Open AccessBalloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension concomitant with Klippel–Trenaunay–Weber syndrome
Author(s) -
Suetomi Takeshi,
Shimokawahara Hiroto,
Sugiyama Yoichi,
Miyagi Ayane,
Ogawa Aiko,
Nishizaki Mari,
Matsubara Hiromi
Publication year - 2022
Publication title -
pulmonary circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.791
H-Index - 40
ISSN - 2045-8940
DOI - 10.1002/pul2.12155
Subject(s) - medicine , chronic thromboembolic pulmonary hypertension , angioplasty , klippel trenaunay syndrome , concomitant , klippel trenaunay weber syndrome , balloon , pulmonary hypertension , cardiology , radiology , muscle hypertrophy , soft tissue
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.