History

Classic symptoms of pulmonary embolism (PE) are rarely encountered. The frequency with which the diagnosis is missed in both adults and children is striking. Adding to the clinical dilemmas is the fact that few symptoms are sensitive or specific for pulmonary embolism. In adult series, clinical diagnosis has a sensitivity of 85% but a specificity of 38%, reflecting the vast differential diagnosis found in both adults and children. Symptoms vary according to the severity of the pulmonary embolism and the presence of underlying conditions. Pulmonary emboli of small-to-moderate size are generally asymptomatic. Case series of pediatric emergency department presentations for PE showed that the most common risk factors included BMI > 25, oral contraceptive use, and previous history of thrombus without PE.^[15]

Respiratory symptoms

Pleuritic chest pain is reported to occur in as many as 84% of children and adults with pulmonary emboli. Its presence suggests that the embolus is located more peripherally and, thus, may be smaller.

Tachypnea and dyspnea are observed in as many as 60% of adult patients with pulmonary emboli but are generally less frequent in children.

Cough is present in approximately 50% of children with pulmonary emboli. Hemoptysis is a feature in a minority of children with pulmonary emboli, occurring in about 30% of cases.

Other symptoms

A feeling of apprehension is a manifestation of arousal of the sympathetic system. Sweating and syncope are rarely present.

Risk factors to elicit on history taking

Obtain a detailed history of any previous pulmonary embolism/thromboembolism, oral contraceptive use, recent pregnancy, termination of pregnancy, drug history, and family history.

Sickle cell disease

Patients with sickle cell disease may present with manifestations of sickle cell anemia other than acute chest syndrome. These may include anemia, sequestration crisis, pain crisis, stroke, and priapism.

Physical Examination

The use of physical findings as a diagnostic aid in suspected cases of pulmonary embolism brings the same problems as are outlined in History. Many physical findings are typically less marked than they are in adults, presumably because children have greater hemodynamic reserve and, thus, are better able to tolerate the significant hemodynamic and pulmonary changes.

Pulmonary findings include the following:

Tachypnea is a feature in almost 50% of children with pulmonary emboli.
Crackles are heard in a minority of cases.
Cyanosis and hypoxemia are not prominent features of pulmonary embolism. If present, cyanosis suggests a massive embolism leading to a marked V/Q mismatch and systemic hypoxemia. Some case reports have described massive pediatric pulmonary embolism with normal saturation.
A pleural rub is often associated with pleuritic chest pain and indicates an embolism in a peripheral location in the pulmonary vasculature.
Signs that indicate pulmonary hypertension and right ventricular failure include a loud pulmonary component of the second heart sound, right ventricular lift, distended neck veins, and hypotension. An increase in pulmonary artery pressure is reportedly not evident until at least 60% of the vascular bed has been occluded.

Cardiovascular findings include the following:

A gallop rhythm signifies ventricular failure.
Peripheral edema is a sign of congestive heart failure.
Various heart murmurs may be audible, including a tricuspid regurgitant murmur signifying pulmonary hypertension.

Other signs include the following:

Fever is an unusual sign that is nonspecific.
Diaphoresis is a manifestation of sympathetic arousal.
Signs of other organ involvement in patients with sickle cell disease would be elicited, such as sequestration crisis, priapism, anemia, and stroke.

Complications

Complications of pulmonary embolism include the following:

Death
Hemorrhage
Heparin-induced thrombocytopenia
Thrombophlebitis

Differential Diagnoses

Ak K, Birkan Y, Akalın F, Gunay D. Salvage pulmonary thromboembolectomy for massive pulmonary embolism in a child presented with syncope and subsequent cardiac arrest. Cardiol Young. 2019 Aug. 29(8):1094-6. [QxMD MEDLINE Link].
Kalaniti K, Lo Rito M, Hickey EJ, Sivarajan VB. Successful pulmonary embolectomy of a saddle pulmonary thromboembolism in a preterm neonate. Pediatrics. 2015 May. 135(5):e1317-20. [QxMD MEDLINE Link]. [Full Text].
Zaidi AU, Hutchins KK, Rajpurkar M. Pulmonary embolism in children. Front Pediatr. 2017. 5:170. [QxMD MEDLINE Link]. [Full Text].
Morgan RW, Stinson HR, Wolfe H, et al. Pediatric in-hospital cardiac arrest secondary to acute pulmonary embolism. Crit Care Med. 2018 Mar. 46(3):e229-e234. [QxMD MEDLINE Link]. [Full Text].
Ivy DD, Abman SH, Barst RJ, et al. Pediatric pulmonary hypertension. J Am Coll Cardiol. 2013 Dec 24. 62(25 Suppl):D117-26. [QxMD MEDLINE Link]. [Full Text].
David M, Andrew M. Venous thromboembolic complications in children. J Pediatr. 1993 Sep. 123(3):337-46. [QxMD MEDLINE Link].
Biss TT, Brandao LR, Kahr WH, Chan AK, Williams S. Clinical features and outcome of pulmonary embolism in children. Br J Haematol. 2008 Sep. 142(5):808-18. [QxMD MEDLINE Link].
Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 2000 Jun 22. 342(25):1855-65. [QxMD MEDLINE Link].
Cook A, Shackford S, Osler T, et al. Use of vena cava filters in pediatric trauma patients: data from the National Trauma Data Bank. J Trauma. 2005 Nov. 59(5):1114-20. [QxMD MEDLINE Link].
van den Heuvel-Eibrink MM, Lankhorst B, Egeler RM, Corel LJ, Kollen WJ. Sudden death due to pulmonary embolism as presenting symptom of renal tumors. Pediatr Blood Cancer. 2008 May. 50(5):1062-4. [QxMD MEDLINE Link].
Dollery CM. Pulmonary embolism in parenteral nutrition. Arch Dis Child. 1996 Feb. 74(2):95-8. [QxMD MEDLINE Link].
Kimhi G, Rubinshtein M, Tirosh-Wagner T, Mishaly D, Kenet G, Paret G. Dehydration as a rare cause of pulmonary artery thrombosis in a 2-week-old term neonate. J Pediatr Intensive Care. 2018 Jun. 7(2):102-5. [QxMD MEDLINE Link]. [Full Text].
Nuss R, Hays T, Chudgar U, Manco-Johnson M. Antiphospholipid antibodies and coagulation regulatory protein abnormalities in children with pulmonary emboli. J Pediatr Hematol Oncol. 1997 May-Jun. 19(3):202-7. [QxMD MEDLINE Link].
Lawton A, Machta J, Semple T, Gupta A. Pulmonary manifestations of systemic vasculitis in childhood. Breathe (Sheff). 2020 Dec. 16(4):200211. [QxMD MEDLINE Link]. [Full Text].
Agha BS, Sturm JJ, Simon HK, Hirsh DA. Pulmonary embolism in the pediatric emergency department. Pediatrics. 2013 Oct. 132(4):663-7. [QxMD MEDLINE Link].
Bernstein D, Coupey S, Schonberg SK. Pulmonary embolism in adolescents. Am J Dis Child. 1986 Jul. 140(7):667-71. [QxMD MEDLINE Link].
Kotsakis A, Cook D, Griffith L, et al. Clinically important venous thromboembolism in pediatric critical care: a Canadian survey. J Crit Care. 2005 Dec. 20(4):373-80. [QxMD MEDLINE Link].
Van Ommen CH, Peters M. Acute pulmonary embolism in childhood. Thromb Res. 2006. 118(1):13-25. [QxMD MEDLINE Link].
Babyn PS, Gahunia HK, Massicotte P. Pulmonary thromboembolism in children. Pediatr Radiol. 2005 Mar. 35(3):258-74. [QxMD MEDLINE Link].
Baird JS, Killinger JS, Kalkbrenner KJ, Bye MR, Schleien CL. Massive pulmonary embolism in children. J Pediatr. 2010 Jan. 156(1):148-51. [QxMD MEDLINE Link].
Evans DA, Wilmott RW. Pulmonary embolism in children. Pediatr Clin North Am. 1994 Jun. 41(3):569-84. [QxMD MEDLINE Link].
Rajpurkar M, Warrier I, Chitlur M, et al. Pulmonary embolism-experience at a single children's hospital. Thromb Res. 2007. 119(6):699-703. [QxMD MEDLINE Link].
Lee EY, Tse SK, Zurakowski D, et al. Children suspected of having pulmonary embolism: multidetector CT pulmonary angiography--thromboembolic risk factors and implications for appropriate use. Radiology. 2012 Jan. 262(1):242-51. [QxMD MEDLINE Link].
Lee EY, Zurakowski D, Boiselle PM. Pulmonary embolism in pediatric patients survey of CT pulmonary angiography practices and policies. Acad Radiol. 2010 Dec. 17(12):1543-9. [QxMD MEDLINE Link].
The PIOPED Investigators. Value of the ventilation/perfusion scan in acute pulmonary embolism. Results of the prospective investigation of pulmonary embolism diagnosis (PIOPED). JAMA. 1990 May 23-30. 263(20):2753-9. [QxMD MEDLINE Link].
Revel MP, Sanchez O, Couchon S, et al. Diagnostic accuracy of magnetic resonance imaging for an acute pulmonary embolism: results of the 'IRM-EP' study. J Thromb Haemost. 2012 May. 10(5):743-50. [QxMD MEDLINE Link].
Goldenberg NA, Durham JD, Knapp-Clevenger R, Manco-Johnson MJ. A thrombolytic regimen for high-risk deep venous thrombosis may substantially reduce the risk of postthrombotic syndrome in children. Blood. 2007 Jul 1. 110(1):45-53. [QxMD MEDLINE Link].
Akam-Venkata J, Forbes TJ, Schreiber T, et al. Catheter-directed therapy for acute pulmonary embolism in children. Cardiol Young. 2019 Mar. 29(3):263-9. [QxMD MEDLINE Link].
Badheka A, Bangalore Prakash P, Allareddy V. Successful use of extracorporeal membrane oxygenation in a child with obstructive shock due to massive bilateral pulmonary embolism. Perfusion. 2018 May. 33(4):323-5. [QxMD MEDLINE Link].
Jain S, Bakshi N, Krishnamurti L. Acute chest syndrome in children with sickle cell disease. Pediatr Allergy Immunol Pulmonol. 2017 Dec 1. 30(4):191-201. [QxMD MEDLINE Link]. [Full Text].
Raffini L, Cahill AM, Hellinger J, Manno C. A prospective observational study of IVC filters in pediatric patients. Pediatr Blood Cancer. 2008 Oct. 51(4):517-20. [QxMD MEDLINE Link].
Faustino EV, Patel S, Thiagarajan RR, Cook DJ, Northrup V, Randolph AG. Survey of pharmacologic thromboprophylaxis in critically ill children. Crit Care Med. 2011 Jul. 39(7):1773-8. [QxMD MEDLINE Link]. [Full Text].
[Guideline] Ortel TL, Neumann I, Ageno W, et al. American Society of Hematology 2020 guidelines for management of venous thromboembolism: treatment of deep vein thrombosis and pulmonary embolism. Blood Adv. 2020 Oct 13. 4(19):4693-738. [QxMD MEDLINE Link]. [Full Text].
Hull RD, Raskob GE, Brant RF, et al. Low-molecular-weight heparin vs heparin in the treatment of patients with pulmonary embolism. American-Canadian Thrombosis Study Group. Arch Intern Med. 2000 Jan 24. 160(2):229-36. [QxMD MEDLINE Link].
Baird JS, Greene A, Schleien CL. Massive pulmonary embolus without hypoxemia. Pediatr Crit Care Med. 2005 Sep. 6(5):602-3. [QxMD MEDLINE Link].
Brandao LR, Labarque V, Diab Y, Williams S, Manson DE. Pulmonary embolism in children. Semin Thromb Hemost. 2011 Oct. 37(7):772-85. [QxMD MEDLINE Link].
Kossel H, Bartsch H, Philippi W, et al. Pulmonary embolism and myocardial hypoxia during extracorporeal membrane oxygenation. J Pediatr Surg. 1999 Mar. 34(3):485-7. [QxMD MEDLINE Link].
Macartney CA, Chan AK. Thrombosis in children. Semin Thromb Hemost. 2011 Oct. 37(7):763-1. [QxMD MEDLINE Link].
McCrory MC, Brady KM, Takemoto C, Tobias JD, Easley RB. Thrombotic disease in critically ill children. Pediatr Crit Care Med. 2011 Jan. 12(1):80-9. [QxMD MEDLINE Link].
Monagle P, Chan A, DeVeber G, Massicotte MP. Andrew's Pediatric Thromboembolism and Stroke. 3rd ed. Ontario, Canada: BC Decker; 2006.
Sandoval JA, Sheehan MP, Stonerock CE, Shafique S, Rescorla FJ, Dalsing MC. Incidence, risk factors, and treatment patterns for deep venous thrombosis in hospitalized children: an increasing population at risk. J Vasc Surg. 2008 Apr. 47(4):837-43. [QxMD MEDLINE Link]. [Full Text].
Tay ET, Stone MB, Tsung JW. Emergency ultrasound diagnosis of deep venous thrombosis in the pediatric emergency department: a case series. Pediatr Emerg Care. 2012 Jan. 28(1):90-5. [QxMD MEDLINE Link].
Truitt AK, Sorrells DL, Halvorson E, et al. Pulmonary embolism: which pediatric trauma patients are at risk?. J Pediatr Surg. 2005 Jan. 40(1):124-7. [QxMD MEDLINE Link].
[Guideline] Gali N, Humbert M, Vachiery JL, et al, for the ESC Scientific Document Group . 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016 Jan 1. 37(1):67-119. [QxMD MEDLINE Link]. [Full Text].
Lilje C, Chauhan A, Turner JP, et al. Pediatric pulmonary embolism: diagnostic and management challenges. World J Pediatr Congenit Heart Surg. 2018 Jan. 9(1):110-3. [QxMD MEDLINE Link]. [Full Text].
Agha BS, Sturm JJ, Simon HK, Hirsh DA. Pulmonary embolism in the pediatric emergency department. Pediatrics. 2013 Oct. 132(4):663-7. [QxMD MEDLINE Link].

Media Gallery

of 0

Author

Lennox H Huang, MD, FAAP Chief Medical Officer, The Hospital for Sick Children; Associate Professor of Pediatrics, University of Toronto Faculty of Medicine; Associate Clinical Professor of Pediatrics, McMaster University School of Medicine, Canada

Lennox H Huang, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Physician Leadership, Canadian Medical Association, Ontario Medical Association, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Barry J Evans, MD Assistant Professor of Pediatrics, Temple University Medical School; Director of Pediatric Critical Care and Pulmonology, Associate Chair for Pediatric Education, Temple University Children's Medical Center

Barry J Evans, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Chief Editor

Kenan Haver, MD Associate Professor of Pediatrics, Harvard Medical School; Director of Asthma Program, Director of Flexible Bronchoscopy Program, Director of Pulmonary Division Asthma Program, Co-Director of Primary Ciliary Dyskinesia, Co-Leader of Empyema Standardized Clinical Assessment and Management Plans (SCAMP), Boston Children’s Hospital

Kenan Haver, MD is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society

Disclosure: Author for: UptoDate.

Additional Contributors

G Patricia Cantwell, MD, FCCM Professor of Clinical Pediatrics, Chief, Division of Pediatric Critical Care Medicine, University of Miami Leonard M Miller School of Medicine/ Holtz Children's Hospital, Jackson Memorial Medical Center; Medical Director, Palliative Care Team, Holtz Children's Hospital; Medical Manager, FEMA, South Florida Urban Search and Rescue, Task Force 2

G Patricia Cantwell, MD, FCCM is a member of the following medical societies: American Academy of Hospice and Palliative Medicine, American Academy of Pediatrics, American Heart Association, American Trauma Society, National Association of EMS Physicians, Society of Critical Care Medicine, Wilderness Medical Society

Disclosure: Nothing to disclose.