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首页> 外文期刊>Pulmonary Circulation >Pulmonary Arterial Hypertension in a Patient with β-Thalassemia Intermedia and Reversal with Infusion Epoprostenol Then Transition to Oral Calcium Channel Blocker Therapy: Review of Literature:
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Pulmonary Arterial Hypertension in a Patient with β-Thalassemia Intermedia and Reversal with Infusion Epoprostenol Then Transition to Oral Calcium Channel Blocker Therapy: Review of Literature:

机译：β-地中海贫血中级患者的肺动脉高压并输注依前列烯醇逆转然后过渡为口服钙通道阻滞剂治疗：文献综述：

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摘要

Pulmonary arterial hypertension (PAH) is a potentially life-threatening complication of thalassemia. A sexagenarian with β-thalassemia intermedia presented with new-onset dyspnea and syncope. Right heart catheterization confirmed severe PAH. Her functional class IV symptoms and severely elevated mean pulmonary artery pressure prompted the initiation of continuous epoprostenol therapy. Clinical follow-up documented significant improvement in functional class, 6-minute walk distance, and right ventricular size and function as well as pulmonary arterial pressure on echocardiogram. At the patient's request, epoprostenol was down-titrated and eventually discontinued. The patient was then safely transitioned to nifedipine therapy after verification of vasoresponsiveness.
机译：肺动脉高压（PAH）是地中海贫血的潜在威胁生命的并发症。一位患有中间型β地中海贫血的六性生殖器官患者，出现新发作的呼吸困难和晕厥。右心导管检查证实有严重的PAH。她的IV级功能症状和平均肺动脉压严重升高，促使人们开始进行持续的依泊汀治疗。临床随访表明，功能分类，6分钟步行距离，右心室大小和功能以及超声心动图上的肺动脉压都有明显改善。应患者的要求，依普舒替尼滴定度低，最终停药。在确认血管反应性之后，该患者随后安全地过渡到硝苯地平治疗。

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