Is Pulmonary Arterial Hypertension in Neurofibromatosis Type 1 Secondary to a Plexogenic Arteriopathy?

CHEST Journal ◽  
2007 ◽  
Vol 132 (3) ◽  
pp. 798-808 ◽  
Author(s):  
Douglas R. Stewart ◽  
Joy D. Cogan ◽  
Mordechai R. Kramer ◽  
Wallace T. Miller ◽  
Leif E. Christiansen ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Pulmonary Arterial

scholarly journals Acute Deterioration of Pulmonary Arterial Hypertension (PAH) in a Patient with Neurofibromatosis Type 1 (NF1)

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Seiya Tanaka ◽  
Fuko Kawahara ◽  
Taro Miyamoto ◽  
Satoshi Tsurusaki ◽  
Yoshihito Sanuki ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Pressure ◽  
Arterial Hypertension ◽  
Neurofibromatosis Type 1 ◽  
Pulmonary Arterial Pressure ◽  
Neurofibromatosis Type ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Arterial

A 56-year-old woman was diagnosed as having chronic obstructive pulmonary disease with heavy smoking. Mild pulmonary hypertension (mean pulmonary arterial pressure: 31 mmHg) was detected at the first visit. She was diagnosed with pulmonary hypertension due to pulmonary disease and medicated only with bronchodilators. Simultaneous, multiple freckling in the trunk of her body and café au lait macules in her back with some cutaneous neurofibromas were also detected. A plastic surgeon removed one of the neurofibromas and pathologically diagnosed it as neurofibromatosis type 1 (NF1). We finally rediagnosed her with pulmonary hypertension with unclear and/or multifactorial factors when she deteriorated 1 year after being treated only with bronchodilators. We then administrated upfront combination therapy with macitentan and tadalafil. Mean pulmonary arterial pressure rapidly improved. Learning Objective. Pulmonary arterial hypertension (PAH) in neurofibromatosis type 1 (NF1) can occur due to lung disease or due to certain involvement of pulmonary arteries, or a combination of both. Increased awareness of PAH in NF1 is very important for patients survival. The current therapeutic strategy is almost identical to that of idiopathic PAH; however, there is no clinical evidence. Insights gained from clinical experiences should help identify promising novel therapeutic approaches in NF1-PAH.

scholarly journals Pulmonary arterial hypertension: A rare yet fatal complication of Neurofibromatosis Type 1

2019 ◽  
Vol 27 ◽  
pp. 100832
Author(s):  
Melanie Rojas ◽  
Ateeq Mubarik ◽  
Elizabeth Ann Henderson ◽  
Fatima Agha ◽  
Lakshpaul Chauhan ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Fatal Complication ◽  
Pulmonary Arterial

Pulmonary Arterial Hypertension in Neurofibromatosis Type 1

CHEST Journal ◽  
2017 ◽  
Vol 152 (4) ◽  
pp. A1017
Author(s):  
Amirahwaty Abdullah ◽  
Lydia Winnicka ◽  
James Walsh
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Pulmonary Arterial

scholarly journals Pulmonary arterial hypertension associated with neurofibromatosis type 1

2010 ◽  
Vol 2010 (nov26 1) ◽  
pp. bcr0520102961-bcr0520102961 ◽  
Author(s):  
M. F. Carrascosa ◽  
I. C. Larroque ◽  
J.-L. G. Rivero ◽  
J.-A. S.-Q. Garcia ◽  
M. C. Hoz ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Pulmonary Arterial

scholarly journals Polymorphism in the Angiotensin II Type 1 Receptor (AGTR1) is Associated With Age at Diagnosis in Pulmonary Arterial Hypertension

2009 ◽  
Vol 28 (4) ◽  
pp. 373-379 ◽  
Author(s):  
Wendy K. Chung ◽  
Liyong Deng ◽  
J. Sheila Carroll ◽  
Nicole Mallory ◽  
Beverly Diamond ◽  
...  
Keyword(s):  
Angiotensin Ii ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Age At Diagnosis ◽  
Pulmonary Arterial
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