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:
    • 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.
    • 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.
  • 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 failuremalignancy, 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.
  • 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.
  • 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.
  • 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.
  • 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.
  • 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.
  • 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
    1. Morris P, Warriner D, Morton A. Eureka: Cardiovascular Medicine. Scion Publishing Ltd; 2015.
    2. Sattar HA. Fundamentals of pathology : medical course and step 1 review. Chicago, Illinois: Pathoma.com; 2024.

Last Updated: February 2025