Acute Deterioration of Pulmonary Arterial Hypertension (PAH) in a Patient with Neurofibromatosis Type 1 (NF1)
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 wit...
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| Format: | Article |
| Language: | English |
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Wiley
2019-01-01
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| Series: | Case Reports in Cardiology |
| Online Access: | http://dx.doi.org/10.1155/2019/2987461 |
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| author | Seiya Tanaka Fuko Kawahara Taro Miyamoto Satoshi Tsurusaki Yoshihito Sanuki Kiyoshi Ozumi Takashi Harada Hiromi Tasaki |
| author_facet | Seiya Tanaka Fuko Kawahara Taro Miyamoto Satoshi Tsurusaki Yoshihito Sanuki Kiyoshi Ozumi Takashi Harada Hiromi Tasaki |
| author_sort | Seiya Tanaka |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-0d71100e5883498899a8d0067e1d029b |
| institution | Kabale University |
| issn | 2090-6404 2090-6412 |
| language | English |
| publishDate | 2019-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Cardiology |
| spelling | doaj-art-0d71100e5883498899a8d0067e1d029b2025-02-03T06:08:09ZengWileyCase Reports in Cardiology2090-64042090-64122019-01-01201910.1155/2019/29874612987461Acute Deterioration of Pulmonary Arterial Hypertension (PAH) in a Patient with Neurofibromatosis Type 1 (NF1)Seiya Tanaka0Fuko Kawahara1Taro Miyamoto2Satoshi Tsurusaki3Yoshihito Sanuki4Kiyoshi Ozumi5Takashi Harada6Hiromi Tasaki7Department of Cardiovascular Medicine, Kitakyushu Municipal Yahata Hospital, 2-6-2 Okura, Yahatahigashi-ku, Kitakyushu 805-8534, JapanDepartment of Cardiovascular Medicine, Kitakyushu Municipal Yahata Hospital, 2-6-2 Okura, Yahatahigashi-ku, Kitakyushu 805-8534, JapanDepartment of Cardiovascular Medicine, Kitakyushu Municipal Yahata Hospital, 2-6-2 Okura, Yahatahigashi-ku, Kitakyushu 805-8534, JapanDepartment of Internal Medicine, Kitakyushu Municipal Yahata Hospital, 2-6-2 Okura, Yahatahigashi-ku, Kitakyushu 805-8534, JapanDepartment of Cardiovascular Medicine, Kitakyushu Municipal Yahata Hospital, 2-6-2 Okura, Yahatahigashi-ku, Kitakyushu 805-8534, JapanDepartment of Cardiovascular Medicine, Kitakyushu Municipal Yahata Hospital, 2-6-2 Okura, Yahatahigashi-ku, Kitakyushu 805-8534, JapanDepartment of Cardiovascular Medicine, Kitakyushu Municipal Yahata Hospital, 2-6-2 Okura, Yahatahigashi-ku, Kitakyushu 805-8534, JapanDepartment of Cardiovascular Medicine, Kitakyushu Municipal Yahata Hospital, 2-6-2 Okura, Yahatahigashi-ku, Kitakyushu 805-8534, JapanA 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.http://dx.doi.org/10.1155/2019/2987461 |
| spellingShingle | Seiya Tanaka Fuko Kawahara Taro Miyamoto Satoshi Tsurusaki Yoshihito Sanuki Kiyoshi Ozumi Takashi Harada Hiromi Tasaki Acute Deterioration of Pulmonary Arterial Hypertension (PAH) in a Patient with Neurofibromatosis Type 1 (NF1) Case Reports in Cardiology |
| title | Acute Deterioration of Pulmonary Arterial Hypertension (PAH) in a Patient with Neurofibromatosis Type 1 (NF1) |
| title_full | Acute Deterioration of Pulmonary Arterial Hypertension (PAH) in a Patient with Neurofibromatosis Type 1 (NF1) |
| title_fullStr | Acute Deterioration of Pulmonary Arterial Hypertension (PAH) in a Patient with Neurofibromatosis Type 1 (NF1) |
| title_full_unstemmed | Acute Deterioration of Pulmonary Arterial Hypertension (PAH) in a Patient with Neurofibromatosis Type 1 (NF1) |
| title_short | Acute Deterioration of Pulmonary Arterial Hypertension (PAH) in a Patient with Neurofibromatosis Type 1 (NF1) |
| title_sort | acute deterioration of pulmonary arterial hypertension pah in a patient with neurofibromatosis type 1 nf1 |
| url | http://dx.doi.org/10.1155/2019/2987461 |
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