Vaso reactivity test using inhaled nitric oxide for pulmonary arterial hypertension accompanied by severe interstitial lung disease attributed to systemic sclerosis: A case report
Vaso reactivity test using inhaled nitric oxide for pulmonary arterial hypertension accompanied by severe interstitial lung disease attributed to systemic sclerosis: A case report
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使用吸入一氧化氮进行血管反应性试验，用于治疗肺动脉高压并伴有系统性硬化症导致的严重间质性肺疾病：病例报告
DOI：
10.1016/j.jccase.2021.08.003
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发表时间：
2022
影响因子：
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通讯作者：
Yoshiyama Minoru
中科院分区：
文献类型：
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作者：
Yamaguchi Tomohiro;Izumiya Yasuhiro;Hayashi Ou;Hayashi Hiroya;Ogawa Mana;Shibata Atsushi;Yamazaki Takanori;Yoshiyama Minoru
一位70岁男性，因局限性皮肤系统性硬化症导致严重间质性肺炎，因呼吸困难恶化而转介到我们的机构。高分辨率计算机断层扫描与以前的图像相比没有显示出相当大的进展，而经胸超声心动图显示严重的右心室功能障碍。在室内空气中，氧饱和度降至84%。血液检查显示血浆脑钠肽水平升高（289.4 pg/mL）。右心导管插入术（RHC）显示在室内空气中平均肺动脉压（mPAP）非常高，为48 mmHg。使用吸入一氧化氮的血管反应性试验显示mPAP、肺血管阻力（PVR）和动脉血氧分压改善。这些结果表明，患者对肺动脉高压（PH）靶向药物有反应。然后，我们处方他达拉非10 mg和吸入伊洛前列素5 µg，每日6次。在开始PH靶向药物治疗3周后，RHC显示血流动力学改善与血管反应性试验中的血流动力学变化相似（mPAP：28 mmHg; PVR：4.2 W.U.）。患者出院，症状改善。当PH合并系统性硬化症和严重间质性肺病时，RHC期间吸入一氧化氮可能有助于考虑治疗策略。学习目的：在系统性硬化症和间质性肺病引起的肺动脉高压（PH）患者中，确定PH的血管扩张治疗适应症在临床上是困难的。在右心导管插入术期间使用吸入性一氧化氮进行血管反应性试验可能有助于识别对PH靶向治疗有反应的患者。
A 70-year-old man with severe interstitial pneumonia attributed to limited cutaneous systemic sclerosis was referred to our institution because of worsening dyspnea. High-resolution computed tomography did not show considerable progression compared with previous images, whereas transthoracic echocardiography showed severe right ventricular dysfunction. Oxygen saturation was decreased to 84% at room air. A blood test showed an increase in the plasma brain natriuretic peptide level (289.4 pg/mL). Right heart catheterization (RHC) showed a remarkably high mean pulmonary arterial pressure (mPAP) of 48 mmHg at room air. A vaso reactivity test using inhaled nitric oxide showed improvement of mPAP, pulmonary vascular resistance (PVR), and partial pressure of arterial oxygen. These findings suggested that the patient responded to pulmonary hypertension (PH)-targeted drugs. We then prescribed tadalafil 10 mg and inhaled iloprost 5 µg six times daily. Three weeks after initiating PH-targeted drugs, RHC indicated hemodynamic improvement similar to hemodynamic changes in the vaso reactivity test (mPAP: 28 mmHg; PVR: 4.2 W.U.). He was discharged with improved symptoms. Inhaled nitric oxide during RHC might be helpful to consider the treatment strategy when patients have PH comorbid systemic sclerosis and severe interstitial lung disease.<Learning objective:In patients with pulmonary hypertension (PH) attributed to systemic sclerosis and interstitial lung disease, determining the indication of vaso dilatory therapies for PH is clinically difficult. A vaso reactivity test using inhaled nitric oxide during right heart catheterization might be useful for identifying patients who will respond to PH-targeted therapy.>