Maruyama Hidekazu, Sakai Satoshi, Suzuki Shoji
Department of Cardiology, National Hospital Organization Kasumigaura Medical Center, 2-7-14 Shimotakatsu, Tsuchiura, Ibaraki 300-8585, Japan.
Department of Cardiology, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki 305-8577, Japan.
Eur Heart J Case Rep. 2023 Aug 4;7(8):ytad365. doi: 10.1093/ehjcr/ytad365. eCollection 2023 Aug.
The use of pulmonary vasodilators for pulmonary arterial hypertension (PAH) has led to a favourable prognosis. In contrast, pulmonary veno-occlusive disease (PVOD) is characterized by the possibility of severe pulmonary oedema after specific PAH therapy. Pulmonary oedema presumably develops in association with pulmonary arterial vasodilation without concomitant pulmonary venodilation. The venous circulation maximally dilates with small amounts of nitroglycerine.
A 59-year-old woman with advanced PVOD was referred to our hospital. We performed a right heart catheterization after administering combination therapy with selexipag and macitentan, and intravenous nitroglycerine at 0.2 and 0.4 μg/kg/min decreased pulmonary arterial wedge pressure (PAWP) and mean pulmonary arterial pressure (PAP) to minimal levels. The final dose of 1 μg/kg/min yielded an ∼20% decrease in mean PAP and pulmonary vascular resistance (PVR).
Here, we described the acute effect of intravenous nitroglycerine on PAWP and PVR in a patient with PVOD. This case highlights the venodilation response even in advanced PVOD, suggesting the importance of further research into selective venous dilators as potent therapy.
使用肺血管扩张剂治疗肺动脉高压(PAH)已带来良好预后。相比之下,肺静脉闭塞病(PVOD)的特点是在接受特定的PAH治疗后可能发生严重肺水肿。肺水肿可能是在肺动脉扩张而肺静脉未随之扩张的情况下发生的。静脉循环在使用少量硝酸甘油后会达到最大程度的扩张。
一名患有晚期PVOD的59岁女性被转诊至我院。我们在给予司来帕格和马昔腾坦联合治疗后进行了右心导管检查,静脉注射硝酸甘油,剂量为0.2和0.4μg/kg/min时,肺动脉楔压(PAWP)和平均肺动脉压(PAP)降至最低水平。最终剂量为1μg/kg/min时,平均PAP和肺血管阻力(PVR)降低了约20%。
在此,我们描述了静脉注射硝酸甘油对一名PVOD患者的PAWP和PVR的急性影响。该病例突出了即使在晚期PVOD中也存在静脉扩张反应,提示进一步研究选择性静脉扩张剂作为有效治疗方法的重要性。