The Labatt Family Heart Center, Division of Cardiology, Hospital for Sick Children and University of Toronto, 555 University Avenue, Toronto, ON, Canada.
Am Heart J. 2013 Jun;165(6):1024-31. doi: 10.1016/j.ahj.2013.02.027. Epub 2013 Apr 6.
Echocardiographic measures of right ventricular (RV) function are associated with adverse outcomes in adults with idiopathic pulmonary arterial hypertension (iPAH) but have not been adequately studied in children. We investigated the prognostic value of 2D, M-mode and Doppler indices of RV function in relation to death or lung transplant in children with iPAH and PAH associated with congenital heart diseases (cPAH).
Children with iPAH and cPAH were studied. Two echocardiograms were analyzed for each patient: at diagnosis and at last follow-up. Clinical data, catheter hemodynamics and 6-minute walk distance were recorded. Echo indices of RV function were compared between the first and follow-up echo, between iPAH and cPAH patients, and between iPAH patients alive at follow-up versus those who had died or had undergone lung transplant. Survival probability stratified by RV function was analyzed.
Fifty-four children were studied: 36 cPAH patients (7.5 ± 5.9 years) and 18 iPAH patients (8.9 ± 5.7 years) of whom 12 were alive and 6 had died or were transplanted. Despite similar pulmonary hemodynamics, RV function, including right atrial volume, tricuspid annular planar excursion, fractional area of change, and left ventricular eccentricity index were worse in iPAH at presentation and at follow-up. At last echo there was further worsening of RV function in iPAH patients, particularly in those who had died or were transplanted, compared with improved or unchanged indices in cPAH patients or iPAH survivors.
Conventional echo RV functional parameters are valuable to identify risk for transplant or death in children with PAH.
超声心动图测量右心室（RV）功能与特发性肺动脉高压（iPAH）成人的不良结局相关，但在儿童中尚未得到充分研究。我们研究了二维、M 模式和 RV 功能多普勒指数与 iPAH 儿童和与先天性心脏病相关的肺动脉高压（cPAH）儿童的死亡或肺移植相关的预后价值。
研究了 iPAH 和 cPAH 的儿童患者。对每位患者进行了两次超声心动图分析：诊断时和最后一次随访时。记录临床数据、导管血液动力学和 6 分钟步行距离。比较了第一次和随访超声心动图、iPAH 和 cPAH 患者、随访时存活的 iPAH 患者与死亡或接受肺移植的 iPAH 患者之间的 RV 功能超声心动图指数，并分析 RV 功能分层的生存概率。
研究了 54 名儿童：36 名 cPAH 患者（7.5 ± 5.9 岁）和 18 名 iPAH 患者（8.9 ± 5.7 岁），其中 12 名存活，6 名死亡或移植。尽管肺血流动力学相似，但 RV 功能包括右心房容积、三尖瓣环平面位移、面积变化分数和左心室偏心指数在 iPAH 患者的表现和随访时均较差。在最后一次超声心动图中，与 cPAH 患者或 iPAH 存活者的改善或不变指数相比，iPAH 患者的 RV 功能进一步恶化，特别是那些死亡或移植的患者。
常规超声 RV 功能参数可用于识别 PAH 儿童移植或死亡的风险。
