Medscape is available in 5 Language Editions – Choose your Edition here.


Pediatric Malignant Pericardial Effusion Clinical Presentation

  • Author: Poothirikovil Venugopalan, MBBS, MD, FRCPCH; Chief Editor: Stuart Berger, MD  more...
Updated: Feb 04, 2014


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.[7, 8] 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.[9]


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) [10]
  • 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
Contributor Information and Disclosures

Poothirikovil Venugopalan, MBBS, MD, FRCPCH Consultant Pediatrician with Cardiology Expertise, Department of Child Health, Brighton and Sussex University Hospitals, NHS Trust; Honorary Senior Clinical Lecturer, Brighton and Sussex Medical School, UK

Poothirikovil Venugopalan, MBBS, MD, FRCPCH is a member of the following medical societies: Royal College of Paediatrics and Child Health, Paediatrician with Cardiology Expertise Special Interest Group, British Congenital Cardiac Association

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.


Hugh D Allen, MD Professor, Department of Pediatrics, Division of Pediatric Cardiology and Department of Internal Medicine, Ohio State University College of Medicine

Hugh D Allen, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Ira H Gessner, MD Professor Emeritus, Pediatric Cardiology

Ira H Gessner, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

  1. Castillo JJ, Shum H, Lahijani M, Winer ES, Butera JN. Prognosis in primary effusion lymphoma is associated with the number of body cavities involved. Leuk Lymphoma. 2012 Jun 13. [Medline].

  2. Thomas-de-Montpreville V, Nottin R, Dulmet E, Serraf A. Heart tumors in children and adults: clinicopathological study of 59 patients from a surgical center. Cardiovasc Pathol. 2007 Jan-Feb. 16(1):22-8. [Medline].

  3. Bien E, Stefanowicz J, Aleszewicz-Baranowska J, et al. [Cardio-vascular disorders at the time of diagnosis of malignant solid tumours in children--own experiences]. Med Wieku Rozwoj. 2005 Jul-Sep. 9(3 Pt 2):551-9. [Medline].

  4. McCurdy MT, Shanholtz CB. Oncologic emergencies. Crit Care Med. 2012 Jul. 40(7):2212-22. [Medline].

  5. Medary I, Steinherz LJ, Aronson DC, La Quaglia MP. Cardiac tamponade in the pediatric oncology population: treatment by percutaneous catheter drainage. J Pediatr Surg. 1996 Jan. 31(1):197-9; discussion 199-200. [Medline].

  6. Ben-Horin S, Bank I, Guetta V, Livneh A. Large symptomatic pericardial effusion as the presentation of unrecognized cancer: a study in 173 consecutive patients undergoing pericardiocentesis. Medicine (Baltimore). 2006 Jan. 85(1):49-53. [Medline].

  7. Devlieger R, Hindryckx A, Van Mieghem T, Debeer A, De Catte L, Gewillig M, et al. Therapy for foetal pericardial tumours: survival following in utero shunting, and literature review. Fetal Diagn Ther. 2009. 25(4):407-12. [Medline].

  8. Walsh MA, Carcao M, Pope E, Lee KJ. Kaposiform hemangioendothelioma presenting antenatally with a pericardial effusion. J Pediatr Hematol Oncol. 2008 Oct. 30(10):761-3. [Medline].

  9. 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. [Medline].

  10. Singh J, Rana SS, Kaur A, Srivastava V, Singh H, Sharma R. Intrapericardial teratoma presenting as recurrent pericardial tamponade: report of a case. Surg Today. 2009. 39(8):700-4. [Medline].

  11. Karatolios K, Pankuweit S, Moosdorf RG, Maisch B. Vascular endothelial growth factor in malignant and benign pericardial effusion. Clin Cardiol. 2012 Jun. 35(6):377-81. [Medline].

  12. Luna A, Ribes R, Caro P, et al. Evaluation of cardiac tumors with magnetic resonance imaging. Eur Radiol. 2005 Jul. 15(7):1446-55. [Medline].

  13. Palma JH, Gaia DF, Guilhen JC, Branco JN, Buffolo E. Video-thoracoscopic pericardial drainage in the treatment of pericardial effusions. Rev Bras Cir Cardiovasc. 2009 Mar. 24(1):44-9. [Medline].

  14. Georghiou GP, Stamler A, Sharoni E, et al. Video-assisted thoracoscopic pericardial window for diagnosis and management of pericardial effusions. Ann Thorac Surg. 2005 Aug. 80(2):607-10. [Medline].

  15. Puri A, Agarwal N, Dwivedi SK, Narain VS. Percutaneous balloon pericardiotomy for the treatment of recurrent malignant pericardial effusion. Indian Heart J. 2012 Jan-Feb. 64(1):88-9. [Medline].

  16. Jones DA, Jain AK. Percutaneous balloon pericardiotomy for recurrent malignant pericardial effusion. J Thorac Oncol. 2011 Dec. 6(12):2138-9. [Medline].

  17. Nieh S, Chen SF, Fu E, et al. Detection of the human telomerase RNA component by in situ hybridization in cells from body fluids. Acta Cytol. 2005 Jan-Feb. 49(1):31-7. [Medline].

  18. Politi E, Kandaraki C, Apostolopoulou C, et al. Immunocytochemical panel for distinguishing between carcinoma and reactive mesothelial cells in body cavity fluids. Diagn Cytopathol. 2005 Mar. 32(3):151-5. [Medline].

Plain chest radiograph from 3-month-old infant with pneumonia and malignant pericardial effusion, showing cardiomegaly and bilateral pneumonic patches.
Two-dimensional echocardiograph from subcostal window, showing large pericardial effusion.
M-mode echocardiograph from child with pericardial effusion.
Cytologic features of malignant pericardial effusion. Smear of centrifuged pericardial fluid from patient with malignant pericardial involvement from lymphoma. Low-power view showing numerous mononuclear cells along with large atypical malignant cells.
Cytologic features of malignant pericardial effusion. Smear of centrifuged pericardial fluid from patient with malignant pericardial involvement from lymphoma. High-power view showing morphologic details of malignant cells. These cells are large and show oval hyperchromatic nuclei, some of them having nucleoli. Cytoplasm is reduced to thin rim.
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.