van der Bruggen Cathelijne E, Spruijt Onno A, Nossent Esther J, Trip Pia, Marcus J Tim, de Man Frances S, Jan Bogaard Harm, Vonk Noordegraaf Anton
Department of Pulmonary Medicine, VU University Medical Center, Amsterdam, The Netherlands.
Department of Physics and Medical Technology, VU University Medical Center, Amsterdam, The Netherlands.
Pulm Circ. 2017 Mar 13;7(1):137-144. doi: 10.1086/690016. eCollection 2017 Mar.
Patients with idiopathic pulmonary arterial hypertension (IPAH) and a reduced diffusion capacity of the lung for carbon monoxide (DLCO) have a worse survival compared to IPAH patients with a preserved DLCO. Whether this poor survival can be explained by unresponsiveness to pulmonary hypertension (PH)-specific vasodilatory therapy is unknown. Therefore, the aim of this study was to evaluate the hemodynamic and cardiac response to PH-specific vasodilatory therapy in patients with IPAH and a reduced DLCO. Retrospectively, we studied treatment naïve hereditary and IPAH patients diagnosed between January 1990 and May 2015 at the VU University Medical Center. After exclusion of participants without available baseline DLCO measurement or right heart catheterization data and participants carrying a BMPR2 mutation, 166 participants could be included in this study. Subsequently, hemodynamics, cardiac function, exercise capacity, and oxygenation at baseline and after PH-specific vasodilatory therapy were compared between IPAH patients with a preserved DLCO (DLCO >62%), IPAH patients with a moderately reduced DLCO (DLCO 43-62%), and IPAH patients with a severely reduced DLCO (DLCO <43%). Baseline hemodynamics and right ventricular function were not different between groups. Baseline oxygenation was worse in patients with IPAH and a severely reduced DLCO. Hemodynamics and cardiac function improved in all groups after PH-specific vasodilatory therapy without worsening of oxygenation at rest or during exercise. Patients with IPAH and a severely reduced DLCO show a similar response to PH-specific vasodilatory therapy in terms of hemodynamics, cardiac function, and exercise capacity as patients with IPAH and a moderately reduced or preserved DLCO.
与一氧化碳肺弥散量（DLCO）正常的特发性肺动脉高压（IPAH）患者相比，DLCO降低的IPAH患者生存率更低。这种较差的生存率是否可以用对肺动脉高压（PH）特异性血管舒张治疗无反应来解释尚不清楚。因此，本研究的目的是评估DLCO降低的IPAH患者对PH特异性血管舒张治疗的血流动力学和心脏反应。我们回顾性研究了1990年1月至2015年5月在VU大学医学中心诊断的未经治疗的遗传性和IPAH患者。排除没有可用基线DLCO测量值或右心导管检查数据的参与者以及携带BMPR2突变的参与者后，166名参与者可纳入本研究。随后，比较了DLCO正常（DLCO>62%）的IPAH患者、DLCO中度降低（DLCO 43-62%）的IPAH患者和DLCO严重降低（DLCO<43%）的IPAH患者在基线时以及PH特异性血管舒张治疗后的血流动力学、心脏功能、运动能力和氧合情况。各组之间的基线血流动力学和右心室功能无差异。DLCO严重降低的IPAH患者基线氧合情况较差。PH特异性血管舒张治疗后，所有组的血流动力学和心脏功能均得到改善，静息或运动时氧合情况未恶化。在血流动力学、心脏功能和运动能力方面，DLCO严重降低的IPAH患者对PH特异性血管舒张治疗的反应与DLCO中度降低或正常的IPAH患者相似。
