Fetal & Neonatal Cardiology Program, Division of Cardiology, Department of Pediatrics, Stollery Children's Hospital, Edmonton, Alberta, Canada.
Department of Pediatrics, Section of Cardiology, Alberta Children's Hospital, University of Calgary, Calgary, Alberta, Canada.
Ultrasound Obstet Gynecol. 2021 Sep;58(3):405-410. doi: 10.1002/uog.23558.
To determine which echocardiographic features of hypoplastic left heart complex (HLHC) in the fetal period are predictive of biventricular (BV) circulation and to evaluate the long-term outcome of patients with HLHC, including rates of mortality, reintervention and development of further cardiac disease.
Echocardiograms of fetuses with HLHC obtained at 18-26 weeks and 27-36 weeks' gestation between 2004 and 2017 were included in the analysis. The primary outcome was successful BV circulation (Group 1). Group 2 included patients with single-ventricle palliation, death or transplant. Univariate analysis was performed on data obtained at 18-26 and 27-36 weeks and multivariate logistic regression was performed on data obtained at 27-36 weeks only.
Of the 51 included cases, 44 achieved successful BV circulation (Group 1) and seven did not (Group 2). Right-to-left/bidirectional foramen ovale (FO) flow and a higher mitral valve (MV) annulus Z-score were associated with successful BV circulation on both univariate and multivariate analysis. Bidirectional or left-to-right FO flow, left ventricular length (LVL) Z-score of < -2.4 and a MV Z-score of < -4.5 correctly predicted 80% of Group 2 cases. Late follow-up was available for 41 patients. There were two late deaths in Group 2. Thirteen patients in Group 1 required reintervention, 12 developed mitral stenosis and five developed isolated subaortic stenosis.
BV circulation is common in fetuses with HLHC. Higher MV annulus and LVL Z-scores and right to left direction of FO flow are important predictors of BV circulation. Long-term sequelae in those with BV circulation may include mitral and subaortic stenosis. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.
确定胎儿时期左心发育不全综合征（HLHC）的哪些超声心动图特征可预测双心室（BV）循环，并评估 HLHC 患者的长期预后，包括死亡率、再次干预和进一步心脏疾病的发展。
纳入 2004 年至 2017 年期间在 18-26 周和 27-36 周妊娠时获得的 HLHC 胎儿的超声心动图。主要结局是成功的 BV 循环（第 1 组）。第 2 组包括单心室姑息治疗、死亡或移植的患者。对 18-26 周和 27-36 周获得的数据进行单因素分析，对仅在 27-36 周获得的数据进行多因素逻辑回归分析。
在 51 例纳入病例中，44 例成功实现 BV 循环（第 1 组），7 例未实现（第 2 组）。右向左/双向卵圆孔（FO）血流和较高的二尖瓣（MV）瓣环 Z 评分在单因素和多因素分析中均与成功的 BV 循环相关。双向或左向右 FO 血流、左心室长度（LVL）Z 评分＜-2.4 和 MV Z 评分＜-4.5 正确预测了第 2 组病例的 80%。41 例患者可获得晚期随访。第 2 组有 2 例晚期死亡。第 1 组中有 13 例需要再次干预，12 例发生二尖瓣狭窄，5 例发生孤立性主动脉瓣下狭窄。
在 HLHC 胎儿中，BV 循环较为常见。较高的 MV 瓣环和 LVL Z 评分以及 FO 血流的右向左方向是 BV 循环的重要预测指标。具有 BV 循环的患者的长期后遗症可能包括二尖瓣和主动脉瓣下狭窄。
