Successful bosentan therapy for pulmonary arterial hypertension associated with hereditary hemorrhagic telangiectasia.
Hereditary hemorrhagic telangiectasia (HHT) is a hereditary vascular disease with multiple arterial venous malformations. Pulmonary arterial hypertension is occasionally associated with HHT; however, there is no evidence of targeted therapy for this subgroup of patients. This report describes a 37-year-old woman who presented with dyspnea on exertion. She was finally diagnosed with pulmonary arterial hypertension associated with HHT (HHT2, MIM 600376), and had multiple arterial venous malformations in the lung, liver and pancreas. An oral dual endothelin receptor (ET(A)/ET(B)) antagonist, bosentan, was prescribed, and her symptoms, exercise capacity and laboratory findings improved greatly after treatment. Pulmonary arterial pressure also decreased. Endothelin receptor antagonist is a possible treatment option for pulmonary arterial hypertension associated with HHT.
Division of Cardiology, Department of Medicine, Cardiovascular Imaging Center, Cardiac and Vascular Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Irwon-dong, Gangnam-gu, Seoul, 135-710, Republic of Korea.
Sung-A
SA
Chang
Shin Yi
SY
Jang
Chang-Seok
CS
Ki
I-Seok
IS
Kang
Duk-Kyung
DK
Kim
D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D001794:Blood Pressure / Q000187:drug effects; D000077300:Bosentan; D015150:Echocardiography, Doppler; D065128:Endothelin Receptor Antagonists; D017079:Exercise Tolerance / Q000187:drug effects; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D020127:Recovery of Function; D013449:Sulfonamides / Q000627:therapeutic use*; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications* / Q000175:diagnosis; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D013449:Sulfonamides; D000077300:Bosentan