肺动脉高压左心室舒张功能障碍特点和预后预测价值

Characteristics and Prognostic Value of Left Ventricular Diastolic Dysfunction in Pulmonary Arterial Hypertension

目的探究在特发性肺动脉高压和结缔组织疾病相关性肺动脉高压患者,左心室舒张功能障碍特点及其与疾病严重程度和长期预后的关系。方法应用超声心动图方法探讨特发性肺动脉高压和结缔组织疾病相关性肺动脉高压左室舒张功能障碍的特点,并评估与预后的关系。结果研究共入选特发性肺动脉高压和结缔组织疾病相关肺动脉高压患者195名,有7名患者资料不全,共188名患者进入研究。中位随访时间为34.8个月,其中有4名患者失访,37名患者死亡。研究总体的性别比为女性:男性为3.3:1(144/44)。正常舒张功能、松弛功能破坏、假性正常化和限制性充盈障碍患者比例分别为37.8%,38.3%,3.2%和20.7%。舒张早期二尖瓣血流流速E峰与六分钟步行距离(r=0.337,P值<0.001)、三尖瓣环收缩期位移(r=0.417,P值<0.001)和三尖瓣环组织多普勒运动速度(r=0.493,P值<0.001)正相关;与肺血管阻力(r=-0.367,P值<0.001)负相关。E峰≥0.57m/s组患者生存率显著高于E峰<0.57m/s组,P值=0.020。经过校正年龄等因素后,两组生存率差异仍然有统计学意义。结论左室舒张功能障碍患者中松弛功能破坏情况占多数,舒张早期二尖瓣血流流速E峰的降低伴随肺动脉高压更严重的病情和更差的预后。

Objective To investigate the characteristics of left ventricular （LV） diastolic dysfunction, and its association with disease severity and outcomes, in patients with idiopathic and connective tissue disease-associated pulmonary arterial hypertension （PAH）. Methods We identified subjects with heritable and connective tissue disease-associated PAII who underwent Doppler echocardiography and right-sided heart catheterization. Echocardiographic diastolic parameters were assessed in each patient. Results A total of 188 patients met the inclusion criteria. During a median follow-up of 34.8 months, 4 patients were lost to follow-up and 37 patients died. The sex ratio of females to males was 3.3：1 （n = 144/44） in the total population. Normal LV diastolic function, impaired relaxation, pseudonormalization and restrictive LV filing were seen in 37.8%, 38.3%, 3.2% and 20.7% of the patients, respectively. Transmitral flow early diastolic （peak E） velocity was directly associated with 6-min walk distance（r = 0.337, P 〈 0.001）, TAPSE（r =0.417, P 〈 0.001）, and STr（r =0.493, P 〈 0.001）, and inversely associated with the pulmonary vascular resistance（r =-0.367, P 〈 0.001）. Peak E velocity was associated with mortality adjusted for age and sex （P=0.020）. Conclusion LV diastolic dysfunction of the impaired relaxation type is observed in the majority of patients with PAH. A decrease in transmittal flow peak E velocity is associated with worse hemodynamics and outcome.