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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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Detalles Bibliográficos
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
Descripción
Sumario: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.