Murakami Tsutomu, Ohno Yohei, Noda Satoshi, Hashimoto Kaho, Horinouchi Hitomi, Ohmura Ryosuke, Miyamoto Junichi, Kamioka Norihiko, Ikari Yuji
Department of Cardiology, Tokai University School of Medicine, Kanagawa, Japan.
J Cardiol Cases. 2025 Jul 7;32(3):119-122. doi: 10.1016/j.jccase.2025.06.008. eCollection 2025 Sep.
A 74-year-old female presented with dyspnea [New York Heart Association (NYHA) class IV and 94 % percutaneous oxygen saturation at room air]. She was diagnosed with pre-capillary pulmonary arterial hypertension (PAH) due to connective tissue disease [mean pulmonary arterial wedge pressure (mPAWP): 6 mmHg; pulmonary arterial pressure (PAP): 93/36 [59] mmHg; pulmonary vascular resistance (PVR): 12.2 Wood units; cardiac index (CI): 2.95 L/min/m] and paradoxical low-flow low-gradient severe aortic stenosis (AS) [mean gradient: 16.7 mmHg; max jet velocity: 2.87 m/s; aortic valve area: 0.70 cm; left ventricular ejection fraction (LVEF): 65 %; stroke volume index (SVi): 31.1 mL/m]. The patient was treated for PAH, which was considered to be the underlying cause of the paradoxical low-flow low-gradient severe AS. After 10-month titration of riociguat (7.5 mg/day) and selexipag (1.6 mg/day), PAH [mPAWP: 9 mmHg; PAP: 55/23 (36) mmHg; PVR: 5.9 Wood units; CI: 3.10 L/min/m] improved and normal-flow high-gradient severe AS became evident (mean gradient: 41.9 mmHg; max jet velocity: 4.04 m/s; aortic valve area: 0.70 cm; LVEF: 65 %; SVi: 41.7 mL/m). Although symptoms improved to NYHA class II, exertional dyspnea persisted. Accordingly, medication dosages were further increased, and transcatheter aortic valve replacement was successfully performed 12 months after treatment initiation.
We aimed to understand how group 1 pulmonary arterial hypertension (PAH) can lead to paradoxical low-flow low-gradient severe aortic stenosis (AS), recognize its key clinical and hemodynamic features, and differentiate it from group 2 pulmonary hypertension (PH) associated with left heart disease, including isolated post-capillary PH and combined post- and pre-capillary PH. We also explored hemodynamic changes after PAH therapy, including transition to normal-flow high-gradient severe AS.
一名74岁女性出现呼吸困难（纽约心脏协会[NYHA]心功能IV级，室内空气下经皮氧饱和度为94%）。她被诊断为因结缔组织病导致的毛细血管前肺动脉高压（PAH）[平均肺动脉楔压（mPAWP）：6 mmHg；肺动脉压（PAP）：93/36 [59] mmHg；肺血管阻力（PVR）：12.2伍德单位；心脏指数（CI）：2.95 L/min/m²]以及矛盾性低流量低梯度重度主动脉瓣狭窄（AS）[平均压差：16.7 mmHg；最大射流速度：2.87 m/s；主动脉瓣面积：0.70 cm²；左心室射血分数（LVEF）：65%；每搏量指数（SVi）：31.1 mL/m²]。该患者接受了PAH治疗，PAH被认为是矛盾性低流量低梯度重度AS的潜在病因。在对利奥西呱（7.5 mg/天）和司来帕格（1.6 mg/天）进行10个月的滴定治疗后，PAH [mPAWP：9 mmHg；PAP：55/23 (36) mmHg；PVR：5.9伍德单位；CI：3.10 L/min/m²]有所改善，正常流量高梯度重度AS变得明显（平均压差：41.9 mmHg；最大射流速度：4.04 m/s；主动脉瓣面积：0.70 cm²；LVEF：65%；SVi：41.7 mL/m²）。尽管症状改善至NYHA心功能II级，但劳力性呼吸困难仍然存在。因此，进一步增加了药物剂量，并在开始治疗12个月后成功进行了经导管主动脉瓣置换术。
我们旨在了解1型肺动脉高压（PAH）如何导致矛盾性低流量低梯度重度主动脉瓣狭窄（AS），识别其关键的临床和血流动力学特征，并将其与2型肺动脉高压（PH）相关的左心疾病相鉴别，包括单纯毛细血管后PH和毛细血管后与毛细血管前合并的PH。我们还探讨了PAH治疗后的血流动力学变化，包括转变为正常流量高梯度重度AS。
