Atrial septostomy and disease targeting therapy in pulmonary hypertension secondary to neurofibromatosis.
| Title | Atrial septostomy and disease targeting therapy in pulmonary hypertension secondary to neurofibromatosis. |
| Publication Type | Journal Article |
| Year of Publication | 2016 |
| Authors | Giannakoulas, G., Savvoulidis P., Grosomanidis V., Mouratoglou S-A., Karvounis H., & Hadjimiltiades S. |
| Journal | BMC Pulm Med |
| Volume | 16 |
| Issue | 1 |
| Pagination | 175 |
| Date Published | 2016 12 07 |
| ISSN | 1471-2466 |
| Keywords | Antihypertensive Agents, Cardiac Surgical Procedures, Echocardiography, Epoprostenol, Female, Heart Atria, Hemodynamics, Humans, Hypertension, Pulmonary, Injections, Subcutaneous, Middle Aged, Neurofibromatosis 1, Prognosis, Ventricular Remodeling |
| Abstract | BACKGROUND: 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.CASE PRESENTATION: 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.CONCLUSIONS: PH secondary to NF1 may be successfully treated with the combination of atrial septostomy and PH targeted therapy in selected patients. |
| DOI | 10.1186/s12890-016-0337-7 |
| Alternate Journal | BMC Pulm Med |
| PubMed ID | 27927206 |
| PubMed Central ID | PMC5142319 |
