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

• Yeo J
• Shin N
• Ahn KJ
• Seo M
• Jang AY
• Kim M
• Chung WJ

• KMID: 2527998
• DOI: http://doi.org/10.1186/s40885-021-00191-1

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