Steele Jeremy M, Komarlu Rukmini, Worley Sarah, Alsaied Tarek, Statile Christopher, Erenberg Francine G
The Heart Institute, Cincinnati Children's Hospital, Cincinnati, Ohio.
Department of Pediatric Cardiology, Cleveland Clinic Children's, Cleveland, Ohio.
Congenit Heart Dis. 2019 Nov;14(6):1193-1198. doi: 10.1111/chd.12829. Epub 2019 Sep 6.
Deciding on a surgical pathway for neonates with ≥2 left heart obstructive lesions is complex. Predictors of the successful biventricular (2V) repair in these patients are poorly defined. The goal of our study was to identify patients who underwent the 2V repair and assess anatomic and echocardiographic predictors of success.
Infants born between July 2015 and August 2017 with ≥2 left heart obstructive lesions with no prior interventions were identified (n = 19). Patients with aortic or mitral valve (MV) atresia and critical aortic stenosis were excluded. Initial echocardiograms were reviewed for aortic, MV, tricuspid valve annulus size, and left (LV) and right (RV) ventricle diastolic longitudinal dimensions. The valve morphology and presence of a ventricular septal defect (VSD) and coarctation were assessed. Clinical outcomes included successful 2V repair, complications, and repeat interventions or surgeries. Failed 2V repair was defined as a takedown to single ventricle (1V) physiology, cardiac transplantation, or death.
For 2V repair, 14/19 patients were selected and for 1V, 5/19 patients were selected. Initial surgical procedures of the 2V group were simple coarctation repair (5), complex coarctation/arch reconstruction +/- septal defect closure (6), hybrid stage 1 (2), and none (1). Three of the 2V patients required reintervention in the first 90 days. The LV to RV diastolic longitudinal ratio >0.75 and mitral/tricuspid ratio of <0.8 were observed in 13/14 of the 2V patients. The LV:RV ratio and the aortic valve z score were significantly larger in the 2V group compared to the 1V group. All patients in the 1V group had a nonapex forming LV. There was no mortality with follow-up to three years of age.
This study showed excellent short-term and midterm surgical results in the 2V population. The LV:RV diastolic longitudinal ratio may be a useful tool in the risk stratification of a successful 2V repair even in cases with a small MV.
为患有≥2处左心梗阻性病变的新生儿确定手术路径很复杂。这些患者双心室（2V）修复成功的预测因素尚不明确。我们研究的目的是确定接受2V修复的患者，并评估成功的解剖学和超声心动图预测因素。
确定2015年7月至2017年8月出生、患有≥2处左心梗阻性病变且未接受过先前干预的婴儿（n = 19）。排除患有主动脉或二尖瓣闭锁以及严重主动脉狭窄的患者。回顾初始超声心动图，以评估主动脉、二尖瓣、三尖瓣环大小以及左心室（LV）和右心室（RV）舒张期纵向维度。评估瓣膜形态、室间隔缺损（VSD）的存在情况以及缩窄情况。临床结局包括2V修复成功、并发症以及再次干预或手术。2V修复失败定义为转为单心室（1V）生理状态、心脏移植或死亡。
对于2V修复，选择了14/19例患者；对于1V修复，选择了5/19例患者。2V组的初始手术操作包括单纯缩窄修复（5例）、复杂缩窄/主动脉弓重建+/-室间隔缺损闭合（6例）、一期杂交手术（2例）以及无手术操作（1例）。2V组中有3例患者在术后90天内需再次干预。14例2V患者中有13例观察到左心室与右心室舒张期纵向比率>0.75且二尖瓣/三尖瓣比率<0.8。与1V组相比，2V组的左心室：右心室比率和主动脉瓣z值显著更大。1V组的所有患者左心室均未形成心尖。随访至3岁时无死亡病例。
本研究显示2V人群的短期和中期手术结果良好。即使在二尖瓣较小的病例中，左心室：右心室舒张期纵向比率也可能是成功进行2V修复风险分层的有用工具。
