Stone Matthew L, Schäfer Michal, DiMaria Michael V, von Alvensleben Johannes C, Campbell David N, Jaggers James, Mitchell Max B
Division of Pediatric Cardiothoracic Surgery, Department of Surgery, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, Colo.
Division of Pediatric Cardiology, Department of Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, Colo.
J Thorac Cardiovasc Surg. 2022 Mar;163(3):1195-1207. doi: 10.1016/j.jtcvs.2021.06.064. Epub 2021 Jul 17.
This study used cardiac magnetic resonance imaging to evaluate flow characteristics and ventricular hemodynamics for children with single right (hypoplastic left heart syndrome) and single left (hypoplastic right heart syndrome) systemic ventricle anatomy after Fontan palliation compared with normal biventricular controls.
Twenty children with single ventricle anatomy (hypoplastic left heart syndrome, n = 10; hypoplastic right heart syndrome, n = 10) underwent standardized 4-dimensional flow cardiac magnetic resonance and were compared with age-matched controls (n = 10). End-diastolic volume was partitioned into 4 defined components of variable kinetic energy (direct flow, retained inflow, delayed ejection, and residual volume) and compared between groups. Further, volumetric and functional parameters as defined by cardiac magnetic resonance were evaluated.
Children with hypoplastic left heart syndrome had significantly increased indexed end-diastolic and end-systolic volumes compared with both hypoplastic right heart syndrome and control groups. Flow component analysis demonstrated diastolic inefficiency in both hypoplastic left heart syndrome and hypoplastic right heart syndrome groups compared with controls as defined by decreased direct flow and increased residual volumes. Decreased direct flow correlated with decreased ejection fraction and increased end-diastolic and end-systolic volume indices. Increased residual volume correlated with decreased ejection fraction and increased end-systolic volume index.
Fontan-palliated patients with single ventricle physiology (hypoplastic left heart syndrome and hypoplastic right heart syndrome) demonstrate altered and inefficient flow patterns in the systemic ventricle as defined by 4-dimensional flow cardiac magnetic resonance compared with normal biventricular controls. Decreased direct flow and increased residual volume indicate that diastolic ventricular dysfunction is prevalent after Fontan palliation. This study provides a foundation for future predictive modeling and cardiac magnetic resonance flow diagnostic studies in this high-risk patient population.
本研究采用心脏磁共振成像技术，评估在Fontan姑息治疗后，右心单心室（左心发育不全综合征）和左心单心室（右心发育不全综合征）患儿的血流特征和心室血流动力学，并与正常双心室对照进行比较。
20名单心室解剖结构的患儿（左心发育不全综合征，n = 10；右心发育不全综合征，n = 10）接受标准化的四维血流心脏磁共振检查，并与年龄匹配的对照组（n = 10）进行比较。舒张末期容积被划分为4个具有可变动能的定义成分（直接血流、保留流入、延迟射血和残余容积），并在组间进行比较。此外，还评估了心脏磁共振定义的容积和功能参数。
与右心发育不全综合征组和对照组相比，左心发育不全综合征患儿的指数化舒张末期和收缩末期容积显著增加。血流成分分析表明，与对照组相比，左心发育不全综合征和右心发育不全综合征组的舒张功能均存在低效，表现为直接血流减少和残余容积增加。直接血流减少与射血分数降低以及舒张末期和收缩末期容积指数增加相关。残余容积增加与射血分数降低和收缩末期容积指数增加相关。
与正常双心室对照相比，Fontan姑息治疗的单心室生理患儿（左心发育不全综合征和右心发育不全综合征）通过四维血流心脏磁共振显示，体循环心室的血流模式发生改变且低效。直接血流减少和残余容积增加表明Fontan姑息治疗后舒张期心室功能障碍普遍存在。本研究为这一高危患者群体未来的预测模型和心脏磁共振血流诊断研究奠定了基础。
