Pediatric Malignant Pericardial Effusion Clinical Presentation

Updated: Jul 03, 2019
  • Author: Poothirikovil Venugopalan, MBBS, MD, FRCPCH; Chief Editor: Syamasundar Rao Patnana, MD  more...
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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. [9, 10] 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. In rare instances, cardiac tamponade may be the initial manifestation of systemic malignancy. Infrequently, pericardial malignancy may present as superior venacaval syndrome, either due to a coexisting tumor mass or just resulting from the rapid accumulation of pericardial effusion. [11]


Physical Examination

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 through fourth intercostal spaces along the left sternal border or along the midclavicular line, and it is loudest in the upright position with the patient leaning forward. The rub is often accentuated in inspiration.

The occasional persistence of a rub in pericardial tamponade is believed to reflect friction between the inflamed parietal pericardium and the pleura.

Ewart sign—that is, subscapular dullness to percussion—may be observed. This sign reflects 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.

Characteristic features of cardiac tamponade are as follows:

  • Low cardiac output

  • Elevated central venous pressures

  • Paradoxical pulse

  • Muffled or diminished heart sounds

  • Tachycardia

  • Jugular venous distention 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 by observing intra-arterial blood pressure tracings, rather than by palpating the pulse itself.

Congenital intrapericardial tumors may be associated with fetal hydrops secondary to compression of fetal venous structures.



Complications of pediatric malignant pericardial effusion include the following:

  • Cardiac tamponade (mostly occurring in infants but also reported in older children) [12]

  • Sudden death

  • Retinoic acid syndrome – Retinoic acid syndrome is characterized by fever and respiratory distress, along with weight gain, pleural or pericardial effusions, peripheral edema, thromboembolic events, and intermittent hypotension; these are related to all trans- retinoic acid therapy for underlying malignancy

  • Leukemic coronary artery occlusion