Callahan Ryan, Gauvreau Kimberlee, Keochakian Mirjam, Esch Jesse J, Porras Diego, Bergersen Lisa, Beroukhim Rebecca, Farias Michael, Harrild David M, Ireland Christina M, Kwatra Neha, Jenkins Kathy J
Department of Pediatrics, Children's Hospital of Philadelphia, and University of Pennsylvania Perelman School of Medicine (R.C.).
Department of Cardiology (K.G., M.K., J.J.E., D.P., L.B., R.B., M.F., D.M.H., C.M.I., K.J.J.), Boston Children's Hospital and Harvard Medical School, MA.
Circ Cardiovasc Interv. 2025 Sep;18(9):e015002. doi: 10.1161/CIRCINTERVENTIONS.124.015002. Epub 2025 Jul 14.
Pediatric intraluminal pulmonary vein stenosis (PVS) occurs in a heterogeneous patient population but is unified by its cellular composition in affected pulmonary veins. Enhanced delineation of the hemodynamic and physiological vein consequences of PVS and its impact on vein lumen, vein pressure, wall structure, vein compliance, and wall shear stress has not been studied. The objective is to assess PVS characteristics using multimodal catheterization techniques (angiography, pulmonary vein pressure, intravascular ultrasound, balloon compliance testing, and wall shear stress calculation) and determine their association with vein outcomes.
The study is a prospective trial applying a standardized catheterization assessment to patients under 13 years of age who are undergoing cardiac catheterization for PVS. The primary outcome is vein status at 12 months, assigned by an end point committee. The secondary outcome is transplant-free survival.
Overall, 50 patients with a median age of 2.7 years (range, 0.3-11.8) underwent assessment and included angiography of 200 veins, 171 pressure assessment, 169 intravascular ultrasound, 58 balloon compliance testing, and 141 wall shear stress calculations. The probability of a worse vein outcome increased as angiographic severity score, percent wall thickness by intravascular ultrasound, postintervention gradient, or postintervention wall shear stress increased. Multivariable analysis demonstrated veins with angiographic upstream luminal narrowing (odds ratio, 3.26 [95% CI, 1.38-7.7]; =0.007), ostial diameter <6 mm (odds ratio, 3.93 [95% CI, 1.29-12]; =0.016), and ≥35% upstream wall thickness by intravascular ultrasound (odds ratio, 5.05 [95% CI, 2.03-12.5]; <0.001) are associated with a worse vein outcome. A 3-point Invasive Pulmonary Vein Score prediction tool was created. Transplant-free survival was 92% at 12 months.
A multimodal pulmonary vein catheterization assessment determined angiographic and intravascular ultrasound findings to be the most predictive of 12-month pulmonary vein outcome.
URL: https://www.clinicaltrials.gov; Unique identifier: NCT04696289.
小儿腔内肺静脉狭窄（PVS）发生于不同的患者群体，但受累肺静脉的细胞组成具有一致性。尚未对PVS的血流动力学和生理静脉后果及其对静脉腔、静脉压力、壁结构、静脉顺应性和壁剪切应力的影响进行更深入的研究。目的是使用多模态导管技术（血管造影、肺静脉压力、血管内超声、球囊顺应性测试和壁剪切应力计算）评估PVS特征，并确定它们与静脉转归的关联。
本研究是一项前瞻性试验，对因PVS接受心脏导管检查的13岁以下患者应用标准化导管评估。主要结局是12个月时的静脉状态，由终点委员会判定。次要结局是无移植生存。
总体而言，50例中位年龄为2.7岁（范围0.3 - 11.8岁）的患者接受了评估，包括对200条静脉进行血管造影、171次压力评估、169次血管内超声检查、58次球囊顺应性测试和141次壁剪切应力计算。随着血管造影严重程度评分、血管内超声测量的壁厚度百分比、干预后梯度或干预后壁剪切应力增加，静脉转归变差的可能性增加。多变量分析表明，血管造影显示上游管腔狭窄（比值比，3.26 [95% CI，1.38 - 7.7]；P = 0.007）、开口直径<6 mm（比值比，3.93 [95% CI，1.29 - 12]；P = 0.016）以及血管内超声显示上游壁厚度≥35%（比值比，5.05 [95% CI，2.03 - 12.5]；P < 0.001）与较差的静脉转归相关。创建了一个三分制有创肺静脉评分预测工具。12个月时无移植生存率为92%。
多模态肺静脉导管评估确定血管造影和血管内超声检查结果对12个月时肺静脉转归的预测性最强。
