Article
Phenotype and Outcomes of Pulmonary Hypertension Associated with Neurofibromatosis Type 1.
American journal of respiratory and critical care medicine - 15 Sept 2020
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, Bertoletti Laurent, Gueffet Jean-Pierre, Chaouat Ari, Goupil François, Moceri Pamela, Borie Raphael, Fadel Elie, Wolkenstein Pierre, Brillet Pierre-Yves, Simonneau Gérald, Sitbon Olivier, Humbert Marc, Montani David
Abstract excerpt
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...
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