Background

The pathogenesis of pulmonary hypertension (PH) in lymphangioleiomyomatosis (LAM) has not yet been completely clarified. The aim of this study was to conduct a noninvasive evaluation of the main hemodynamic mechanisms of exercise-induced PH in patients with LAM, assessed using exercise stress echocardiography.

Methods

Fifteen patients with LAM (mean age, 47 ± 13 years; all women) without resting PH were enrolled in a prospective single-center study and compared with 15 healthy female control subjects (mean age, 45.2 ± 8 years; P = .65). A complete echocardiographic study with Doppler tissue imaging was performed at baseline and during semisupine symptom-limited exercise testing to evaluate (1) left ventricular systolic and diastolic function, (2) right ventricular contractile function, (3) estimated pulmonary capillary wedge pressure, (4) estimated systolic and mean pulmonary artery pressure, and (5) estimated pulmonary vascular resistance.

Results

Compared with healthy control subjects, patients with LAM during exercise showed echocardiographic signs of right ventricular overload and right ventricular systolic dysfunction and significant increases in mean pulmonary artery pressure (14.4 ± 6.5 vs 4.2 ± 3.1 mm Hg, P < .0001), pulmonary vascular resistance (+68.3 ± 42.1 vs −0.1 ± 18.3 dyne-sec/cm⁵, P < .0001), and, unexpectedly, pulmonary capillary wedge pressure (+8.3 ± 5.3 vs −0.5 ± 1.3 mm Hg, P < .0001).

Conclusions

Exercise-induced PH in patients with LAM could be related not only to hypoxic pulmonary vascular vasoconstriction during exercise (precapillary PH) but also to a significant exercise-induced increase in estimated pulmonary capillary wedge pressure, probably secondary to diastolic dysfunction (postcapillary PH).

中文翻译：

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运动诱发的肺动脉高压在淋巴管平滑肌肌瘤病患者中的血流动力学机制：运动应激超声心动图的作用

背景

淋巴管平滑肌瘤病（LAM）中肺动脉高压（PH）的发病机理尚未完全阐明。这项研究的目的是对运动性超声心动图评估的LAM患者运动诱发的PH的主要血液动力学机制进行非侵入性评估。

方法

15名没有休息PH的LAM患者（平均年龄47±13岁；所有女性）参加了一项前瞻性单中心研究，并与15名健康的女性对照受试者（平均年龄45.2±8岁；P  = .65）进行了比较。 。在基线和半仰卧位症状受限运动测试期间进行了具有多普勒组织成像的完整超声心动图研究，以评估（1）左心室收缩和舒张功能，（2）右心室收缩功能，（3）估计的肺毛细血管楔压，（ 4）估计的收缩压和平均肺动脉压，以及（5）估​​计的肺血管阻力。

结果

与健康对照组相比，运动期间LAM患者的超声心动图表现为右心室超负荷和右心室收缩功能障碍，平均肺动脉压显着升高（14.4±6.5 vs 4.2±3.1 mm Hg，P  <.0001），肺血管阻力（+68.3±42.1 vs -0.1±18.3达因-秒/ cm ⁵，P  <.0001），以及出乎意料的是肺毛细血管楔压（+8.3±5.3 vs -0.5±1.3 mm Hg，P  <.0001） 。

结论

LAM患者运动引起的PH值不仅可能与运动过程中的低氧性肺血管收缩（毛细血管前PH）有关，而且还可能与运动引起的估计肺毛细血管楔压显着升高有关，可能是舒张功能障碍（毛细血管后PH）所致。