Phenotype and Outcomes of Pulmonary Hypertension Associated with Neurofibromatosis Type 1.

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.
PMID: 32437637 [PubMed - as supplied by publisher]

Source: American Journal of Respiratory and Critical Care Medicine - May 20, 2020 Category: Respiratory Medicine Authors: Jutant EM, Jaïs X, Girerd B, Savale L, Ghigna MR, Perros F, Mignard X, Jevnikar M, Bourlier D, Prevot G, Tromeur C, Bauer F, Bergot E, Dauphin C, Favrolt N, Traclet J, Soumagne T, De Groote P, Chabanne C, Magro P, Bertoletti L, Gueffet JP, Chaouat A, Gou Tags: Am J Respir Crit Care Med Source Type: research