title: Effects of treatment with a combination of cardiac rehabilitation and bosentan in patients with pulmonary Langerhans cell histiocytosis associated with pulmonary hypertension.
abstract: Pulmonary hypertension (PH), which is classified as group 5 in the clinical classification of PH, is sometimes a complication of Pulmonary langerhans cell histiocytosis (PLCH), and is associated with high mortality. A 36-year-old woman had suffered from severe dyspnea 9 years previously and was diagnosed with PLCH and was on a waiting list for a lung transplant. Right heart failure had been observed and the mean pulmonary artery pressure was over 40 mmHg. The patient was diagnosed as PLCH with PH. After combined treatment with exercise rehabilitation and bosentan for 6 months, the cardiothoracic ratio, brain natriuretic peptide, and bodyweight were significantly decreased (cardiothoracic ratio from 43 to 38%, brain natriuretic peptide from 284 to10 pg/ml and bodyweight from 63 to 58 kg). Six-minute walk test also improved from 214 to 275 meters and the SF36 score for screening of depressive and anxiety disorders was improved. This is the report demonstrating the efficacy and safety of cardiac rehabilitation in combination with bosentan in a single patient with PLCH associated with PH.
authors.affiliation: Department of Cardiology, Fukuoka University School of Medicine, Fukuoka, Japan zfukusuke@minf.med.fukuoka-u.ac.jp.
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Fukuda
authors.affiliation: Department of Cardiology, Fukuoka University School of Medicine, Fukuoka, Japan.
authors.forename: Shin-ichiro
authors.initials: S
authors.lastname: Miura
authors.affiliation: Department of Cardiology, Fukuoka University School of Medicine, Fukuoka, Japan.
authors.forename: Kanta
authors.initials: K
authors.lastname: Fujimi
authors.affiliation: Department of Cardiology, Fukuoka University School of Medicine, Fukuoka, Japan.
authors.forename: Masaya
authors.initials: M
authors.lastname: Yano
authors.affiliation: Department of Cardiology, Fukuoka University School of Medicine, Fukuoka, Japan.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Nishikawa
authors.affiliation: Department of General Thoracic, Breast and Pediatric Surgery, Fukuoka University School of Medicine, Fukuoka, Japan.
authors.forename: Jun
authors.initials: J
authors.lastname: Yanagisawa
authors.affiliation: Department of General Thoracic, Breast and Pediatric Surgery, Fukuoka University School of Medicine, Fukuoka, Japan.
authors.forename: Masafumi
authors.initials: M
authors.lastname: Hiratsuka
authors.affiliation: Department of General Thoracic, Breast and Pediatric Surgery, Fukuoka University School of Medicine, Fukuoka, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Shiraishi
authors.affiliation: Department of General Thoracic, Breast and Pediatric Surgery, Fukuoka University School of Medicine, Fukuoka, Japan.
authors.forename: Akinori
authors.initials: A
authors.lastname: Iwasaki
authors.affiliation: Department of Cardiology, Fukuoka University School of Medicine, Fukuoka, Japan.
authors.forename: Keijiro
authors.initials: K
authors.lastname: Saku
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use*; D005081:Exercise Therapy; D017079:Exercise Tolerance / Q000187:drug effects; D005260:Female; D006333:Heart Failure / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000534:rehabilitation*; D006439:Hemodynamics / Q000187:drug effects; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D049268:Positron-Emission Tomography; D020127:Recovery of Function; D013449:Sulfonamides / Q000627:therapeutic use*; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D013449:Sulfonamides; D000077300:Bosentan
keywords: Bosentan; cardiac rehabilitation; pulmonary Langerhans cell histiocytosis; pulmonary hypertension