title: Atrial septostomy and disease targeting therapy in pulmonary hypertension secondary to neurofibromatosis.
abstract: Neurofibromatosis 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.
  
  
  
  We 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.
  
  
  
  PH secondary to NF1 may be successfully treated with the combination of atrial septostomy and PH targeted therapy in selected patients.
authors.affiliation: Department of Cardiology, AHEPA University Hospital, Aristotle University of Thessaloniki, Stilp. Kiriakidi 1, Thessaloniki, 54637, Greece. giannak@med.auth.gr.
authors.forename: George
authors.initials: G
authors.lastname: Giannakoulas
authors.affiliation: Department of Cardiology, AHEPA University Hospital, Aristotle University of Thessaloniki, Stilp. Kiriakidi 1, Thessaloniki, 54637, Greece.
authors.forename: Panagiotis
authors.identifier: 0000-0002-2266-8357
authors.initials: P
authors.lastname: Savvoulidis
authors.affiliation: Department of Anesthesiology and Intensive Care Medicine, AHEPA University Hospital, Aristotle University of Thessaloniki, Stilp. Kiriakidi 1, Thessaloniki, 54637, Greece.
authors.forename: Vasilios
authors.initials: V
authors.lastname: Grosomanidis
authors.affiliation: Department of Cardiology, AHEPA University Hospital, Aristotle University of Thessaloniki, Stilp. Kiriakidi 1, Thessaloniki, 54637, Greece.
authors.forename: Sophia-Anastasia
authors.initials: SA
authors.lastname: Mouratoglou
authors.affiliation: Department of Cardiology, AHEPA University Hospital, Aristotle University of Thessaloniki, Stilp. Kiriakidi 1, Thessaloniki, 54637, Greece.
authors.forename: Haralambos
authors.initials: H
authors.lastname: Karvounis
authors.affiliation: Department of Cardiology, AHEPA University Hospital, Aristotle University of Thessaloniki, Stilp. Kiriakidi 1, Thessaloniki, 54637, Greece.
authors.forename: Stavros
authors.initials: S
authors.lastname: Hadjimiltiades
mesh_terms: D000959:Antihypertensive Agents / Q000008:administration & dosage*; D006348:Cardiac Surgical Procedures / Q000379:methods*; D004452:Echocardiography; D011464:Epoprostenol / Q000008:administration & dosage / Q000031:analogs & derivatives*; D005260:Female; D006325:Heart Atria / Q000601:surgery; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology / Q000628:therapy*; D007279:Injections, Subcutaneous; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications*; D011379:Prognosis; D020257:Ventricular Remodeling
chemical_list: D000959:Antihypertensive Agents; D011464:Epoprostenol; C427248:treprostinil
keywords: Case report; Neurofibromatosis; Pulmonary hypertension; Septostomy