Primary Pulmonary Hypertension Treated with Endothelin-1 Antagonist, Bosentan: Case Report and Review of the Literature

原發性肺動脈高壓是一種罕見和持續性惡化與致命的疾病。在臨床上呈現持續性的高肺動脈壓而無其它的致病因素。它的診斷是利用排除法。如果沒有治療的話，從診斷確定開始，平均存活約2.8年。目前治療的方法包括氧氣輔助、使用抗凝血劑、利尿劑、毛地黃、鈣離子阻斷劑、前列腺素和吸入性一氧化氮；然而，內皮素－1受體拮抗劑較少被報告。我們提出一位19歲的女性表現二個月持續性呼吸困難和運動不能，經過一系列的檢查之後，診斷為重度原發性肺動脈高壓。建議接受肺移植手術，但病人猶豫不決。因為心臟衰竭愈來愈惡化，開始接受內皮素－l受體拮抗劑Bosentan治療。經過16週的治療後，病人的心臟衰竭症狀和運動功能有明顯改善。

關鍵字

原發性肺動脈高壓；內皮素拮抗劑； Bosentan

並列摘要

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.

並列關鍵字

Primary pulmonary hypertension ； Endothelin receptor antagonist ； Bosentan

國際替代計量

Primary Pulmonary Hypertension Treated with Endothelin-1 Antagonist, Bosentan: Case Report and Review of the Literature

全文下載

主題瀏覽