التفاصيل البيبلوغرافية
| العنوان: |
Phenotype and Outcomes of Pulmonary Hypertension Associated with Neurofibromatosis Type 1. |
| المؤلفون: |
Jutant, Etienne-Marie, Jaïs, Xavier, Girerd, Barbara, Savale, Laurent, Ghigna, Maria-Rosa, Perros, Frédéric, Mignard, Xavier, Jevnikar, Mitja, Bourlier, Delphine, Prevot, Grégoire, Tromeur, Cécile, Bauer, Fabrice, Bergot, Emmanuel, Dauphin, Claire, Favrolt, Nicolas, Traclet, Julie, Soumagne, Thibaud, De Groote, Pascal, Chabanne, Céline, Magro, Pascal |
| المصدر: |
American Journal of Respiratory & Critical Care Medicine; 9/15/2020, Vol. 202 Issue 6, p843-852, 13p |
| مصطلحات موضوعية: |
PULMONARY hypertension, NEUROFIBROMATOSIS 1, CARDIAC catheterization, PULMONARY artery, VASCULAR remodeling |
| مستخلص: |
Rationale: Pulmonary hypertension (PH) associated with neurofibromatosis type 1 (NF1) is a rare and largely unknown complication of NF1.Objectives: To describe characteristics and outcomes of PH-NF1.Methods: We reported the clinical, functional, radiologic, histologic, and hemodynamic characteristics, response to pulmonary arterial hypertension (PAH)-approved drugs, and transplant-free survival of patients with PH-NF1 from the French PH registry.Measurements and Main Results: We identified 49 PH-NF1 cases, characterized by a female/male ratio of 3.9 and a median (minimum-maximum) age at diagnosis of 62 (18-82) years. At diagnosis, 92% were in New York Heart Association functional class III or IV. The 6-minute-walk distance was 211 (0-460) m. Pulmonary function tests showed low DlCO (30% [12-79%]) and severe hypoxemia (PaO2 56 [38-99] mm Hg). Right heart catheterization showed severe precapillary PH with a mean pulmonary artery pressure of 45 (10) mm Hg and a pulmonary vascular resistance of 10.7 (4.2) Wood units. High-resolution computed tomography images revealed cysts (76%), ground-glass opacities (73%), emphysema (49%), and reticulations (39%). Forty patients received PAH-approved drugs with a significant improvement in functional class and hemodynamic parameters. Transplant-free survival at 1, 3, and 5 years was 87%, 54%, and 42%, respectively, and four patients were transplanted. Pathologic assessment showed nonspecific interstitial pneumonia and major pulmonary vascular remodeling.Conclusions: PH-NF1 is characterized by a female predominance, a low DlCO, and severe functional and hemodynamic impairment. Despite a potential benefit of PAH treatment, prognosis remains poor, and double-lung transplantation is an option for eligible patients. [ABSTRACT FROM AUTHOR] |
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| قاعدة البيانات: |
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