Cardiology Division, Cardiac Arrhythmia Center and Cardiomyopathies Unit, St. Camillo-Forlanini Hospital, Rome, Italy.
Cardiac Unit, G. da Saliceto Hospital, AUSL Piacenza and University of Parma, Parma, Italy.
Int J Cardiovasc Imaging. 2022 Nov;38(11):2345-2352. doi: 10.1007/s10554-022-02671-3. Epub 2022 Jun 28.
Pulmonary arterial hypertension (PAH), documented in a significant portion of hypertrophic cardiomyopathy (HCM) patients, has been shown to adversely impact prognosis. In most HCM patients congestive symptoms are consistently elicited by exercise, thus suggesting the need for a provocative test to assess cardiac hemodynamics during effort. Combining cardiopulmonary exercise test (CPET) with echocardiography, we aimed to evaluate the presence of exercise induced pulmonary arterial hypertension (EiPAH), its role in functional limitation and its prognostic significance in a cohort of patients with obstructive and non-obstructive HCM. Study population included 182 HCM patients evaluated combining CPET and stress echocardiography. Left-ventricular outflow tract (LVOT) velocities, trans-tricuspid gradient, and cardiopulmonary variables were continuously measured. Thirty-seven patients (20%) developed EiPAH, defined as systolic pulmonary arterial pressure (sPAP) > 40 mmHg during exercise. EiPAH was associated with lower exercise performance, larger left atrial volumes, higher LVOT gradient and higher VE/VCO slope. At multivariable analysis baseline sPAP (p < 0.0001) and baseline LVOT obstruction (p = 0.028) were significantly associated with EiPAH. Kaplan-Meier curve analysis showed EiPAH was a significant predictor of HCM-related morbidity (Hazard Ratio 6.21, 95% CI 1.47-26.19; p = 0.05; 4.21, 95% CI 1.94-9.12; p < 0.001 for the primary and the secondary endpoint respectively). EiPAH was present in about one fifth of HCM patients without evidence of elevated pulmonary pressures at rest and was associated with adverse clinical outcome. Diagnosing EiPAH by exercise echocardiography/CPET may help physicians to detect early stage of PAH thus allowing a closer clinical monitoring and individualized therapies.
肺动脉高压（PAH）在很大一部分肥厚型心肌病（HCM）患者中都有记录，它已被证明会对预后产生不利影响。在大多数 HCM 患者中，充血性症状始终是由运动引起的，因此需要进行激发试验来评估运动期间的心脏血液动力学。我们结合心肺运动试验（CPET）和超声心动图，旨在评估存在运动诱导的肺动脉高压（EiPAH），其在功能受限中的作用及其在梗阻性和非梗阻性 HCM 患者中的预后意义。研究人群包括 182 名接受 CPET 和应激超声心动图评估的 HCM 患者。连续测量左心室流出道（LVOT）速度、跨三尖瓣梯度和心肺变量。37 名患者（20%）出现 EiPAH，定义为运动时收缩期肺动脉压（sPAP）>40mmHg。EiPAH 与运动表现降低、左心房容积增大、LVOT 梯度增加和 VE/VCO 斜率增加相关。多变量分析显示，基线 sPAP（p<0.0001）和基线 LVOT 梗阻（p=0.028）与 EiPAH 显著相关。Kaplan-Meier 曲线分析显示，EiPAH 是 HCM 相关发病率的显著预测因子（风险比 6.21，95%CI 1.47-26.19；p=0.05；4.21，95%CI 1.94-9.12；p<0.001 为主要和次要终点）。约五分之一的 HCM 患者在静息时没有证据表明肺动脉压升高，但存在 EiPAH，与不良临床结局相关。通过运动超声心动图/CPET 诊断 EiPAH 可以帮助医生早期发现 PAH，从而进行更密切的临床监测和个体化治疗。
