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Key Features

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  • Acute onset dyspnea and tachypnea

  • Evidence of embolism on chest imaging

  • Rare in children

  • Etiologies include

    • Sickle cell anemia (as part of the acute chest syndrome)

    • Malignancy

    • Rheumatic fever

    • Infective endocarditis

    • Bone fracture

    • Dehydration

    • Polycythemia

    • Nephrotic syndrome

    • Atrial fibrillation

  • A majority of children with pulmonary emboli referred for hematology evaluation have coagulation regulatory protein abnormalities and antiphospholipid antibodies

  • In children, tumor emboli are a more common cause of massive pulmonary embolism than embolization from a lower extremity deep venous thrombosis

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Clinical Findings

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  • Acute onset of dyspnea and tachypnea

  • Heart palpitations, pleuritic chest pain, and a sense of impending doom may be reported

  • Hemoptysis is rare but may occur along with splinting, cyanosis, and tachycardia

  • Massive emboli may be present with syncope and cardiac arrhythmias

  • Mild hypoxemia, rales, focal wheezing, or a pleural friction rub may be found

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Diagnosis

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  • Radiography

    • May be normal

    • However, a peripheral infiltrate, small pleural effusion, or elevated hemidiaphragm can be present

    • If the emboli are massive, differential blood flow and pulmonary artery enlargement may be appreciated

  • Electrocardiogram is usually normal unless the pulmonary embolus is massive

  • Echocardiography is useful in detecting the presence of a large embolus in the great vessels

  • A negative D-dimer has a more than 95% negative predictive value for an embolus, but has poor specificity

  • Ventilation-perfusion scans show localized areas of ventilation without perfusion

  • Spiral CT with contrast may be helpful

  • Pulmonary angiography is the gold standard

  • Coagulation studies are abnormal in up to 70% of pediatric patients

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Treatment

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  • Supplemental oxygen

  • Anticoagulation: heparin therapy to maintain an activated partial thromboplastin time of > 1.5 times the control value for the first 24 hours

  • Urokinase or tissue plasminogen activator

    • Can be used to help dissolve the embolus

    • Should be followed by warfarin therapy for at least 6 weeks with an international normalized ratio (INR) > 2

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