Archives of the Turkish Society of Cardiology
Type 1 neurofibromatosis complicated by pulmonary arterial hypertension: a case report [Turk Kardiyol Dern Ars]
Turk Kardiyol Dern Ars. 2017; 45(5): 458-461 | DOI: 10.5543/tkda.2016.36897

Type 1 neurofibromatosis complicated by pulmonary arterial hypertension: a case report

Murathan Küçük1, Can Ramazan Öncel2, Mustafa Uçar3, Aytül Belgi Yıldırım1
1Department of Cardiology, Akdeniz University Faculty of Medicine, Antalya, Turkey
2Department of Cardiology, Bucak State Hospital, Burdur, Turkey
3Department of Cardiology, Akhisar Mustafa Kirazoğlu State Hospital, Manisa, Turkey

Neurofibromatosis type I (NF1) is a rare genetic disease caused by mutations in the NF1 gene, which encodes the tumor suppressor neurofibromin. Precapillary pulmonary hypertension is a severe complication of NF1, initially described in patients with advanced parenchymal lung disease, which may complicate the course of NF1. Presently described is a case of neurofibromatosis complicated by pulmonary hypertension.

Keywords: Neurofibromatosis, pulmonary arterial hypertension, vasoreactivity test.

Murathan Küçük, Can Ramazan Öncel, Mustafa Uçar, Aytül Belgi Yıldırım. Type 1 neurofibromatosis complicated by pulmonary arterial hypertension: a case report. Turk Kardiyol Dern Ars. 2017; 45(5): 458-461

Corresponding Author: Can Ramazan Öncel, Türkiye
© copyright 2017 Archives of the Turkish Society of Cardiology
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