<abstract xmlns="http://www.w3.org/1999/xhtml"><p> Background: Prothrombotic state and impaired clot dissolution can contribute to the occurrence of chronic thromboembolic pulmonary hypertension in primary antiphospholipid syndrome. Pulmonary endarterectomy - the surgical removal of the organized thromboembolic material from the proximal pulmonary arteries - is the procedure of choice and potentially a curative option for patients with chronic thromboembolic pulmonary hypertension, including patients with antiphospholipid syndrome. We report an exceptionally severe and complicated case with favourable outcome. Case presentation: We present a case of a successful high risk pulmonary endarterectomy in a 29-year-old female with primary antiphospholipid syndrome and end-stage chronic thromboembolic pulmonary hypertension. Despite highly complicated perioperative course an impressive improvement in symptoms and functional status was achieved. Conclusion: We hope that this complicated but successfully managed case of a combination of two rare diseases will arouse earlier suspicion and timely diagnosis for such patients and will encourage physicians to promptly refer the suitable patients to a pulmonary endarterectomy team. It is important to remember that severe thrombocytopenia may occur in patients with antiphospholipid syndrome</p></abstract>

Background: Prothrombotic state and impaired clot dissolution can contribute to the occurrence of chronic thromboembolic pulmonary hypertension in primary antiphospholipid syndrome. Pulmonary endarterectomy - the surgical removal of the organized thromboembolic material from the proximal pulmonary arteries - is the procedure of choice and potentially a curative option for patients with chronic thromboembolic pulmonary hypertension, including patients with antiphospholipid syndrome. We report an exceptionally severe and complicated case with favourable outcome. Case presentation: We present a case of a successful high risk pulmonary endarterectomy in a 29-year-old female with primary antiphospholipid syndrome and end-stage chronic thromboembolic pulmonary hypertension. Despite highly complicated perioperative course an impressive improvement in symptoms and functional status was achieved. Conclusion: We hope that this complicated but successfully managed case of a combination of two rare diseases will arouse earlier suspicion and timely diagnosis for such patients and will encourage physicians to promptly refer the suitable patients to a pulmonary endarterectomy team. It is important to remember that severe thrombocytopenia may occur in patients with antiphospholipid syndrome

Case report: Life-saving pulmonary endarterectomy for a young female with primary antiphospholipid syndrome complicated by severe chronic thromboembolic pulmonary hypertension

Seminars in Cardiovascular Medicine

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Background: Prothrombotic state and impaired clot dissolution can contribute to the occurrence of chronic thromboembolic pulmonary hypertension in primary...

Case report: Life-saving pulmonary endarterectomy for a young female with primary antiphospholipid syndrome complicated by severe chronic thromboembolic pulmonary hypertension

Published Online: Sep 01, 2018

Page range: 1 - 8

Received: Aug 10, 2017

Accepted: Jan 22, 2018

DOI: https://doi.org/10.2478/semcard-2018-0001

Keywords
chronic thromboembolic pulmonary hypertension, pulmonary endarterectomy, antiphospholipid syndrome

© 2018 Eglė Palevičiūtė et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Case report: Life-saving pulmonary endarterectomy for a young female with primary antiphospholipid syndrome complicated by severe chronic thromboembolic pulmonary hypertension

Published Online: Sep 01, 2018

Page range: 1 - 8

Received: Aug 10, 2017

Accepted: Jan 22, 2018

DOI: https://doi.org/10.2478/semcard-2018-0001

Keywordschronic thromboembolic pulmonary hypertension, pulmonary endarterectomy, antiphospholipid syndrome

© 2018 Eglė Palevičiūtė et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Keywords
chronic thromboembolic pulmonary hypertension, pulmonary endarterectomy, antiphospholipid syndrome