseline late gadolinium enhancement (LGE) seen on CMR may correlate with inflammation severity. These techniques demonstrate pericardial thickening (>5 mm). This technique also has several weaknesses as it is time-consuming, high cost, not widely available, and requires stable patients and rhythms. In addition, patients with severe renal impairment and pacemakers may not be eligible candidates. 

The following groups of patients seem most likely to benefit from cardiac MRI imaging for pericarditis:

1. Patients with persistent chest pain after a negative ischemic evaluation.
2. Patients with non-ischemic chest pain suspicious for pericarditis, but no detectable effusion on 2-D echocardiography.
3. Patients with non-ischemic chest pain suspicious for pericarditis, but a poor quality or non-diagnostic 2-D echo study.
4. Patients with recurrent chest pain after a negative ischemic evaluation and a negative “non-cardiac” chest pain evaluation.
5. Younger patients with atypical chest discomfort and a low likelihood of coronary atherosclerotic disease.

Nuclear medicine

In selected cases, positron emission tomography (PET) alone, or preferably in combination with CT (PET/CT), can be indicated to depict the metabolic activity of pericardial disease. Pericardial uptake of 18F-fluorodeoxyglucose (FDG) tracer in patients with solid cancers and lymphoma is indicative of (malignant) pericardial involvement, thus providing essential information on the diagnosis, staging and assessment of the therapeutic response.

Cardiac catheterization

This demonstrates elevation and equalization of filling pressures. In the typical case, the difference in filling pressures between the right ventricle and left ventricle does not exceed 6 mmHg. The right atrial waveform shows rapid ‘x’ and ‘y’ descents, and the mean pressure does not decrease normally with inspiration or may show Kussmauls sign.

According to the 2015 ESC guidelines on pericardial disease, the definite diagnosis of viral pericarditis derives from the comprehensive workup of histological, cytological, immunobiological and molecular investigations in pericardial fluid and pericardial/epicardial biopsies; otherwise, the term ‘presumed viral pericarditis’ should be used.

For the diagnosis of purulent pericarditis, urgent pericardiocentesis is recommended and pericardial fluid should be sent for bacterial, fungal and tuberculous studies. Blood should be drawn for cultures.

Furthermore, urgent TTE or CT is recommended in patients with a history of chest trauma and systemic arterial hypotension. Lastly, as far as malignancies are concerned, cytological analyses of pericardial fluid are recommended.

Main analyses to be performed on pericardial fluid:

Definitions and diagnostic criteria for pericarditis

Differential diagnosis

Major differential diagnoses include acute coronary syndromes with ST-segment elevation and early repolarization.

Diagnosis formulation

I. Etiological characteristics.

• Pericarditis in bacterial infections I32.0.
• Pericarditis in infectious and parasitic diseases I32.1.
• Pericarditis in other diseases I32.8.
• Pericarditis unspecified I32.9.

II. Pathogenetic and morphological variants.

• Chronic adhesive I31.0.
• Chronic constrictive I31.1, including pericardial calcification.
• Hemopericardium I31.2.
• Pericardial effusion (non-inflammatory) – hydropericardium I31.3, including chylopericardium.

III. Complication.

• Myopericarditis.
• Perimyocarditis.

IV. The character of the flow.

• Acute.
• Subacute.
• Chronic.
• Recurrent.

V. Assessment of the severity of pericardial effusion according to ultrasound (ultrasound) and other research methods (minor, moderate, significant).

VI. CH (0-III stage, I-IV FC).

Tactics

It is not mandatory to search for the aetiology in all patients, especially in countries with a low prevalence of TB, because of the relatively benign course associated with the common causes of pericarditis and the relatively low yield of diagnostic investigations.

The major risk factors associated with poor prognosis after multivariate analysis include:

• high fever [>38°C (>100.4°F)]
• subacute course (symptoms over several days without a clear-cut acute onset)
• evidence of large pericardial effusion (i.e. diastolic echo-free space >20 mm), cardiac tamponade and failure to respond within 7 days to non-steroidal anti-inflammatory drugs (NSAIDs).

Other risk factors should also be considered (i.e. ‘minor risk factors’); these are based on expert opinion and literature review, including pericarditis associated with myocarditis (myopericarditis), immunodepression, trauma and oral anticoagulant therapy.

On this basis a triage for acute pericarditis is proposed. Any clinical presentation that may suggest an underlying aetiology (e.g. a systemic inflammatory disease) or with at least one predictor of poor prognosis (major or minor risk factors) warrants hospital admission and an aetiology search.

In the management of pericardial syndromes, a major controversy is the role of an extensive aetiological search and admission for all patients with pericarditis or pericardial effusion.

On this basis, auscultation, ECG, echocardiography, chest X-ray, routine blood tests, including markers of inflammation (i.e., CRP and/or ESR) and myocardial lesions (CK, troponins), are recommended in all cases of suspected pericarditis.

The major specific causes to be ruled out are bacterial pericarditis (especially TB), neoplastic pericarditis and pericarditis associated with a systemic disease (generally an autoimmune disease).

Next, an assessment of risk factors (major or minor) is carried out.

CT and/or CMR are recommended as second-level testing for diagnostic workup in pericarditis.

Diagnostic tests of the first and second levels at suspicion of pericarditis

Treatment

Patients identified with a cause other than viral infection, specific therapy appropriate to the underlying disorder is indicated.

The first non-pharmacological recommendation is to restrict physical activity beyond ordinary sedentary life until resolution of symptoms and normalization of CRP for patients not involved in competitive sports. Athletes are recommended to return to competitive sports only after symptoms have resolved and diagnostic tests (i.e. CRP, ECG and echocardiogram) have been normalized. A minimal restriction of 3 months (after the initial onset of the attack) has been arbitrarily defined according to expert consensus.

Aspirin or NSAIDs are mainstays of therapy for acute pericarditis.

The choice of drug should be based on the history of the patient (contraindications, previous efficacy or side effects), the presence of concomitant diseases (favouring aspirin over other NSAIDs when aspirin is already needed as antiplatelet treatment) and physician expertise.

Colchicine is recommended at low, weight-adjusted doses to improve the response to medical therapy and prevent recurrences. Tapering of colchicine is not mandatory but may be considered to prevent persistence of symptoms and recurrence.

Corticosteroids should be considered as a second option in patients with contraindications and failure of aspirin or NSAIDs because of the risk of favouring the chronic evolution of the disease and promoting drug dependence; in this case they are used with colchicine. If used, low to moderate doses (i.e. prednisone 0.2–0.5 mg/kg/day or equivalent) should be recommended instead of high doses (i.e. prednisone 1.0 mg/kg/day or equivalent). The initial dose should be maintained until resolution of symptoms and normalization of CRP, then tapering should be considered.

Recommendations for the treatment of acute pericarditis:

• Aspirin or NSAIDs are recommended as first-line therapy for acute pericarditis with gastroprotection.
• Colchicine is recommended as first-line therapy for acute pericarditis as an adjunct to aspirin/NSAID therapy.
• Serum CRP should be considered to guide the treatment length and assess the response to therapy.
• Low-dose corticosteroids should be considered for acute pericarditis in cases of contraindication/failure of aspirin/ NSAIDs and colchicine, and when an infectious cause has been excluded, or when there is a specific indication such as autoimmune disease.
• Exercise restriction should be considered for non-athletes with acute pericarditis until resolution of symptoms and normalization of CRP. ECG and echocardiogram.
• For athletes, the duration of exercise restriction should be considered until resolution of symptoms and normalization of CRP. ECG and echocardiogram—at least 3 months is recommended.
• Corticosteroids are not recommended as first-line therapy for acute pericarditis.

Recurrent pericarditis

Recurrent pericarditis therapy should be targeted at the underlying aetiology in patients with an identified cause. Aspirin or NSAIDs remain the mainstay of therapy. Colchicine is recommended on top of standard anti-inflammatory therapy, without a loading dose and using weight-adjusted doses (i.e. 0.5 mg once daily if body weight is <70 kg or 0.5 mg twice daily if it is ≥70 kg, for ≥6 months) in order to improve the response to medical therapy, improve remission rates and prevent recurrences.

In cases of incomplete response to aspirin/NSAIDs and colchicine, corticosteroids may be used, but they should be added at low to moderate doses to aspirin/NSAIDs and colchicine as triple therapy, not replace these drugs, in order to achieve better control of symptoms. Corticosteroids at low to moderate doses (i.e. prednisone 0.2–0.5 mg/kg/day) should be avoided if infections, particularly bacterial and TB, cannot be excluded and should be restricted to patients with specific indications (i.e. systemic inflammatory diseases, post-pericardiotomy syndromes, pregnancy) or NSAID contraindications (true allergy, recent peptic ulcer or gastrointestinal bleeding, oral anticoagulant therapy when the bleeding risk is considered high or unacceptable) or intolerance or persistent disease despite appropriate doses. Although corticosteroids provide rapid control of symptoms, they favour chronicity, more recurrences and side effects. If corticosteroids are used, their tapering should be particularly slow. A critical threshold for recurrences is a 10–15 mg/day dose of prednisone or equivalent. At this threshold, very slow decrements as small as 1.0–2.5 mg at intervals of 2–6 weeks are useful. In cases of recurrence, every effort should be made not to increase the dose or to reinstate corticosteroids. After obtaining a complete response, tapering should be done with a single class of drug at a time before colchicine is gradually discontinued (over several months in the most difficult cases). Recurrences are possible after discontinuation of each drug. Each tapering should be attempted only if symptoms are absent and CRP is normal.

Drugs such as IVIG, anakinra and azathioprine may be considered in cases of proven infection-negative, corticosteroid-dependent, recurrent pericarditis not responsive to colchicine after careful assessment of the costs, risks and eventually consultation by multidisciplinary experts, including immunologists and/or rheumatologists, in the absence of a specific expertise.

As a last resort, pericardiectomy may be considered, but only after a thorough trial of unsuccessful medical therapy, and with referral of the patient to a centre with specific expertise in this surgery.

Prednisolone reduction regimen:

Avoid higher doses except for special cases, and only for a few days, with rapid tapering to 25 mg/day. Prednisone 25 mg are equivalent to methylprednisolone 20 mg.

Every decrease in prednisone dose should be done only if the patient is asymptomatic and C-reactive protein is normal, particularly for doses <25 mg/day.

Calcium intake (supplement plus oral intake) 1,200–1,500 mg/day and vitamin D supplementation 800–1000 IU/day should be offered to all patients receiving glucocorticoids. Moreover, bisphosphonates are recommended to prevent bone loss in all men ≥50 years and postmenopausal women in whom long-term treatment with glucocorticoids is initiated at a dose ≥5.0–7.5 mg/day of prednisone or equivalent.

Pericardiocentesis or surgical drainage are indicated for cardiac tamponade or suspected bacterial and neoplastic pericarditis.

Algorithm for the treatment of acute and recurrent pericarditis

Low-dose corticosteroids arc considered when there arc contraindications to other drugs or when there is an incomplete response to aspirin/NSAIDs plus colchicine; in this case physicians should consider adding these drugs instead of replacing other anti-inflammatory therapies.

Azathioprine is steroid-sparing and has a slow onset of action compared with IVIG and anakinra. Co« considerations may apply considering the cheaper solution first (e.g. aaathiophne) and resorting to more expensive options (e g. IVIG and anakinra) for refractory cases.

Complications

• Pericardial effusion and tamponade.

Prognosis

• Can be acute or chronic and may recur.
• Viral pericarditis is usually self-limited.

Most patients with acute pericarditis (generally those with presumed viral or idiopathic pericarditis) have a good long-term prognosis. Cardiac tamponade rarely occurs in patients with acute idiopathic pericarditis, and is more common in patients with a specific underlying aetiology such as malignancy, TB or purulent pericarditis. Constrictive pericarditis may occur in <1% of patients with acute idiopathic pericarditis, and is also more common in patients with a specific aetiology. The risk of developing constriction can be classified as low (<1%) for idiopathic and presumed viral pericarditis; intermediate (2–5%) for autoimmune, immune-mediated and neoplastic aetiologies; and high (20–30%) for bacterial aetiologies, especially with TB and purulent pericarditis. Approximately 15–30% of patients with idiopathic acute pericarditis who are not treated with colchicine will develop either recurrent or incessant disease, while colchicine may halve the recurrence rate.

This article is for informational purposes only. Self-medication can be harmful to your health. The use of any drugs mentioned in this article is possible only as directed and under the supervision of a physician.

Literature:
1. Klein A. L., Abbara S., Agler D. A., Appleton C. P., Asher C. R., Hoit B., Hung J., Garcia M. J., Kronzon I., Oh J. K., Rodriguez E. R., Schaff H. V., Schoenhagen P., Tan C. D., & White R. D. American Society of Echocardiography clinical recommendations for multimodality cardiovascular imaging of patients with pericardial disease: endorsed by the Society for Cardiovascular Magnetic Resonance and Society of Cardiovascular Computed Tomography. J Am Soc Echocardiogr, 2013, Sept 26. (9): 965-1012.e15.
2. Markel G., Imazio M., Koren-Morag N., Galore-Haskel G., Schachter J., Besser M., Cumetti D., Maestroni S., Altman A., Shoenfeld Y., Brucato A., & Adler Y. CEACAM1 and MICA as novel serum biomarkers in patients with acute and recurrent pericarditis. Oncotarget, 2016 April 5. 7 (14): 17885-95.
3. Imazio M., Brucato A., Badano L., Charron P., & Adler Y. What’s new in 2015 ESC guidelines on pericardial diseases? J Cardiovasc Med (Hagerstown), 2016, May 17. (5): 315-22.
4. J. Ronald Mikolich. New Diagnostic Criteria for Acute Pericarditis: A Cardiac MRI Perspective Nov 05, 2015, , MD, FACC. Expert Analysis
5. Yehuda Adler, Philippe Charron, Massimo Imazio, Luigi Badano, Gonzalo Barón-Esquivias, Jan Bogaert, Antonio Brucato, Pascal Gueret, Karin Klingel, Christos Lionis, Bernhard Maisch, Bongani Mayosi, Alain Pavie, Arsen D Ristić, Manel Sabaté Tenas, Petar Seferovic, Karl Swedberg, Witold Tomkowski, ESC Scientific Document Group, 2015 ESC Guidelines for the diagnosis and management of pericardial diseases: The Task Force for the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology (ESC) Endorsed by: The European Association for Cardio-Thoracic Surgery (EACTS), European Heart Journal, Volume 36, Issue 42, 7 November 2015, Pages 2921-2964, https://doi.org/10.1093/eurheartj/ehv318

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