Hugues T, Lemoigne F, Macone F, Cerboni P, Gibelin P
Service de cardiologie, centre hospitalier Princesse-Grace, Monaco, Monaco.
Ann Cardiol Angeiol (Paris). 2012 Feb;61(1):1-7. doi: 10.1016/j.ancard.2011.04.008. Epub 2011 May 25.
To characterize and compare patients with disproportionate PH versus patients with primary pulmonary arterial hypertension (PAH).
All patients referred to our cardiology unit for echocardiography from November 2006 to May 2008 and who have been followed by our pneumologist were screened for severe PH (i.e mean arterial pulmonary pressure>35-40 mmHg at rest). Patients were excluded if a factor that could influence pulmonary hemodynamics was present. We investigated these patients by pulmonary function tests, echocardiography and right heart catheterisation.
We reported 16 cases of severe PH in stable patients (n=8, chronic obstructive pulmonary disease-emphysema) and 13 patients with PAH. Our findings suggest that the patients with disproportionate PH had right heart dysfunction similar to that observed in PAH. But their outcomes were more severe. It seemed that specific vasodilatator therapy was not efficient.
对不成比例的肺动脉高压（PH）患者与原发性肺动脉高压（PAH）患者进行特征描述和比较。
对2006年11月至2008年5月转诊至我院心脏科进行超声心动图检查且随后由呼吸科医生随访的所有患者进行严重PH筛查（即静息时平均动脉肺动脉压>35 - 40 mmHg）。如果存在可影响肺血流动力学的因素，则将患者排除。我们通过肺功能测试、超声心动图和右心导管检查对这些患者进行了研究。
我们报告了16例稳定患者中的严重PH病例（n = 8，慢性阻塞性肺疾病 - 肺气肿）和13例PAH患者。我们的研究结果表明，不成比例的PH患者存在与PAH患者相似的右心功能障碍。但他们的预后更严重。似乎特定的血管扩张剂治疗无效。
