Division of Cardiovascular Surgery, The Hospital for Sick Children, Toronto, CA .
Division of Pediatric Cardiology, Radboud University Medical Center, Nijmegan, the Netherlands.
Comput Methods Programs Biomed. 2018 Oct;165:107-116. doi: 10.1016/j.cmpb.2018.08.014. Epub 2018 Aug 20.
Qualitative features of aortic and mitral valvar pathology have traditionally been used to classify congenital cardiac anomalies for which the left heart structures are unable to sustain adequate systemic cardiac output. We aimed to determine if novel groups of patients with greater clinical relevance could be defined within this population of patients with critical left heart obstruction (CLHO) using a data-driven approach based on both qualitative and quantitative echocardiographic measures.
An independent standardized review of recordings from pre-intervention transthoracic echocardiograms for 651 neonates with CLHO was performed. An unsupervised cluster analysis, incorporating 136 echocardiographic measures, was used to group patients with similar characteristics. Key measures differentiating the groups were then identified.
Based on all measures, cluster analysis linked the 651 neonates into groups of 215 (Group 1), 338 (Group 2), and 98 (Group 3) patients. Aortic valve atresia and left ventricular (LV) end diastolic volume were identified as significant variables differentiating the groups. The median LV end diastolic area was 1.35, 0.69, and 2.47 cm in Groups 1, 2, and 3, respectively (p < 0.0001). Aortic atresia was present in 11% (24/215), 87% (294/338), and 8% (8/98), in Groups 1, 2, and 3, respectively (p < 0.0001). Balloon aortic valvotomy was the first intervention for 9% (19/215), 2% (6/338), and 61% (60/98), respectively (p < 0.0001). For those with an initial operation, single ventricle palliation was performed in 90% (176/215), 98% (326/338), and 58% (22/38) (p < 0.0001). Overall mortality in each group was 27% (59/215), 41% (138/338), and 12% (12/98) (p < 0.0001).
Using a data-driven approach, we conceptualized three distinct patient groups, primarily based quantitatively on baseline LV size and qualitatively by the presence of aortic valve atresia. Management strategy and overall mortality differed significantly by group. These groups roughly correspond anatomically and are analogous to multi-level LV hypoplasia, hypoplastic left heart syndrome, and critical aortic stenosis, respectively. Our analysis suggests that quantitative and qualitative assessment of left heart structures, particularly LV size and type of aortic valve pathology, may yield conceptually more internally consistent groups than a simplistic scheme limited to valvar pathology alone.
传统上，主动脉瓣和二尖瓣瓣病变的定性特征被用于对左心结构无法维持足够全身心输出量的先天性心脏畸形进行分类。我们旨在通过基于定性和定量超声心动图测量的无监督聚类分析，确定在这些具有严重左心梗阻 (CLHO) 的患者中是否可以定义更具临床相关性的新患者群体。
对 651 例 CLHO 新生儿的经胸超声心动图术前记录进行独立的标准化审查。采用包含 136 项超声心动图测量的非监督聚类分析对患者进行分组，以分组具有相似特征的患者。然后确定区分组的关键措施。
基于所有测量值，聚类分析将 651 名新生儿分为 215 组（第 1 组）、338 组（第 2 组）和 98 组（第 3 组）。主动脉瓣闭锁和左心室（LV）舒张末期容积被确定为区分组的重要变量。第 1、2 和 3 组的 LV 舒张末期面积中位数分别为 1.35、0.69 和 2.47 cm（p < 0.0001）。主动脉瓣闭锁分别存在于第 1、2 和 3 组的 11%（24/215）、87%（294/338）和 8%（8/98）的患者中（p < 0.0001）。球囊主动脉瓣切开术分别是第 1、2 和 3 组的 9%（19/215）、2%（6/338）和 61%（60/98）的首次干预措施（p < 0.0001）。对于那些有初始手术的患者，单心室姑息治疗分别在第 1、2 和 3 组中的 90%（176/215）、98%（326/338）和 58%（22/38）中进行（p < 0.0001）。每组的总死亡率分别为 27%（59/215）、41%（138/338）和 12%（12/98）（p < 0.0001）。
我们使用数据驱动的方法，主要基于基线 LV 大小进行定量分析，主要基于主动脉瓣闭锁的存在进行定性分析，将患者分为三个不同的患者群体。管理策略和总体死亡率因组而异。这些组大致对应解剖结构，分别类似于多水平 LV 发育不良、左心发育不全综合征和严重主动脉瓣狭窄。我们的分析表明，对左心结构的定量和定性评估，特别是 LV 大小和主动脉瓣病变类型，可能比仅基于瓣膜病变的简单方案产生更具内在一致性的组。
