Dyspnea in antiphospholipid syndrome: Beyond pulmonary embolism

Rev Port Cardiol (Engl Ed). 2020 Jan;39(1):47.e1-47.e5. doi: 10.1016/j.repc.2018.01.015. Epub 2020 Mar 12.
[Article in English, Portuguese]

Abstract

Pulmonary embolism due to primary antiphospholipid syndrome is rarely associated with chronic thromboembolic pulmonary hypertension, and therefore according to the latest guidelines on pulmonary hypertension, routine screening is not recommended. We describe a young patient with a late diagnosis of chronic thromboembolic pulmonary hypertension in the context of pulmonary embolism, primary antiphospholipid syndrome and suboptimal anticoagulation. Of note, mild cardiopulmonary symptoms were consistently misattributed to a depressive disorder because physical examination was normal, serial Doppler echocardiography failed to show pulmonary hypertension, and all other diagnostic tests were normal. Once symptoms became severe, positive screening tests led to the correct diagnosis and surgical referral, and bilateral pulmonary endarterectomy was successfully performed. This case demonstrates the need for extra awareness in patients with antiphospholipid syndrome and pulmonary embolism.

Keywords: Antiphospholipid syndrome; Chronic thromboembolic pulmonary hypertension; Embolia pulmonar; Hipertensão pulmonar; Hipertensão pulmonar tromboembólica crónica; Pulmonary embolism; Pulmonary endarterectomy; Síndrome antifosfolípido.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antiphospholipid Syndrome / complications*
  • Awareness
  • Chronic Disease
  • Depressive Disorder / diagnosis
  • Dyspnea / etiology*
  • Echocardiography, Doppler / adverse effects
  • Echocardiography, Doppler / standards
  • Endarterectomy / methods
  • Female
  • Humans
  • Hypertension, Pulmonary / diagnosis*
  • Pulmonary Embolism / complications*
  • Pulmonary Embolism / surgery
  • Treatment Outcome