Pericardial Disease & Cardiac Tumours
Acute Pericarditis
1. Acute Pericarditis
1.1 Epidemiology
- Incidence:
- Acute pericarditis accounts for approximately 5% of all acute chest pain admissions.
- Clinical Relevance:
- Despite its similarity in presentation to acute coronary syndromes, pericarditis is managed differently.
1.2 Aetiology
- Primary (Idiopathic) Pericarditis:
- The cause often remains unknown.
- Frequently follows a viral infection by several weeks.
- Secondary Causes:
- Trauma (e.g. seatbelt injury)
- Uraemia (in renal failure)
- Myocardial Infarction (Dressler’s syndrome)
- Other infections: Viral (e.g. Epstein–Barr virus, CMV, HIV, Coxsackie virus), bacterial (e.g. tuberculosis, rheumatic fever), fungal.
- Autoimmune and Inflammatory Disorders: Rheumatoid arthritis, systemic lupus erythematosus, radiation.
- Surgical Causes: Post-coronary artery bypass graft surgery.
1.3 Risk Factors
- Preceding Infection: A recent viral illness is a recognised trigger.
- Uraemia: Seen in patients with renal failure.
- Post-Myocardial Infarction: Dressler’s syndrome is a recognised complication.
- Autoimmune Disorders: Conditions such as rheumatoid arthritis and lupus increase risk.
- Post-Surgical Status: Cardiac surgery may precipitate pericardial inflammation.
1.4 Symptoms
- Chest Pain:
- Typically pleuritic in nature – a sharp, stabbing or burning pain.
- Pain is exacerbated by lying flat and relieved by sitting upright (due to the heart’s position within the pericardial sac).
- Respiratory Symptoms:
- Patients are often tachypnoeic and take shallow, rapid breaths to avoid painful deep inspiration.
- Associated Features:
- A pericardial rub may be heard on auscultation along the lower left sternal edge.
- If myocardial involvement occurs (myopericarditis), signs of heart failure may be present.
1.5 Diagnosis
- Clinical History and Examination:
- Diagnosis is primarily based on the characteristic chest pain and positional nature of the pain.
- A pericardial rub is an important physical finding.
- Blood Tests:
- Inflammatory markers such as C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and white cell count are typically elevated.
- Cardiac enzymes (e.g. troponin) may be raised if there is concomitant myocardial involvement.
- Electrocardiography (ECG):
- If the myocardium is involved, the ECG may show:
- Concave, ‘saddle-shaped’ ST-segment elevation.
- Tall T waves.
- PR-segment depression (especially if the atrial myocardium is affected).
- Importantly, the ST-segment elevation in pericarditis is diffuse and not confined to a single coronary territory.
- If the myocardium is involved, the ECG may show:
- Chest X-ray:
- An enlarged, globular cardiac silhouette may indicate a pericardial effusion.
- Echocardiography:
- Performed in all cases to exclude or assess for pericardial effusion and to evaluate myocardial systolic function.
1.6 Immediate Management
- Idiopathic Pericarditis:
- Reassurance and Analgesia: Patient education and symptomatic relief.
- Non-Steroidal Anti-Inflammatory Drugs (NSAIDs):
- Common agents include aspirin or colchicine.
- Rest: Advising the patient to limit physical activity during the acute phase.
- Pericarditis with Effusion:
- Pericardiocentesis is indicated when there is evidence of cardiac tamponade (a clinical emergency) or when diagnostic analysis of the fluid is needed.
- Myocarditis Involvement:
- If myocardial involvement is suspected, a cardiac MRI should be performed to assess the degree of myocardial inflammation and damage.
- These patients may require heart failure therapy and closer monitoring.
1.7 Long-Term Management
- Recurrence Prevention:
- Colchicine is often used to treat recurrent pericarditis.
- Monitoring:
- Regular follow-up is required to monitor for the development of pericardial scarring or constrictive pericarditis, which can impair diastolic function.
- Management of Underlying Causes:
- If pericarditis is secondary, treat the underlying condition (e.g. dialysis for uraemia, antimicrobials for tuberculosis).
- Follow-Up Imaging:
- Periodic echocardiography may be necessary to monitor for changes in effusion size or myocardial function.
Pericardial Effusion
2. Pericardial Effusion
2.1 Epidemiology
- Uncommon Condition: Pericardial effusion is an infrequent clinical finding.
- Clinical Context: It may occur in various settings, including following invasive cardiac procedures where patients become acutely unwell with low blood pressure.
2.2 Aetiology
- Definition: The condition is defined as the collection of fluid in the space between the visceral and parietal pericardial layers.
- Multiple Causes:
- Acute pericarditis (idiopathic or post-viral)
- Myocardial infarction
- Trauma
- Post-cardiac surgery or intervention (e.g. CABG, EP, PCI)
- Aortic dissection
- Malignancy
- Renal failure (uraemic effusion)
- Infection:
- Viral (e.g. Coxsackievirus, Echovirus, Cytomegalovirus, HIV)
- Bacterial (e.g. Tuberculosis)
- Autoimmune diseases (e.g. rheumatoid arthritis, systemic lupus erythematosus)
2.3 Risk Factors
- Underlying Pathologies: Risk of pericardial effusion increases with conditions that cause pericardial inflammation or injury, such as:
- Recent viral infections leading to pericarditis.
- Cardiac surgery or interventional procedures.
- Systemic conditions (e.g. renal failure, malignancy, autoimmune disorders).
- Haemodynamic Factors:
- The rate of fluid accumulation is critical; a rapid, low-volume accumulation may precipitate cardiac tamponade, whereas a slowly developing effusion can reach large volumes without immediate haemodynamic compromise.
2.4 Symptoms
- Small or Chronic Effusions:
- Patients are often asymptomatic.
- Large Effusions:
- Dyspnoea and chest pain may develop.
- Cardiac Tamponade:
- When the effusion acutely compromises cardiac filling, patients may present in extremis or following cardiac arrest.
2.5 Diagnosis
- Clinical Assessment:
- History and Examination: Consider pericardial effusion in patients with recent invasive cardiac procedures who become acutely unwell with hypotension.
- Physical Findings:
- Elevated jugular venous pressure (JVP)
- Hypotension
- An impalpable apex beat
- Tachycardia and tachypnoea
- Pulsus paradoxus (abnormal decrease in pulse volume on inspiration)
- Kussmaul’s sign (abnormal rise in JVP with inspiration)
- Electrocardiography (ECG):
- Small QRS Complexes: Due to the insulating effect of the pericardial fluid.
- Electrical Alternans: The cardiac axis alternates as the heart swings within a large effusion.
- Chest X-Ray:
- Shows an enlarged, globular cardiac silhouette.
- Echocardiography:
- Confirmation of the diagnosis is made by demonstrating the fluid collection between the visceral and parietal pericardium.
- Assesses the hemodynamic impact, such as evidence of tamponade.
2.6 Immediate Management
- Management Depends on Severity:
- Cardiac Tamponade:
- Requires emergency pericardiocentesis to drain the fluid.
- It is not unusual to drain more than 2 litres from a large effusion.
- Stable Patients with Small Effusions:
- Managed conservatively with diuretic therapy.
- Cardiac Tamponade:
- Additional Considerations:
- Management should also focus on treating the underlying cause (e.g. addressing infection, managing renal failure, or post-surgical care).
2.7 Long-Term Management
- Monitoring:
- Patients managed conservatively are followed with serial echocardiography until the effusion resolves.
- Underlying Cause Treatment:
- Definitive management is directed at the underlying aetiology to prevent recurrence.
- Follow-Up:
- Regular clinical reviews to assess symptoms and any changes in haemodynamic status.
2.8 Complications
- Cardiac Tamponade:
- Life-threatening haemodynamic compromise due to rapid accumulation of fluid.
- Progressive Haemodynamic Deterioration:
- Even a small, rapidly accumulating effusion can cause significant compromise.
- Chronic Effusions:
- May lead to constrictive pericarditis if scarring and fibrosis develop over time.
Cardiac Tumours
3. Cardiac Tumours
3.1 Epidemiology
- Rarity: All primary cardiac tumours are rare.
- Classification:
- Benign tumours (e.g. myxomas, rhabdomyomas, lipomas, fibro‑mas) are more common than primary malignant tumours and are generally not life‑threatening.
- Primary malignant tumours (e.g. rhabdomyosarcomas, angiosarcomas, myxosarcomas, fibrosarcomas, leiomyosarcomas) are approximately four times rarer than benign types.
- Secondary tumours (metastases) are almost always malignant and carry a very poor prognosis.
- Cardiac Myxoma Specifics:
- Occurs in approximately 1 in 5000 adults.
- Affects about 75% women.
- Most frequently arises in the left atrium.
3.2 Aetiology
- Primary Tumours:
- Benign Tumours:
- Myxomas: Benign mesenchymal tumours with a gelatinous appearance and abundant ground substance on histology.
- Other benign tumours include rhabdomyomas (most common in children, often associated with tuberous sclerosis), lipomas and fibromas.
- Malignant Tumours:
- Primary malignant tumours such as rhabdomyosarcomas, angiosarcomas, myxosarcomas, fibrosarcomas and leiomyosarcomas.
- Benign Tumours:
- Secondary Tumours:
- Result from metastases from distant primary sites (e.g. breast carcinoma, lung carcinoma, melanoma, lymphoma).
- These tumours are malignant and generally have a very poor prognosis.
3.3 Risk Factors
- For Primary Benign Tumours (e.g. Myxoma):
- Female gender
- For Primary Malignant Tumours:
- Family history of cardiac tumours (genetic predisposition)
- Advanced age
- Chest irradiation
- Certain genetic syndromes
- For Secondary (Metastatic) Tumours:
- Presence of an aggressive primary cancer (e.g. breast or lung carcinoma, melanoma, lymphoma) is the key risk factor.
3.4 Symptoms
- General Presentation:
- Many cardiac tumours are asymptomatic and discovered incidentally during echocardiography.
- Constitutional Symptoms (common in myxomas):
- Weight loss, malaise, fever, and night sweats.
- Local Effects:
- A tumour may interfere with valve function, causing a murmur.
- In the case of a cardiac myxoma, a characteristic ‘diastolic plop’ may be heard if a semi‑mobile mass prolapses through the mitral valve annulus, potentially causing syncope.
- Other Presentations:
- Rhabdomyomas in children may present with arrhythmias or signs of heart failure and are often associated with tuberous sclerosis.
- Malignant Tumours:
- May present with signs of systemic spread and are more likely to recur after treatment.
3.5 Diagnosis
- Imaging Modalities:
- Echocardiography:
- The primary investigation for cardiac tumours.
- Transthoracic echo typically identifies a mobile, pedunculated mass, most commonly in the left atrium (e.g. myxoma).
- Transoesophageal echo provides greater detail regarding the size and attachment site of the tumour.
- CT and MRI Scanning:
- Used to define the tumour’s extent, location, and degree of invasiveness.
- Echocardiography:
- Histological Examination:
- May be required for definitive diagnosis, particularly to distinguish between benign and malignant tumours.
- For example, myxomas show a benign mesenchymal appearance with abundant ground substance.
3.6 Immediate Management
- Benign Tumours:
- Surgical Removal:
- Indicated for symptomatic tumours, particularly if they interfere with valve function (e.g. causing a diastolic plop or syncope).
- Once removed, benign tumours rarely recur.
- Surgical Removal:
- Malignant Tumours:
- Palliative Management:
- Treatment is usually palliative due to the tendency of malignant tumours to spread and recur.
- Resection is often not curative, and postoperative mortality is high.
- Palliative Management:
- Secondary Tumours:
- Generally managed as part of the overall treatment of the primary malignancy, with palliative care measures due to their poor prognosis.
3.7 Long-Term Management
- Follow-Up:
- Benign Tumours:
- After surgical excision, regular follow-up is advised to monitor for recurrence, although recurrence is rare.
- Malignant and Secondary Tumours:
- Long-term management is palliative and focuses on symptom control and maintaining quality of life.
- Benign Tumours:
- Multidisciplinary Approach:
- Involvement of cardiology, cardiothoracic surgery, oncology, and palliative care teams is essential for the management of malignant and metastatic cardiac tumours.
3.8 Complications
- For Benign Tumours:
- Local effects such as obstruction of the valve (e.g. mitral valve) causing syncope or heart failure.
- Embolisation of tumour fragments can cause systemic emboli.
- For Malignant Tumours:
- Rapid local invasion and metastasis, with a high likelihood of recurrence even after surgical removal.
- Secondary Tumours:
- Due to metastatic spread, complications are related to the primary cancer and often result in poor outcomes.
Written by Dr Ahmed Kazie MD, MSc
- References
- Morris P, Warriner D, Morton A. Eureka: Cardiovascular Medicine. Scion Publishing Ltd; 2015.
- Sattar HA. Fundamentals of pathology : medical course and step 1 review. Chicago, Illinois: Pathoma.com; 2024.
Last Updated: February 2025