背景:本研究的目的是描述未修复的部分肺静脉回流异常和完整房间隔(PAPVR-IAS)成人的相关性和结局。
结果:我们确定了在梅奥诊所接受治疗的PAPVR-IAS成人,而未修复PAPVR-IAS的患者则为参考组。临床指标(纽约心脏协会类别,峰值耗氧量,和NT-proBNP[N末端B型利钠肽前体])和回声衍生的右心指数(右心房[RA]容积,RA储层应变,右心室[RV]游离壁应变,右心室舒张末期面积,和RV收缩压)在基线以及3年和5年随访时进行评估。有80例患者和38例未修复的PAPVR-IAS患者,分别。手术修复的临床预测因素是异常静脉的数量,RA音量,和右心室舒张末期面积。PAPVR-IAS风险评分,从这些临床预测因子中得出,与手术修复相关(调整后的比值比,风险评分每增加1.37[95%CI,1.24-1.65];曲线下面积,0.742).在3年(n=73)和5年随访(n=36)的未修复PAPVR-IAS患者中,临床指标没有时间变化(纽约心脏协会类别,预测峰值耗氧量,和NT-proBNP)和右心指数(RA体积指数,RA储层应变,右心室舒张末期面积指数,RV自由壁应变,和RV收缩压)。
结论:PAPVR-IAS风险评分可用于评估需要手术修复的几率。此外,在PAPVR-IAS未修复的成人患者随访期间,临床和右心指数无暂时性恶化.
BACKGROUND: The purpose of this study was to describe the correlates and outcomes in adults with unrepaired partial anomalous pulmonary venous return and intact atrial septum (PAPVR-IAS).
RESULTS: We identified adults with PAPVR-IAS who received care at the Mayo Clinic, while those with unrepaired PAPVR-IAS comprised the reference group. Clinical indices (New York Heart Association class, peak oxygen consumption, and NT-proBNP [N-terminal pro-B-type natriuretic peptide]) and echo-derived right heart indices (right atrial [RA] volume, RA reservoir strain, right ventricular [RV] free wall strain, RV end-diastolic area, and RV systolic pressure) were assessed at baseline and 3-year and 5-year follow-up. There were 80 patients and 38 patients with unrepaired versus repaired PAPVR-IAS, respectively. The clinical predictors of surgical repair were the number of anomalous veins, RA volume, and RV end-diastolic area. The PAPVR-IAS risk score, derived from these clinical predictors, was associated with surgical repair (adjusted odds ratio, 1.37 [95% CI, 1.24-1.65] per unit increase in risk score; area under the curve, 0.742). Among patients with unrepaired PAPVR-IAS with 3-year (n=73) and 5-year follow-up (n=36), there was no temporal change in clinical indices (New York Heart Association class, predicted peak oxygen consumption, and NT-proBNP) and right heart indices (RA volume index, RA reservoir strain, RV end-diastolic area index, RV free wall strain, and RV systolic pressure).
CONCLUSIONS: The PAPVR-IAS risk score can be used to assess the odds of requiring surgical repair. Furthermore, there was no temporal deterioration in clinical and right heart indices during follow-up in adults with unrepaired PAPVR-IAS.