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Rescue balloon pulmonary angioplasty for worsening chronic thromboembolic pulmonary hypertension with essential thrombocythaemia and the JAK2-V617F mutation: a case report

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare thromboembolic disease, with occasional critical consequences. Essential thrombocythaemia (ET) is associated with an increased incidence of venous and arterial thrombotic events. In addition, the JAK2-V617F mutation increase...

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Autores principales: Tamura, Hiroto, Hosokawa, Shinobu, Yuba, Kenichiro, Kishi, Koichi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10026540/
https://www.ncbi.nlm.nih.gov/pubmed/36949868
http://dx.doi.org/10.1093/ehjcr/ytad106
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author Tamura, Hiroto
Hosokawa, Shinobu
Yuba, Kenichiro
Kishi, Koichi
author_facet Tamura, Hiroto
Hosokawa, Shinobu
Yuba, Kenichiro
Kishi, Koichi
author_sort Tamura, Hiroto
collection PubMed
description BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare thromboembolic disease, with occasional critical consequences. Essential thrombocythaemia (ET) is associated with an increased incidence of venous and arterial thrombotic events. In addition, the JAK2-V617F mutation increases the risk of thrombosis. Few reports have evaluated the utility of balloon pulmonary angioplasty (BPA) for worsening CTEPH with ET and the JAK2-V617F mutation. CASE SUMMARY: A 76-year-old woman, diagnosed with ET and the JAK2-V617F mutation, presented with dyspnoea. Echocardiography showed severe tricuspid regurgitation with a flattened interventricular septum. Contrast-enhanced computed tomography showed an eccentric thrombus in the right main pulmonary artery (PA) and thrombi in bilateral peripheral PAs. Acute pulmonary embolism (PE) was initially diagnosed, and heparinization was initiated; however, her oxygen saturation gradually worsened despite continued anticoagulation therapy. Her oxygen saturation level decreased to 90% (under a reservoir mask of 10 L). Her haemodynamics suggested CTEPH comorbidity. We decided to perform emergency right heart catheterization (RHC) and pulmonary angiography (PAG). RHC showed severe pulmonary hypertension. PAG showed fresh and organized thrombi and web regions in several segmental PAs. These findings indicated a combination of acute PE and CTEPH. Rescue BPA was performed on the right A1, A3, A8, and A9 segments. After BPA, the patient’s oxygen saturation showed marked improvement. The patient was discharged 18 days after hospitalization without complications. DISCUSSION: Rescue BPA could be an effective treatment for worsening CTEPH in severely impaired conditions, even with ET and the JAK2-V617F mutation.
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spelling pubmed-100265402023-03-21 Rescue balloon pulmonary angioplasty for worsening chronic thromboembolic pulmonary hypertension with essential thrombocythaemia and the JAK2-V617F mutation: a case report Tamura, Hiroto Hosokawa, Shinobu Yuba, Kenichiro Kishi, Koichi Eur Heart J Case Rep Case Report BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare thromboembolic disease, with occasional critical consequences. Essential thrombocythaemia (ET) is associated with an increased incidence of venous and arterial thrombotic events. In addition, the JAK2-V617F mutation increases the risk of thrombosis. Few reports have evaluated the utility of balloon pulmonary angioplasty (BPA) for worsening CTEPH with ET and the JAK2-V617F mutation. CASE SUMMARY: A 76-year-old woman, diagnosed with ET and the JAK2-V617F mutation, presented with dyspnoea. Echocardiography showed severe tricuspid regurgitation with a flattened interventricular septum. Contrast-enhanced computed tomography showed an eccentric thrombus in the right main pulmonary artery (PA) and thrombi in bilateral peripheral PAs. Acute pulmonary embolism (PE) was initially diagnosed, and heparinization was initiated; however, her oxygen saturation gradually worsened despite continued anticoagulation therapy. Her oxygen saturation level decreased to 90% (under a reservoir mask of 10 L). Her haemodynamics suggested CTEPH comorbidity. We decided to perform emergency right heart catheterization (RHC) and pulmonary angiography (PAG). RHC showed severe pulmonary hypertension. PAG showed fresh and organized thrombi and web regions in several segmental PAs. These findings indicated a combination of acute PE and CTEPH. Rescue BPA was performed on the right A1, A3, A8, and A9 segments. After BPA, the patient’s oxygen saturation showed marked improvement. The patient was discharged 18 days after hospitalization without complications. DISCUSSION: Rescue BPA could be an effective treatment for worsening CTEPH in severely impaired conditions, even with ET and the JAK2-V617F mutation. Oxford University Press 2023-02-27 /pmc/articles/PMC10026540/ /pubmed/36949868 http://dx.doi.org/10.1093/ehjcr/ytad106 Text en © The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology. https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Case Report
Tamura, Hiroto
Hosokawa, Shinobu
Yuba, Kenichiro
Kishi, Koichi
Rescue balloon pulmonary angioplasty for worsening chronic thromboembolic pulmonary hypertension with essential thrombocythaemia and the JAK2-V617F mutation: a case report
title Rescue balloon pulmonary angioplasty for worsening chronic thromboembolic pulmonary hypertension with essential thrombocythaemia and the JAK2-V617F mutation: a case report
title_full Rescue balloon pulmonary angioplasty for worsening chronic thromboembolic pulmonary hypertension with essential thrombocythaemia and the JAK2-V617F mutation: a case report
title_fullStr Rescue balloon pulmonary angioplasty for worsening chronic thromboembolic pulmonary hypertension with essential thrombocythaemia and the JAK2-V617F mutation: a case report
title_full_unstemmed Rescue balloon pulmonary angioplasty for worsening chronic thromboembolic pulmonary hypertension with essential thrombocythaemia and the JAK2-V617F mutation: a case report
title_short Rescue balloon pulmonary angioplasty for worsening chronic thromboembolic pulmonary hypertension with essential thrombocythaemia and the JAK2-V617F mutation: a case report
title_sort rescue balloon pulmonary angioplasty for worsening chronic thromboembolic pulmonary hypertension with essential thrombocythaemia and the jak2-v617f mutation: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10026540/
https://www.ncbi.nlm.nih.gov/pubmed/36949868
http://dx.doi.org/10.1093/ehjcr/ytad106
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