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Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension concomitant with Klippel–Trenaunay–Weber syndrome

Klippel–Trenaunay–Weber syndrome (KTWS) is a rare congenital disorder characterized by cutaneous capillary malformations, bone hypertrophy, and multiple venous or lymphatic malformations. KTWS is associated with chronic thromboembolic pulmonary hypertension (CTEPH), presumably due to thromboembolism...

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Autores principales: Suetomi, Takeshi, Shimokawahara, Hiroto, Sugiyama, Yoichi, Miyagi, Ayane, Ogawa, Aiko, Nishizaki, Mari, Matsubara, Hiromi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9684684/
https://www.ncbi.nlm.nih.gov/pubmed/36438451
http://dx.doi.org/10.1002/pul2.12155
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author Suetomi, Takeshi
Shimokawahara, Hiroto
Sugiyama, Yoichi
Miyagi, Ayane
Ogawa, Aiko
Nishizaki, Mari
Matsubara, Hiromi
author_facet Suetomi, Takeshi
Shimokawahara, Hiroto
Sugiyama, Yoichi
Miyagi, Ayane
Ogawa, Aiko
Nishizaki, Mari
Matsubara, Hiromi
author_sort Suetomi, Takeshi
collection PubMed
description Klippel–Trenaunay–Weber syndrome (KTWS) is a rare congenital disorder characterized by cutaneous capillary malformations, bone hypertrophy, and multiple venous or lymphatic malformations. KTWS is associated with chronic thromboembolic pulmonary hypertension (CTEPH), presumably due to thromboembolism from multiple vascular malformations. Here, we report the first case series of patients with KTWS‐CTEPH who underwent balloon pulmonary angioplasty (BPA). Both patients are alive 20 years and 1 year after the initial diagnosis of CTEPH, respectively, and are stable with improved hemodynamics. BPA may be an effective treatment option for patients with KTWS‐CTEPH.
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spelling pubmed-96846842022-11-25 Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension concomitant with Klippel–Trenaunay–Weber syndrome Suetomi, Takeshi Shimokawahara, Hiroto Sugiyama, Yoichi Miyagi, Ayane Ogawa, Aiko Nishizaki, Mari Matsubara, Hiromi Pulm Circ Case Reports Klippel–Trenaunay–Weber syndrome (KTWS) is a rare congenital disorder characterized by cutaneous capillary malformations, bone hypertrophy, and multiple venous or lymphatic malformations. KTWS is associated with chronic thromboembolic pulmonary hypertension (CTEPH), presumably due to thromboembolism from multiple vascular malformations. Here, we report the first case series of patients with KTWS‐CTEPH who underwent balloon pulmonary angioplasty (BPA). Both patients are alive 20 years and 1 year after the initial diagnosis of CTEPH, respectively, and are stable with improved hemodynamics. BPA may be an effective treatment option for patients with KTWS‐CTEPH. John Wiley and Sons Inc. 2022-10-01 /pmc/articles/PMC9684684/ /pubmed/36438451 http://dx.doi.org/10.1002/pul2.12155 Text en © 2022 The Authors. Pulmonary Circulation published by Wiley Periodicals LLC on behalf of the Pulmonary Vascular Research Institute. https://creativecommons.org/licenses/by-nc/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.
spellingShingle Case Reports
Suetomi, Takeshi
Shimokawahara, Hiroto
Sugiyama, Yoichi
Miyagi, Ayane
Ogawa, Aiko
Nishizaki, Mari
Matsubara, Hiromi
Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension concomitant with Klippel–Trenaunay–Weber syndrome
title Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension concomitant with Klippel–Trenaunay–Weber syndrome
title_full Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension concomitant with Klippel–Trenaunay–Weber syndrome
title_fullStr Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension concomitant with Klippel–Trenaunay–Weber syndrome
title_full_unstemmed Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension concomitant with Klippel–Trenaunay–Weber syndrome
title_short Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension concomitant with Klippel–Trenaunay–Weber syndrome
title_sort balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension concomitant with klippel–trenaunay–weber syndrome
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9684684/
https://www.ncbi.nlm.nih.gov/pubmed/36438451
http://dx.doi.org/10.1002/pul2.12155
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