Clin Hypertens.  2022;28(1):10. 10.1186/s40885-021-00191-1.

Pulmonary arterial hypertension due to antiphospholipid syndrome initially mimicking chronic thromboembolic pulmonary hypertension

Affiliations
  • 1Department of Internal Medicine, Gachon University Gil Medical Center, Incheon, Republic of Korea
  • 2Department of Pediatrics, Division of Pediatric Cardiology, Gachon University Gil Medical Center, Incheon, Republic of Korea
  • 3Gachon Cardiovascular Research Institute, Gachon University, Incheon, Republic of Korea
  • 4Division of Rheumatology, Department of Internal Medicine, Gachon University Gil Medical Center, Incheon, Republic of Korea
  • 5Department of Cardiovascular Medicine, Gachon University Gil Medical Center, Incheon, Republic of Korea

Abstract

Pulmonary arterial hypertension (PAH) is the second most common lung complication in antiphospholipid syndrome (APS) patients. However, the concurrent development of APS-related nonthrombotic PAH is rarely reported. Lack of awareness for group 1 PAH in APS patient may contribute to underdiagnosis of this condition. Herein, we reviewed the case of a 56-year-old female who was diagnosed with PAH related to APS that mimicked chronic thromboembolic pulmonary hypertension (CTEPH). It is crucial to be aware of the possibility of a group 1 PAH diagnosis, even though patients have already been diagnosed with CTEPH. Furthermore, a multidisciplinary approach and serial follow-up right heart catheterization with echocardiography are important to make a timely diagnosis and provide optimal treatment for APS-related PAH in patients with CTEPH-like clinical features.

Keyword

Pulmonary arterial hypertension; Antiphospholipid syndrome; Thromboembolism; Endothelin receptor antagonist
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