eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology
Pericardial Effusion, Malignant
Updated: Jul 24, 2008
Introduction
Background
Pericardial involvement in patients with malignancy is common. Widespread use of noninvasive diagnostic techniques, such as echocardiography and CT scanning, has increased awareness of this diagnosis. The mere presence of pericardial effusion does not necessarily imply pericardial infiltration by malignant cells.
Pathophysiology
The pericardium consists of 2 layers, the visceral pericardium (epicardium) and the parietal pericardium, which enclose a potential space (ie, the pericardial cavity) between them. This cavity is normally lubricated by a very small amount of serous fluid (<30 mL in adults). Inflammation of the pericardium or obstruction of lymphatic drainage from the pericardium of any etiology causes an increase in fluid volume, referred to as a pericardial effusion.
Malignant involvement of the pericardium may be primary (less common) or secondary to spread from a nearby or distant focus of malignancy. Secondary neoplasms can involve the pericardium by contiguous extension from a mediastinal mass, nodular tumor deposits from hematogenous or lymphatic spread, and diffuse pericardial thickening from tumor infiltration (with or without effusion). In diffuse pericardial thickening, the heart may be encased by an effusive-constrictive pericarditis.
Other rare mechanisms include chronic myelomonocytic leukemia and intrapericardial extramedullary hematopoiesis with preleukemic conditions or during blast crisis in chronic myeloid leukemia. Obstruction of lymphatic drainage by mediastinal tumors, either benign or malignant, can also give rise to pericardial effusion, which can be chylous. The above mechanisms may act independently or jointly in any particular child with malignancy. The underlying myocardium is not involved in most patients.
Pathogenesis of clinical manifestations
In healthy individuals, the pericardium does not limit filling of the cardiac chambers either at rest or during exercise. When pericardial effusion occurs, chamber capacity may be reduced. Venous return may be severely limited and, therefore, cardiac output may be severely limited. Capacity of the pericardial space is influenced by its natural stiffness. Rapid accumulation of fluid is poorly tolerated, whereas slow accumulation may allow large amounts of pericardial fluid to collect without producing symptoms. With increased pressure within the pericardial space, filling pressure in all chambers of the heart is elevated. In advanced stages, right and left atrial mean pressures and right and left ventricular end-diastolic pressures are virtually identical to the intrapericardial pressure. Therefore, the clinical features result from limitation of cardiac output and elevated venous pressures.
Pulsus paradoxus
In healthy individuals, inspiration causes the systolic blood pressure to fall slightly as a result of the greater volume of blood accommodated by the pulmonary vascular bed. This occurs despite inspiratory increase in venous return to the right heart. In cardiac tamponade, right ventricular filling is maintained at the expense of restricted left ventricular filling, and the systolic blood pressure falls further (>10 mm Hg). This exaggerated fall in systolic blood pressure with inspiration is referred to as pulsus paradoxus. This is an important sign of cardiac tamponade, although, occasionally, severe respiratory distress of any cause (asthma, emphysema, pleural effusion) may give rise to this sign.
Frequency
United States
Pericardial effusion is a common cause of pericarditis, occurring in approximately 5-15% of patients with malignant neoplasms, according to autopsy data.
Most cardiac tumors in infants and children are benign (eg, rhabdomyoma, fibromas) and are rarely associated with pericardial involvement.1
International
A study of 236 children in Poland reported cardiac involvement in 15% of children, including pericardial effusion in 7% of children.2
Mortality/Morbidity
Children with pericardial involvement due to malignancy have more extensive disease and, hence, a worse prognosis; pericardial tamponade may add to the mortality unless promptly detected and appropriately treated.2,3
Sex
Both sexes are affected. Medary et al reported higher incidence in males than in females at a ratio of 7:3.4
Age
All ages are affected, but pericardial effusion is more common in older children and adolescents. Medary et al reported a mean age of 14 y.4 This may be related to the longer survival of older children with malignancy.
Clinical
History
- Pericardial malignancy is often asymptomatic. It is observed on chest radiography performed to evaluate the lungs or diagnosed as an incidental finding at autopsy.
- Although pericardial malignancy may be reported as an incidental finding, it may have contributed to the symptomatology and even death. A review of some cases leads to the conclusion that symptoms may be incorrectly attributed to the underlying neoplasm.
- Shortness of breath or dyspnea 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. Orthopnea may be present.
- Primary cardiac malignancy presents as unresponsive heart failure.
- Cardiac tamponade may rarely be the initial manifestation of systemic malignancy.
Physical
- Central venous pressure is increased.
- Jugular venous pressure is elevated and jugular veins are not pulsatile.
- The liver may be enlarged, and peripheral edema and ascites may be present.
- Encountering evidence of pulmonary edema is unusual because pericardial effusion limits the amount of blood that can enter the heart, and the left atrial pressure does not exceed the right atrial pressure.
- Heart sounds may be distant or faint.
- Pericardial friction rub may be observed.
- A sign of pericardial inflammation is a grating, scratching sound caused by abrading of inflamed pericardial surfaces with cardiac motion.
- Pericardial friction rub may have as many as 3 components.
- In the presence of a large effusion, heart sounds may be muffled, and the rub may disappear.
- It is best heard in the second-fourth intercostal spaces along the left sternal border or along the mid clavicular line and is loudest in the upright position with the patient leaning forward.
- It is often accentuated in inspiration.
- The occasional persistence of a rub in pericardial tamponade is believed to represent a friction between the inflamed parietal pericardium and the pleura.
- Ewart sign may be observed.
- This refers to subscapular dullness to percussion. It represents compression of the left lung by a massively enlarged heart and may be associated with abnormal breath sounds in that region.
- No crepitations or rhonchi are heard.
- Cardiac tamponade features are as follows:
- Low cardiac output
- Elevated central venous pressures
- Paradoxical pulse
- Muffled or diminished heart sounds
- Tachycardia
- Jugular venous distension reflecting high central venous pressure
- Low systolic blood pressure and low pulse pressure
- Pulsus alternans may be present.
- This consists of a drop in systolic blood pressure in alternate beats, another ominous sign.
- This sign is most reliably documented while observing intraarterial blood pressure tracing, rather than by palpating the pulse itself.
- Congenital intrapericardial tumors may be associated with fetal hydrops secondary to compression of fetal venous structures.
Causes
- Primary malignant neoplasms
- Pericardial mesothelioma
- Fibrosarcoma
- Angiosarcoma
- Liposarcoma
- Lymphoma
- Malignant pericardial teratoma
- Rhabdomyosarcoma with tuberous sclerosis
- Pheochromocytoma
- Kaposi sarcoma and primary cardiac lymphoma in association with human immunodeficiency virus (HIV) infection
- Intrapericardial teratoma in the fetus and neonate
- Metastatic or infiltrative disease
- Non-Hodgkin lymphoma
- Neuroblastoma
- Ganglioneuroblastoma
- Pheochromocytoma
- Sarcomas
- Wilms tumor
- Hodgkin lymphoma
- Primary mediastinal (thymic) B-cell lymphoma
- Adenocarcinoma
- Mesothelioma
- Pulmonary lymphangiomatosis
- Malignant fibrous histiocytomas
- Leiomyosarcomas
- Liposarcomas
- High-grade sarcomas
- Burkitt lymphoma
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Further Reading
Keywords
pericardial effusion, malignant pericardial effusion, dropsy of pericardium, mediastinal mass, nodular tumor deposits, lymphatic spread, diffuse pericardial thickening from tumor infiltration, chronic myelomonocytic leukemia, intrapericardial extramedullary hematopoiesis, preleukemic conditions, blast crisis, chronic myeloid leukemia, obstruction of lymphatic drainage, pericarditis, effusive-constrictive pericarditis, cardiac tamponade, asthma, emphysema, pleural effusion, rhabdomyoma, fibroma, heart failure, fetal hydrops, hydrops fetalis, pericardial mesothelioma, angiosarcoma, liposarcoma, lymphoma, rhabdomyosarcoma, tuberous sclerosis, pheochromocytoma, Kaposi sarcoma, HIV infection, non-Hodgkin lymphoma, neuroblastoma, ganglioneuroblastoma, Wilms tumor, Hodgkin lymphoma, adenocarcinoma, leiomyosarcoma, Burkitt lymphoma
Overview: Pericardial Effusion, Malignant