دورية أكاديمية
Pulmonary hypertension associated with veno-occlusive disease in systemic sclerosis: Insight into the mechanism of resistance to vasodilator
العنوان: | Pulmonary hypertension associated with veno-occlusive disease in systemic sclerosis: Insight into the mechanism of resistance to vasodilator |
---|---|
المؤلفون: | Tada Hayato, Konno Tetsuo, Aizu Motohiko, Yokawa Junichiro, Tsubokawa Toshinari, Fujii Hiroshi, Hayashi Kenshi, Uchiyama Katsuharu, Matsumura Masami, Kawano Mitsuhiro, Kawashiri Masa-aki, Yamagishi Masakazu |
بيانات النشر: | Elsevier / Japanese College of Cardiology |
سنة النشر: | 2012 |
المجموعة: | Kanazawa University Repository for Academic Resources (KURA) / 金沢大学学術情報リポジトリ |
مصطلحات موضوعية: | Epoprostenol, Pulmonary arterial hypertension, Pulmonary hypertension, Pulmonary veno-occlusive disease |
الوصف: | We report a case with pulmonary veno-occlusive disease (PVOD) associated with systemic sclerosis which exhibits strong resistance to pulmonary vasodilator.A 55-year-old female with severe pulmonary hypertension was admitted to our hospital to be introduced to epoprostenol infusion therapy. She was diagnosed as having pulmonary arterial hypertension (PAH) associated with systemic sclerosis at the age of 51. Several aggressive treatments with pulmonary vasodilators, including oral prostaglandin, endothelin receptor antagonists, and phosphodiesterase 5 inhibitors, failed to improve her symptoms. We introduced continuous intravenous epoprostenol therapy from 2. μg/kg/min for her. However, pulmonary edema appeared and worsened in a dose-dependent manner. We made a diagnosis of PVOD clinically at that time. Thereafter, pulmonary edema gradually disappeared consistent with the reduction of the dose of epoprostenol infusion. She died of renal failure and infection 4. months after the introduction of epoprostenol infusion therapy. A histological examination revealed severe stenosis and occlusions of pulmonary veins as well as pulmonary arteries over a wide area. We suggest that prevalence of veno-occlusive type of disease could be one of the major mechanisms of less responsive or even refractory to pulmonary vasodilator therapies in patients with PAH associated with connective tissue disease. © 2011 Japanese College of Cardiology. |
نوع الوثيقة: | article in journal/newspaper |
اللغة: | English |
تدمد: | 1878-5409 |
العلاقة: | http://www.elsevier.com/locate/issn/18785409Test; https://kanazawa-u.repo.nii.ac.jp/?action=repository_uri&item_id=26790Test; http://hdl.handle.net/2297/30319Test; Journal of Cardiology Cases, 5(1), e44-e47(2012-02-01); https://kanazawa-u.repo.nii.ac.jp/?action=repository_action_common_download&item_id=26790&item_no=1&attribute_id=26&file_no=1Test |
الإتاحة: | http://hdl.handle.net/2297/30319Test https://kanazawa-u.repo.nii.ac.jp/?action=repository_uri&item_id=26790Test https://kanazawa-u.repo.nii.ac.jp/?action=repository_action_common_download&item_id=26790&item_no=1&attribute_id=26&file_no=1Test |
رقم الانضمام: | edsbas.C12D6C49 |
قاعدة البيانات: | BASE |
تدمد: | 18785409 |
---|