背景:围产期过渡的特征是心脏负荷的急性变化。与正常新生儿联合心输出量(CCO)相比,患有左心发育不良综合征(HLHS)的新生儿的单个右心室(RV)输出明显更大。我们试图研究在HLHS中促进从胎儿晚期到新生儿早期的围产期过渡的心脏适应机制。
方法:前瞻性招募胎儿HLHS并发妊娠(n=35)和健康对照(Ctrl,n=17)在妊娠晚期(38±1周)以及出生后6、24和48小时进行了连续超声心动图检查。使用常规方法评估心功能,组织多普勒和斑点追踪超声心动图。
结果:足月HLHS胎儿的RV输出(RVCO)与CtrlCCO相当,通过更高的每搏输出量(SV)。与Ctrls的左心室(LV)和RV指数相比,他们表现出球状和扩张的RV,相对壁厚(RWT)减小[RWT:0.40±0.08与0.49±0.10,p<0.01],增加的Tei指数\'[HLHS与CtrlLV/CtrlRV:球形指数(SI):0.9±0.25vs.0.5±0.10/0.6±0.11,右心室面积指数:28±6cm2/m2vs.15±3cm2/m2/17±5cm2/m2,Tei指数:0.65±0.11vs.0.43±0.07/0.45±0.09,所有p<0.0001]。与CtrlCCO相比,HLHS新生儿通过更高的心率和SV产生升高的RVCO,随着RV的进一步扩张,48h时纵向收缩应变增加[-17±4%vs.-14±3%/-14±5%],周向和旋转心肌变形减少,舒张功能改变。HLHS新生儿也表现出右心房(RA)增大,纵向应变增加:6h(33±12%vs.26±6%),24h(37±15%vs.26±13%),48h(38±11%vs.24±13%),p<0.0001。
结论:足月HLHS胎儿表现出改变的RV几何结构和RV收缩和舒张功能参数。出生后,这些心脏参数的进一步改变可能反映了心输出量和肺动脉流量需求增加对RV负荷急剧改变的适应.
BACKGROUND: The perinatal transition is characterized by acute changes in cardiac loading. Compared to normal newborn combined cardiac output (CCO), single right ventricular (RV) output of neonates with hypoplastic left heart syndrome (HLHS) is markedly greater. We sought to examine the mechanisms of cardiac adaptation which facilitate this perinatal transition from late fetal to early neonatal life in HLHS.
METHODS: Prospectively recruited pregnancies complicated by fetal HLHS (n=35) and health controls (Ctrl, n=17) underwent serial echocardiography in late gestation (38±1weeks) and 6, 24 and 48 hours after birth. Cardiac function was assessed using conventional, tissue Doppler and speckle tracking echocardiography.
RESULTS: Term HLHS fetuses had an RV output (RVCO) comparable to Ctrl CCO via higher stroke volume (SV). Compared to both left ventricular (LV) and RV indices of Ctrls, they exhibited a globular and dilated RV with reduced relative wall thickness (RWT) [RWT: 0.40±0.08 vs. 0.49±0.10, p<0.01], increased Tei index\' [HLHS vs. Ctrl LV/Ctrl RV: sphericity index (SI): 0.9±0.25 vs. 0.5±0.10/0.6±0.11, RV area index: 28±6cm2/m2 vs. 15±3cm2/m2/17±5cm2/m2, Tei index\': 0.65±0.11 vs. 0.43±0.07/0.45±0.09, all p<0.0001]. HLHS neonates generated elevated RVCO compared to Ctrl CCO via higher heart rate and SV, with further RV dilatation, increased longitudinal systolic strain at 48h [-17±4% vs. -14±3%/-14±5%] with reduced circumferential and rotational myocardial deformation and altered diastolic function. HLHS neonates also demonstrated right atrial (RA) enlargement with increased longitudinal strain: 6h (33±12% vs. 26±6%), 24h (37±15% vs. 26±13%), 48h (38±11% vs. 24±13%), p<0.0001.
CONCLUSIONS: Term HLHS fetuses exhibit altered RV geometry and RV systolic and diastolic functional parameters. After birth, further alterations in these cardiac parameters likely reflect adaptation to acutely altered RV loading from increasing cardiac output and pulmonary artery flow demands.