Pericarditis in Children
Introduction to Pericarditis in Children
Pericarditis is an inflammation of the pericardium, the fibrous sac surrounding the heart. In children, it can present unique challenges in diagnosis and management due to varying etiologies and clinical presentations. Pediatric pericarditis can be acute (lasting less than 6 weeks), recurrent (recurring within 4-6 weeks after an initial episode), or chronic (lasting longer than 6 weeks).
The incidence of pericarditis in children is not well-established, but it is considered less common than in adults. However, its potential for serious complications, including cardiac tamponade and constrictive pericarditis, makes it a significant concern in pediatric cardiology.
Etiology of Pediatric Pericarditis
The causes of pericarditis in children can be diverse:
- Infectious Causes:
- Viral (most common): Coxsackieviruses, Echoviruses, Adenoviruses, Influenza viruses
- Bacterial: Staphylococcus aureus, Streptococcus pneumoniae, Mycobacterium tuberculosis
- Fungal: Histoplasma, Coccidioides (rare in immunocompetent children)
- Parasitic: Toxoplasma gondii (rare)
- Autoimmune Disorders:
- Systemic Lupus Erythematosus (SLE)
- Juvenile Idiopathic Arthritis (JIA)
- Kawasaki Disease
- Post-cardiac Injury Syndromes:
- Post-pericardiotomy syndrome
- Post-myocardial infarction pericarditis (rare in children)
- Neoplastic: Primary tumors or metastases (rare in children)
- Metabolic: Uremia in children with chronic kidney disease
- Traumatic: Chest trauma or iatrogenic during cardiac procedures
- Idiopathic: No identifiable cause (common in children)
Clinical Presentation of Pericarditis in Children
The clinical presentation of pericarditis in children can vary widely, often making diagnosis challenging. Key features include:
- Chest Pain: The most common symptom, typically sharp and pleuritic, worsening with inspiration or supine position.
- Fever: Often present, especially in infectious cases.
- Dyspnea: May occur, particularly if there's significant pericardial effusion.
- Tachycardia: Common, often out of proportion to fever.
- Fatigue and Weakness: Particularly in chronic cases.
- Pericardial Friction Rub: A pathognomonic sign, but can be transient and easily missed.
- Muffled Heart Sounds: In cases with significant effusion.
- Signs of Tamponade: In severe cases, including jugular venous distension, pulsus paradoxus, and hypotension.
It's important to note that younger children may not articulate chest pain clearly, and the presentation can be more subtle, often mimicking other conditions like pneumonia or viral illnesses.
Diagnosis of Pediatric Pericarditis
Diagnosis of pericarditis in children involves a combination of clinical assessment, laboratory tests, and imaging studies:
- Clinical Examination:
- Careful auscultation for pericardial friction rub
- Assessment for signs of cardiac tamponade
- Laboratory Tests:
- Inflammatory markers: Elevated ESR, CRP
- Cardiac enzymes: Troponin, CK-MB (may be elevated if myocardial involvement)
- Complete blood count
- Viral studies and cultures as indicated
- Autoimmune markers if suspecting rheumatologic cause
- Electrocardiogram (ECG):
- Diffuse ST-segment elevation (typically concave up)
- PR segment depression
- Electrical alternans in large effusions
- Imaging:
- Echocardiography: Gold standard for detecting pericardial effusion and assessing hemodynamic impact
- Chest X-ray: May show enlarged cardiac silhouette in effusive pericarditis
- Cardiac MRI: Useful for assessing pericardial inflammation and thickening, especially in chronic cases
- CT scan: May be used if MRI is not available or contraindicated
- Pericardiocentesis: For diagnostic and therapeutic purposes in large effusions or suspected purulent pericarditis
The diagnosis of pericarditis requires at least two of the following criteria:
- Typical chest pain
- Pericardial friction rub
- Suggestive ECG changes
- New or worsening pericardial effusion
Treatment of Pericarditis in Children
The treatment of pericarditis in children depends on the underlying etiology and severity of the condition:
- General Measures:
- Hospital admission for initial evaluation and treatment
- Monitoring for complications, especially cardiac tamponade
- Activity restriction during acute phase
- Pharmacological Management:
- NSAIDs (e.g., Ibuprofen): First-line treatment for idiopathic or viral pericarditis
- Colchicine: Often used in combination with NSAIDs to reduce recurrence
- Corticosteroids: Reserved for refractory cases or specific etiologies (e.g., autoimmune)
- Antibiotics: For bacterial pericarditis, based on culture and sensitivity
- Antiviral agents: Rarely used, except in specific viral etiologies
- Management of Pericardial Effusion:
- Pericardiocentesis: Indicated for large effusions, hemodynamic compromise, or suspected purulent pericarditis
- Pericardial window: For recurrent or loculated effusions
- Treatment of Underlying Cause:
- Management of autoimmune disorders if present
- Specific treatment for infectious causes (e.g., tuberculosis)
- Follow-up:
- Regular echocardiographic monitoring
- Gradual return to normal activities
- Long-term follow-up for recurrent or chronic cases
It's crucial to tailor the treatment to the individual child, considering factors such as age, underlying health conditions, and the specific characteristics of the pericarditis.
Complications of Pediatric Pericarditis
While many cases of pericarditis in children resolve without sequelae, several complications can occur:
- Cardiac Tamponade:
- Life-threatening complication due to accumulation of pericardial fluid
- Presents with Beck's triad: hypotension, jugular venous distension, and muffled heart sounds
- Requires immediate pericardiocentesis
- Constrictive Pericarditis:
- Rare but serious long-term complication
- Results from pericardial scarring and fibrosis
- May require pericardiectomy in severe cases
- Recurrent Pericarditis:
- Occurs in 15-30% of children after an initial episode
- Can lead to significant morbidity and impact on quality of life
- Myocardial Involvement (Myopericarditis):
- Can lead to transient myocardial dysfunction
- Increases risk of arrhythmias
- Pericardial Effusion:
- Can persist or recur, sometimes requiring repeated drainage
- Chronic Pain Syndrome:
- Some children may develop chronic chest pain even after resolution of acute pericarditis
Early recognition and appropriate management of these complications are crucial in improving outcomes in pediatric pericarditis.
Prognosis of Pericarditis in Children
The prognosis of pericarditis in children is generally favorable, but it can vary depending on the underlying cause and the presence of complications:
- Acute Viral or Idiopathic Pericarditis:
- Usually self-limiting with good response to treatment
- Most children recover completely within weeks to months
- Recurrent Pericarditis:
- Can have a more prolonged course
- May require long-term anti-inflammatory therapy
- Generally good long-term prognosis with appropriate management
- Purulent Pericarditis:
- Higher morbidity and mortality if not promptly treated
- Good outcomes with early diagnosis and appropriate antibiotics
- Pericarditis Associated with Systemic Diseases:
- Prognosis often depends on the underlying condition
- May require ongoing management of the primary disease
- Constrictive Pericarditis:
- Rare but serious; may require surgical intervention
- Outcomes are generally good with timely pericardiectomy
Long-term follow-up is important, especially in cases of recurrent or chronic pericarditis. Most children with adequately treated pericarditis can expect to lead normal, active lives without significant cardiac sequelae.
Acute Pericarditis in Children
Acute pericarditis is defined as inflammation of the pericardium lasting less than 6 weeks. It's the most common form of pericarditis in children.
Key Features:
- Etiology: Often viral or idiopathic in children.
- Clinical Presentation:
- Chest pain (often sharp, pleuritic, and positional)
- Fever
- Pericardial friction rub (pathognomonic but often transient)
- Diagnosis:
- ECG changes: Diffuse ST-segment elevation, PR depression
- Echocardiography: May show pericardial effusion
- Elevated inflammatory markers (ESR, CRP)
- Treatment:
- NSAIDs (e.g., Ibuprofen) as first-line treatment
- Colchicine often added to reduce recurrence risk
- Rest and activity restriction during acute phase
- Prognosis: Generally good, with most cases resolving within weeks.
Acute pericarditis in children requires prompt diagnosis and treatment to prevent complications and reduce the risk of recurrence.
Recurrent Pericarditis in Children
Recurrent pericarditis is defined as a repeated episode of acute pericarditis occurring after a symptom-free interval of 4-6 weeks or longer following the initial episode.
Key Features:
- Incidence: Occurs in about 15-30% of children after an initial episode of acute pericarditis.
- Pathophysiology: Thought to involve autoimmune mechanisms or viral persistence.
- Clinical Presentation:
- Similar to acute pericarditis but may be more intense
- Chest pain, fever, and fatigue are common
- Pericardial effusion may be present
- Diagnosis:
- Based on recurrence of symptoms and signs after a symptom-free interval
- ECG changes may be less pronounced than in the initial episode
- Echocardiography to assess for effusion and cardiac function
- Treatment:
- NSAIDs as first-line treatment
- Colchicine is crucial for preventing further recurrences
- Corticosteroids in refractory cases, but used cautiously due to risk of dependence
- Anakinra (IL-1 receptor antagonist) in severe, steroid-dependent cases
- Prognosis: Variable, with some children experiencing multiple recurrences, but generally good long-term outlook with appropriate management.
Management of recurrent pericarditis in children focuses on symptom control and prevention of further recurrences, often requiring a multidisciplinary approach.
Chronic Pericarditis in Children
Chronic pericarditis is defined as pericardial inflammation lasting longer than 6 months. It's less common in children compared to acute and recurrent forms.
Key Features:
- Etiology:
- Often associated with systemic autoimmune diseases (e.g., lupus, rheumatoid arthritis)
- Can result from long-standing infectious causes (e.g., tuberculosis)
- Sometimes idiopathic
- Clinical Presentation:
- May be subtle and nonspecific
- Persistent or intermittent chest pain
- Fatigue, exercise intolerance
- Signs of heart failure in advanced cases
- Diagnosis:
- Echocardiography: May show pericardial thickening or effusion
- Cardiac MRI: Useful for assessing pericardial inflammation and thickening
- CT scan: Can show pericardial calcification in long-standing cases
- Treatment:
- Targeted at underlying cause if identified
- Long-term anti-inflammatory therapy (NSAIDs, colchicine)
- Immunosuppressive agents in autoimmune-related cases
- Pericardiectomy in cases progressing to constrictive pericarditis
- Complications:
- Constrictive pericarditis
- Chronic pericardial effusion
- Myocardial atrophy and dysfunction
Chronic pericarditis in children requires careful long-term management and monitoring to prevent progression to constrictive pericarditis and other complications.
Constrictive Pericarditis in Children
Constrictive pericarditis is a rare but serious condition characterized by a thickened, fibrotic pericardium that restricts normal cardiac filling.
Key Features:
- Etiology:
- Can result from any cause of chronic pericarditis
- In children, often following viral or purulent pericarditis
- Tuberculosis is a significant cause in endemic areas
- Rarely, post-cardiac surgery or radiation therapy
- Pathophysiology:
- Pericardial inflammation leads to fibrosis and calcification
- Restricts diastolic filling of ventricles
- Results in elevated and equalized diastolic pressures in all cardiac chambers
- Clinical Presentation:
- Often insidious onset
- Fatigue, exercise intolerance
- Signs of right heart failure: hepatomegaly, ascites, peripheral edema
- Kussmaul's sign (paradoxical increase in jugular venous pressure with inspiration)
- Pericardial knock (early diastolic sound)
- Diagnosis:
- Echocardiography: Thickened pericardium, septal bounce, dilated IVC
- Cardiac catheterization: Square root sign on ventricular pressure tracings
- CT or MRI: Pericardial thickening, calcification
- Treatment:
- Pericardiectomy (surgical removal of the pericardium) is definitive treatment
- Medical management (diuretics, afterload reduction) for symptom control
- Treatment of underlying cause if identified
- Prognosis:
- Variable, depending on timing of diagnosis and treatment
- Early surgical intervention generally associated with better outcomes
- Some children may have residual cardiac dysfunction even after pericardiectomy
Constrictive pericarditis in children requires a high index of suspicion for diagnosis and often necessitates surgical intervention for definitive treatment.
Infectious Pericarditis in Children
Infectious pericarditis refers to inflammation of the pericardium caused by various pathogens. In children, viral causes are most common, but bacterial and fungal etiologies can occur, especially in immunocompromised patients.
Key Features:
- Viral Pericarditis:
- Most common form in children
- Causative agents: Enteroviruses (especially Coxsackievirus), Adenovirus, Influenza virus
- Often self-limiting but can lead to recurrences
- Treatment: Supportive care, NSAIDs, sometimes colchicine
- Bacterial Pericarditis:
- Less common but more severe
- Causative agents: Staphylococcus aureus, Streptococcus pneumoniae, Haemophilus influenzae
- Often associated with empyema or sepsis
- Treatment: Antibiotics, pericardial drainage, sometimes surgical intervention
- Fungal Pericarditis:
- Rare in immunocompetent children
- Causative agents: Candida, Aspergillus (in immunocompromised hosts)
- Treatment: Antifungal therapy, often prolonged
- Clinical Presentation:
- Fever, chest pain
- Tachycardia, sometimes arrhythmias
- Signs of tamponade in severe cases
- Diagnosis:
- Blood cultures and specific pathogen testing
- Pericardial fluid analysis in cases of effusion
- Echocardiography to assess for effusion and cardiac function
- Complications:
- Cardiac tamponade (especially in bacterial cases)
- Constrictive pericarditis (rare but can occur)
- Recurrent pericarditis (more common after viral infections)
Prompt recognition and appropriate treatment of infectious pericarditis in children is crucial to prevent complications and improve outcomes. The approach varies significantly depending on the causative pathogen.
Noninfectious Pericarditis in Children
Noninfectious pericarditis encompasses a diverse group of conditions causing pericardial inflammation without direct pathogen involvement. These forms are less common in children but can be associated with significant morbidity.
Key Features:
- Autoimmune Pericarditis:
- Associated with systemic autoimmune diseases
- Common causes: Systemic Lupus Erythematosus (SLE), Juvenile Idiopathic Arthritis (JIA)
- Treatment involves managing the underlying autoimmune condition
- Post-cardiac Injury Syndrome:
- Occurs after cardiac surgery or myocardial infarction (rare in children)
- Thought to be immune-mediated
- Treatment: NSAIDs, colchicine, sometimes corticosteroids
- Uremic Pericarditis:
- Occurs in children with end-stage renal disease
- Management includes dialysis intensification and sometimes pericardiocentesis
- Neoplastic Pericarditis:
- Rare in children, can occur with leukemia or lymphoma
- Treatment directed at the underlying malignancy
- Drug-induced Pericarditis:
- Rare in children but can occur with certain medications (e.g., hydralazine, procainamide)
- Management involves discontinuation of the offending drug
- Clinical Presentation:
- Can be similar to infectious pericarditis
- May have features of the underlying systemic disease
- Sometimes presents with large pericardial effusions
- Diagnosis:
- Based on clinical presentation and underlying condition
- Echocardiography to assess for pericardial effusion and cardiac function
- Laboratory tests to evaluate underlying conditions (e.g., ANA, RF for autoimmune diseases)
- Cardiac MRI can be helpful in assessing pericardial inflammation and thickening
- Pericardial fluid analysis when effusion is present and drainage is indicated
- Treatment:
- Tailored to the underlying cause
- NSAIDs often used as first-line treatment for pain and inflammation
- Colchicine may be added to reduce recurrence risk
- Corticosteroids used in refractory cases or when indicated by underlying condition
- Immunosuppressive agents may be necessary in autoimmune-related cases
- Pericardiocentesis for large or symptomatic effusions
- Prognosis:
- Generally good with appropriate management of underlying condition
- Risk of recurrence varies depending on etiology
- Some forms (e.g., recurrent pericarditis) may have a chronic course
- Complications:
- Cardiac tamponade in cases with large effusions
- Constrictive pericarditis (rare but can occur in chronic cases)
- Myocardial involvement leading to myopericarditis
- Follow-up:
- Regular cardiac evaluations, including echocardiography
- Monitoring of underlying condition and response to treatment
- Assessment for recurrence or development of chronic pericarditis
Noninfectious pericarditis in children represents a diverse group of conditions that often require a multidisciplinary approach for optimal management. The key to successful treatment lies in identifying and addressing the underlying cause while managing pericardial inflammation and its potential complications. Long-term follow-up is essential, especially in cases associated with systemic diseases or those at risk for recurrence.
Tuberculous Pericarditis in Children
Tuberculous pericarditis is a severe form of pericardial inflammation caused by Mycobacterium tuberculosis. While less common in developed countries, it remains a significant concern in areas with high TB prevalence.
Key Features:
- Epidemiology:
- More common in developing countries with high TB prevalence
- Can occur in immunocompromised children in any setting
- Often part of disseminated TB infection
- Pathophysiology:
- Usually results from reactivation of primary TB infection
- Spread to pericardium via lymphatic system or hematogenous route
- Can lead to pericardial effusion, granulomatous inflammation, and fibrosis
- Clinical Presentation:
- Often insidious onset with nonspecific symptoms
- Fever, night sweats, weight loss
- Chest pain, dyspnea
- Signs of cardiac tamponade in advanced cases
- May have signs of TB infection at other sites
- Diagnosis:
- High index of suspicion in endemic areas or risk groups
- Tuberculin skin test or Interferon-Gamma Release Assay (IGRA)
- Chest X-ray may show cardiomegaly or pleural effusions
- Echocardiography: Pericardial effusion, sometimes with fibrinous strands
- Pericardiocentesis with fluid analysis (elevated protein, low glucose, lymphocyte predominance)
- PCR and culture of pericardial fluid for M. tuberculosis
- Pericardial biopsy may be necessary in some cases
- Treatment:
- Standard anti-tuberculous therapy for at least 6 months
- Typically includes Isoniazid, Rifampicin, Pyrazinamide, and Ethambutol
- Corticosteroids often added to reduce inflammation and risk of constriction
- Pericardiocentesis for large or symptomatic effusions
- Pericardiectomy may be necessary in cases progressing to constriction
- Complications:
- Cardiac tamponade
- Constrictive pericarditis (occurs in up to 30-50% of cases)
- Myocardial involvement leading to cardiomyopathy
- Prognosis:
- Mortality can be high if untreated or diagnosed late
- Improved outcomes with early diagnosis and appropriate treatment
- Risk of long-term sequelae, particularly constrictive pericarditis
- Follow-up:
- Regular clinical and echocardiographic monitoring during treatment
- Long-term follow-up to assess for development of constrictive pericarditis
- Screening and treatment of family contacts as per local TB protocols
Tuberculous pericarditis in children represents a significant diagnostic and therapeutic challenge. Early recognition, prompt initiation of anti-tuberculous therapy, and appropriate management of complications are crucial for improving outcomes. In endemic areas, it should be considered in the differential diagnosis of any child presenting with pericardial disease.
Objective QnA: Pericarditis in Children
- What is pericarditis?
Inflammation of the pericardium, the protective sac surrounding the heart - What is the most common cause of acute pericarditis in children?
Viral infection - Which virus is most frequently associated with viral pericarditis in children?
Coxsackievirus - What is the classic triad of symptoms in acute pericarditis?
Chest pain, pericardial friction rub, and characteristic ECG changes - Which ECG finding is characteristic of acute pericarditis?
Diffuse ST-segment elevation - What is the most common complication of acute pericarditis?
Pericardial effusion - Which condition is characterized by inflammation of both the pericardium and myocardium?
Myopericarditis - What is the primary difference between pericardial effusion and cardiac tamponade?
Cardiac tamponade involves hemodynamic compromise due to pericardial fluid accumulation - Which imaging modality is considered the gold standard for diagnosing pericardial effusion?
Echocardiography - What is the first-line treatment for acute viral pericarditis in children?
NSAIDs (e.g., ibuprofen) - Which medication is often added to NSAIDs for treating recurrent pericarditis?
Colchicine - What is the most common cause of constrictive pericarditis in children?
Previous cardiac surgery - Which autoimmune disease can cause recurrent pericarditis in children?
Systemic lupus erythematosus (SLE) - What is the primary indication for pericardiocentesis in children with pericardial effusion?
Hemodynamic compromise (cardiac tamponade) - Which physical examination finding is characteristic of cardiac tamponade?
Pulsus paradoxus - What is the most common cause of purulent pericarditis in children?
Bacterial infection (e.g., Staphylococcus aureus) - Which malignancy is most commonly associated with malignant pericardial effusion in children?
Leukemia - What is the role of corticosteroids in treating pericarditis in children?
Reserved for refractory cases or specific etiologies (e.g., autoimmune) - Which imaging finding on echocardiography suggests cardiac tamponade?
Right atrial and ventricular diastolic collapse - What is the primary difference between acute and chronic pericarditis?
Duration of symptoms (acute <6 weeks, chronic >6 months) - Which laboratory test is most useful in diagnosing acute pericarditis?
Elevated C-reactive protein (CRP) - What is the potential long-term complication of recurrent pericarditis?
Constrictive pericarditis - Which procedure is used to diagnose constrictive pericarditis when non-invasive tests are inconclusive?
Cardiac catheterization - What is the primary treatment for constrictive pericarditis?
Pericardiectomy (surgical removal of the pericardium) - Which medication can cause drug-induced pericarditis in children?
Hydralazine - What is the role of pericardial biopsy in evaluating pericarditis?
To determine etiology in cases of recurrent or chronic pericarditis - Which imaging modality is best for evaluating pericardial thickness?
Cardiac MRI - What is the primary difference between exudative and transudative pericardial effusions?
Exudative effusions have higher protein content and are typically inflammatory - Which complication can occur after pericardiocentesis in children?
Cardiac chamber laceration - What is the role of NSAIDs in treating pericarditis associated with Kawasaki disease?
NSAIDs are contraindicated; treatment focuses on IVIG and aspirin
