Management of Rare Disorders Causing Pulmonary Embolism

Diagnostic Approach and Management

• The European Society of Cardiology recommends stratifying patients with suspected pulmonary embolism (PE) based on hemodynamic stability to identify those at high risk of early mortality 4, 5
• For hemodynamically unstable patients, the European Society of Cardiology suggests administering systemic thrombolytic therapy 4, 5
• The European Respiratory Society recommends considering extracorporeal membrane oxygenation (ECMO) in combination with surgical embolectomy or catheter-directed treatment in refractory circulatory collapse or cardiac arrest 3
• For stable patients, the European Society of Cardiology prefers novel oral anticoagulants (NOACs) such as apixaban, dabigatran, edoxaban, or rivaroxaban when eligible 4, 5
• The European Society of Cardiology recommends continuing oral anticoagulant treatment with a vitamin K antagonist (VKA) indefinitely for patients with antiphospholipid syndrome (APS) 4, 5
• Pulmonary endarterectomy provides excellent results and should be considered as first-line treatment for chronic thromboembolic pulmonary hypertension (CTEPH) when feasible 6
• The European Society of Cardiology suggests referring symptomatic patients with mismatched perfusion defects on V/Q scan >3 months after acute PE to a pulmonary hypertension/CTEPH expert center 4, 5
• The European Respiratory Society recommends routinely re-evaluating patients 3-6 months after acute PE 3, 5
• The European Society of Cardiology advises against using NOACs during pregnancy or lactation 3, 4, 5
• The European Society of Cardiology recommends against routinely using inferior vena cava filters 4, 5
• The European Heart Society notes that non-thrombotic PE does not represent a distinct clinical syndrome and may present with a wide spectrum of clinical manifestations 1
• The European Heart Society states that diagnosis of non-thrombotic PE is often challenging due to variable presentations 1
• The European Heart Society reports that with the exception of severe air and fat embolism, hemodynamic consequences of non-thrombotic emboli are usually mild 1