Sykes Michelle C, Ireland Christina, McSweeney Julia E, Rosenholm Emily, Andren Kristofer G, Kulik Thomas J
1 Department of Cardiology, Boston Children's Hospital, Boston, MA, USA.
2 Cardiovascular Nursing Patient Services, Boston Children s Hospital, Boston, MA, USA.
Pulm Circ. 2018 Apr-Jun;8(2):2045894018776894. doi: 10.1177/2045894018776894. Epub 2018 Apr 30.
Pulmonary vein stenosis (PVS) is associated with pulmonary hypertension (PH), but there is little information regarding the impact of PH on right ventricular (RV) systolic function and survival. We conducted a retrospective cohort study of our patients to explore this and other aspects of pulmonary hemodynamics with PVS. RV function was assessed using qualitative two-dimensional echocardiography. The ratio of systolic pulmonary artery (PA) and aortic pressures (PA:Ao) at cardiac catheterization reflected pulmonary hemodynamics. Reactivity testing employed inhaled nitric oxide + 100% fiO, or 100% fiO only; "reactivity" was a ≥ 20% decrease in PA:Ao. There were 105 PVS patients, although not all had data at every time point. (1) The mean PA:Ao at first cardiac catheterization (n = 77) was 0.79 ± 0.36; at last catheterization (n = 54), PA:Ao = 0.69 ± 0.30; 90% had systolic PAP > one-half systemic. Survival was shorter with PA:Ao > 0.5. (2) Differences in survival relative to RV dysfunction on the first echocardiogram were not significant, although they were using the last echocardiogram. (3) The magnitude of RV dysfunction was positively correlated with PA:Ao. (4) Balloon dilation of PV acutely decreased PA:Ao (-0.13 ± 0.37, P = 0.03 [n = 40 patients]). 5. Of 20 patients tested, 13 were acutely reactive to vasodilators. PH is a major feature of PVS. Reduced RV function and PA:Ao appear to be predictors of survival. Given the importance of PH in this disease, clinical studies of PVS treatments should include measures of PAP and RV function as important variables of interest.
肺静脉狭窄（PVS）与肺动脉高压（PH）相关，但关于PH对右心室（RV）收缩功能和生存的影响，相关信息较少。我们对患者进行了一项回顾性队列研究，以探讨PVS患者肺血流动力学的这一及其他方面。使用定性二维超声心动图评估RV功能。心导管检查时收缩期肺动脉（PA）与主动脉压力之比（PA:Ao）反映肺血流动力学。反应性测试采用吸入一氧化氮+100%吸氧浓度，或仅采用100%吸氧浓度；“反应性”定义为PA:Ao降低≥20%。共有105例PVS患者，不过并非所有患者在每个时间点均有数据。（1）首次心导管检查时（n = 77）的平均PA:Ao为0.79±0.36；末次导管检查时（n = 54），PA:Ao = 0.69±0.30；90%的患者收缩期肺动脉压>体循环压力的一半。PA:Ao>0.5时生存时间较短。（2）相对于首次超声心动图显示的RV功能障碍，生存差异不显著，不过采用末次超声心动图时差异显著。（3）RV功能障碍的程度与PA:Ao呈正相关。（4）PV球囊扩张术可使PA:Ao急性降低（-0.13±0.37，P = 0.03 [n = 40例患者]）。5. 在接受测试的20例患者中，13例对血管扩张剂有急性反应。PH是PVS的主要特征。RV功能降低和PA:Ao似乎是生存的预测指标。鉴于PH在这种疾病中的重要性，PVS治疗的临床研究应将肺动脉压和RV功能的测量作为重要的感兴趣变量纳入其中。
