Pericardial Effusion and Tamponade

Pericardial involvement in patients with malignancy is common. Widespread use of non-invasive diagnostic techniques, such as echocardiography and computed tomography (CT) scanning, has increased awareness of this diagnosis. The mere presence of pericardial effusion does not necessarily imply pericardial infiltration by malignant cells.

Pericardial malignancy is often asymptomatic. It is observed on chest radiography performed to evaluate the lungs or diagnosed as an incidental finding at autopsy. It can also present in antenatal scans, especially with fetal teratoma. Although pericardial malignancy may be reported as an incidental finding, it may have contributed to the symptoms and even to death.

Medical care is dictated mainly by the general condition of the patient and the underlying malignancy. The safety and effectiveness of surgical drainage of pericardial fluid via pericardiectomy (complete or partial) or the creation of a pericardial window are well recognized. Further inpatient care is determined by the underlying condition. Further outpatient care is often required to look for evidence of constrictive pericarditis. 

In diffuse pericardial thickening, the heart may be encased by an effusion-constrictive pericarditis.

In addition to the more common mechanisms, there are rare occurrences involving conditions such as chronic myelomonocytic leukemia and intra-pericardial extramedullary hematopoiesis, often associated with preleukemic states or during blast crisis in chronic myeloid leukemia. Furthermore, obstruction of lymphatic drainage by mediastinal tumors, whether benign or malignant, can lead to the development of pericardial effusion, with some cases presenting as chylous effusion.

These mechanisms may manifest independently or concurrently in certain pediatric patients with malignancies, adding complexity to their clinical presentation. Importantly, the underlying myocardium remains unaffected in the majority of cases, highlighting the diverse range of mechanisms contributing to the pathophysiology of mediastinal masses and their associated complications. Understanding these rare mechanisms is crucial for comprehensive management and optimal outcomes in affected children.

Aetiology

Clinical presentation

Shortness of breath or dyspnoea is the most common symptom (85%). Other manifestations may include chest pain, shoulder pain, and a hacking cough that varies with posture. Sitting up and leaning forward improves the cough. Orthopnoea may be present.

Primary cardiac malignancy presents as unresponsive heart failure. In rare instances, cardiac tamponade may be the initial manifestation of systemic malignancy. Infrequently, pericardial malignancy may present as superior vena cava syndrome, either due to a coexisting tumor mass or just resulting from the rapid accumulation of pericardial effusion.

Characteristic features of cardiac tamponade

• Low cardiac output.
• Elevated central venous pressure.
• Paradoxical pulse.
• Muffled or diminished heart sounds.
• Tachycardia.
• Jugular venous distension reflecting high central pressure.
• Low systolic blood pressure and low pulse pressure.
• Hepatomegaly.
• Peripheral edema and ascites.

Imaging

The presence of a pericardial effusion can be suspected based on chest radiograph findings which classically show an enlarged cardiac silhouette.

The classic appearance of a “water bottle heart” that is globular in appearance is a sensitive but nonspecific finding.

An electrocardiogram may reveal low-voltage waveforms and less frequently electrical alternans, a condition where consecutive QRS complexes alternate in height between beats.

Echocardiography, however, has become the preferred diagnostic test for assessing pericardial effusion and cardiac tamponade. It should be ordered when there is a significant pericardial effusion suspected, as it can not only define the size and location of an effusion but also assess the hemodynamic significance and help guide pericardiocentesis.

Treatment

The approach to managing pericardial effusion is contingent upon the severity of the presenting symptoms. For cases where the effusion is asymptomatic, vigilant monitoring for any hemodynamic complications is advised, although such effusions often resolve with the treatment of the primary malignancy.

Should the effusion lead to tamponade or present a significant hemodynamic challenge, immediate fluid drainage is imperative.

Pericardiocentesis, performed under echocardiographic guidance, is the preferred treatment modality, offering a safe and efficacious solution for pediatric oncology patients manifesting symptoms of pericardial effusion or tamponade.

Beyond symptom alleviation, pericardiocentesis facilitates the determination of the effusion’s malignant etiology. Given the potential for a recurrence rate up to 50% following initial drainage, the implementation of an indwelling pigtail drainage catheter during pericardiocentesis for extended drainage is advisable.

Surgical intervention for malignant pericardial effusion is considered a last resort, applicable only in instances where other methods prove ineffective.

In scenarios of acute cardiac tamponade, administering volume resuscitation to surpass pericardial pressures with higher intracardiac pressures is crucial for patient stability.

Mechanical ventilation with positive airway pressure should be avoided in these patients because it can further decrease cardiac output.

References

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• Tsai MH, Yang CP, Chung HT, Shih LY. Acute myeloid leukemia in a young girl presenting with mediastinal granulocytic sarcoma invading pericardium and causing superior vena cava syndrome. J Pediatr Hematol Oncol. 2009 Dec. 31(12):980-2. 
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• Blausen.com staff (2014). Blausen.com staff (2014). Medical gallery of Blausen medical.