Shorter survival in familial versus idiopathic pulmonary arterial hypertension is associated with hemodynamic markers of impaired right ventricular function
Shorter survival in familial versus idiopathic pulmonary arterial hypertension is associated with hemodynamic markers of impaired right ventricular function
复制标题
DOI：
10.1086/674326
复制
发表时间：
2013-09-01
影响因子：
2.6
通讯作者：
Hemnes, Anna R.
中科院分区：
文献类型：
--
作者：
Brittain, Evan L.;Pugh, Meredith E.;Hemnes, Anna R.
尽管与特发性肺动脉高压（IPAH）患者相比，家族性肺动脉高压（FPAH）患者的血流动力学更严重，但尚不清楚这是否会转化为生存差异。在这些组中，右心室（RV）功能对生存率的影响也是未知的。我们回顾了在范德比尔特肺动脉高压研究队列中登记的57例FPAH和66例IPAH患者的血流动力学数据和健康信息。比较两组患者诊断时的血流动力学，计算肺小动脉容量（PC）和右心室每搏作功指数（RVSWI）。使用生存分析，我们比较了FPAH和IPAH患者5年内无死亡或肺移植的复合终点。FPAH患者诊断后5年内死亡或移植的复合结局显著增加（对数秩P < 0：001）。与IPAH相比，FPAH的PC和RVSWI显著降低（P < 0：001）。在单变量分析中，PC（比值比[OR]：0.17 [95%置信区间（95% CI）：0.03-0.83]）和RVSWI（OR：0.86 [95%CI：0.77-0.95]）是死亡率的预测因子，心脏指数也是（OR：0.17 [95% CI：0.06-0.51]）和PVR（OR：1.1 [95% CI：1.01-1.12]）。在FPAH患者中，RVSWI在死亡或接受移植的患者中低于存活者（P = 0：006），而PC则不是（P = 0：5）。我们发现FPAH的无事件生存率显著低于IPAH，PC和RVSWI显著低于IPAH。在死亡或接受移植的FPAH患者中，RVSWI低于存活者，表明不成比例的RV功能障碍。
Although individuals with familial pulmonary arterial hypertension (FPAH) have more severe hemodynamics, compared to individuals with idiopathic PAH (IPAH), it is unclear whether this translates into a survival difference. The influence of right ventricular (RV) function on survival in these groups is also unknown. We reviewed hemodynamic data and health information from a prospective institutional database of 57 FPAH and 66 IPAH patients registered with the Vanderbilt Pulmonary Hypertension Research Cohort. We compared hemodynamics at the time of diagnosis between the two groups and calculated pulmonary arteriolar capacitance (PC) and RV stroke work index (RVSWI). Using survival analysis, we compared freedom from a 5-year composite of death or lung transplantation in FPAH and IPAH patients. The composite outcome of death or transplant at 5 years from diagnosis was significantly increased in FPAH (log rank P < 0: 001). PC and RVSWI were significantly decreased in FPAH, compared to IPAH (P < 0: 001 for both). In univariate analysis, PC (odds ratio [OR]: 0.17 [95% confidence interval (95% CI): 0.03-0.83]) and RVSWI (OR: 0.86 [95% CI: 0.77-0.95]) were predictors of mortality, as were cardiac index (OR: 0.17 [95% CI: 0.06-0.51]) and PVR (OR: 1.1 [95% CI: 1.01-1.12]). Among FPAH patients, RVSWI was lower in those who died or received a transplant than in survivors (P = 0: 006), while PC was not (P = 0: 5). We found significantly worse event-free survival and significantly lower PC and RVSWI in FPAH than in IPAH. In FPAH patients who died or underwent transplantation, RVSWI was lower than that in survivors, suggesting disproportionate RV dysfunction.