Primary Pulmonary Hypertension Treated with Endothelin-1 Antagonist, Bosentan: Case Report and Review of the Literature
نویسندگان
چکیده
Primary pulmonary hypertension (PPH) is a rare and progressive fatal disease. PPH is a diagnosis of exclusion, and median survival from the time of diagnosis is 2.8 years, if without treatment. Current options for treatment include oxygen, anticoagulation, diuretics, digoxin, calcium channel blockers, prostacyclin, and inhaled nitric oxide. However, endothelin-1 receptor antagonist (ERA) is less mentioned. We address a 19-year-old female who presented progressive dyspnea and exercise intolerance for 2 months. After series of studies, severe PPH was diagnosed. She hesitated to receive lung transplantation. Because of worsening of congestive heart failure (CHF), she started treatment with bosentan, an ERA. After 16 weeks of treatment, she experienced a great improvement of CHF and exercise capacity.
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