Cardiology Division, Clinical and Molecular Medicine Department, Sapienza University of Rome, Rome, Italy.
Cardiology Division, Clinical and Molecular Medicine Department, Sapienza University of Rome, Rome, Italy.
Int J Cardiol. 2017 Dec 1;248:326-332. doi: 10.1016/j.ijcard.2017.07.010. Epub 2017 Jul 14.
Pulmonary hypertension (PH) in patients with hypertrophic cardiomyopathy (HCM) has been investigated in a small number of studies. Purpose of this study was to assess the prevalence and its association with outcome in a population of consecutive HCM outpatients.
We retrospectively analyzed data of 361 consecutive HCM outpatients in whom echocardiographic measurements of pulmonary artery systolic pressure (PASP) were available at initial and most recent evaluation. Four different clinical groups were specifically investigated: patients without left ventricular outflow tract obstruction (group A, 165), with obstruction (group B, 126), patients diagnosed at the age≥65 (group C, 50) and patients with end stage (ES) HCM (group D, 20).
PH was identified in 41 (11.4%) of the 361 patients at initial evaluation while it has been recognized in 25 (7,8% [1.1%/year]) during a median follow-up of 3.4years. Analysis of subgroups showed that prevalence of PH increased from patient group A to D (8%, group A, 19%, group B, 28% group C, 70%, group D, respectively, p<0,01). During follow-up, patients with PH showed a significant higher HCM-related mortality (p=0.01) and morbidity (p<0.001) as compared with those without PH, but in multivariable analysis, PH resulted an independent risk factor only for HCM-related morbidity (HR=2.50, 95% CI 1.08-5.79, p=0.03).
PH affects a significant proportion of patients with HCM. Its prevalence varies according to different clinical profiles. It is associated with an unfavorable clinical outcome and is an independent predictor of morbidity.
已有少数研究调查了肥厚型心肌病（HCM）患者的肺动脉高压（PH）。本研究旨在评估连续 HCM 门诊患者人群中 PH 的患病率及其与结局的相关性。
我们回顾性分析了 361 例连续 HCM 门诊患者的超声心动图测量肺动脉收缩压（PASP）数据，这些数据可在初始和最近评估时获得。专门研究了以下四个不同的临床组：无左心室流出道梗阻的患者（组 A，165 例）、有梗阻的患者（组 B，126 例）、年龄≥65 岁的患者（组 C，50 例）和终末期（ES）HCM 患者（组 D，20 例）。
在初始评估时，361 例患者中有 41 例（11.4%）发现 PH，而在中位数为 3.4 年的随访期间，有 25 例（7.8% [1.1%/年]）被诊断为 PH。亚组分析显示，PH 的患病率从患者组 A 增加到 D（8%，组 A；19%，组 B；28%，组 C；70%，组 D，p<0.01）。在随访期间，与无 PH 的患者相比，PH 患者的 HCM 相关死亡率（p=0.01）和发病率（p<0.001）显著更高，但在多变量分析中，PH 仅作为 HCM 相关发病率的独立危险因素（HR=2.50，95%CI 1.08-5.79，p=0.03）。
PH 影响了相当一部分 HCM 患者。其患病率根据不同的临床特征而有所不同。它与不良临床结局相关，是发病率的独立预测因子。
