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BMC Pulmonary Medicine

, 16:175

Infectious, Rare and Idiopathic Pulmonary Diseases

Abstract

BackgroundNeurofibromatosis type 1 NF1 is a rare multisystem genetic disorder. During the course of the disease it can be rarely complicated with pulmonary hypertension PH which confers a dismal prognosis.

Case presentationWe describe the case of a 57-year-old female patient with NF1 complicated by severe precapillary PH despite dual disease-specific oral combination therapy. The patient was treated with initial atrial septostomy followed by administration of high-dose subcutaneous treprostinil with a favorable medium-term clinical and hemodynamic response.

ConclusionsPH secondary to NF1 may be successfully treated with the combination of atrial septostomy and PH targeted therapy in selected patients.

KeywordsNeurofibromatosis Pulmonary hypertension Septostomy Case report Abbreviations6MWTSix minute walk test

NF1Neurofibromatosis type I

NT-proBNPN-terminal pro-brain natriuretic peptide

PHPulmonary hypertension

PAHPulmonary arterial hypertension

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Autor: George Giannakoulas - Panagiotis Savvoulidis - Vasilios Grosomanidis - Sophia-Anastasia Mouratoglou - Haralambos Karvounis -

Fuente: https://link.springer.com/







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