Ittleman Benjamin, Lowenstein Sarah, Edwards Lindsay A, Caris Elizabeth, Bhat Aarti, Conwell Jeffrey, Lewin Mark, Arya Bhawna
Arkansas Children's Hospital, University of Arkansas Medical Sciences, Little Rock, AR, USA.
Metropolitan Pediatrics, Portland, OR, USA.
Pediatr Cardiol. 2025 Mar;46(3):553-561. doi: 10.1007/s00246-024-03453-5. Epub 2024 Apr 22.
Despite significant advancements in the care of patients with hypoplastic left heart syndrome (HLHS) morbidity and mortality remain high. Postnatal right ventricular dysfunction and tricuspid regurgitation (TR) are associated with worse outcomes in HLHS. We aim to determine if right ventricle functional parameters and TR on fetal echocardiogram are associated with postnatal outcomes in HLHS patients. Retrospective review was performed on all fetuses with HLHS from 2014 to 2022 at our institution. Initial and follow up fetal echocardiogram measurements of right ventricular myocardial performance index (MPI), fractional area change (FAC) and global longitudinal strain (GLS) were retrospectively measured. The presence and severity of TR was recorded from the fetal echocardiogram reports. Postnatal outcomes including transplant-free survival, hospital length of stay > 30 days after initial palliation and need for bidirectional Glenn at < 4 months were reviewed. Forty-three subjects met inclusion criteria. Mean gestational age at presentation was 26.1 ± 5.9 weeks. Nine subjects died and 3 required heart transplantation. Initial fetal echocardiogram MPI was significantly lower (better) (0.36 ± 0.06 vs 0.44 ± 0.11; p = < 0.001) and FAC was significantly higher (better) (45 ± 6% vs 40 ± 8%; p = 0.035) in transplant-free survivors. Fetal right ventricular GLS and presence of mild TR were not associated with postnatal outcome. In fetuses with HLHS, abnormal MPI and right ventricular FAC are associated with decreased transplant-free survival. There was no observed association between GLS and postnatal outcomes. To our knowledge this is the first study examining fetal right ventricular function and GLS in HLHS patients and its link to postnatal outcomes.
尽管在左心发育不全综合征（HLHS）患者的治疗方面取得了重大进展，但发病率和死亡率仍然很高。产后右心室功能障碍和三尖瓣反流（TR）与HLHS患者的不良预后相关。我们旨在确定胎儿超声心动图上的右心室功能参数和TR是否与HLHS患者的产后结局相关。对2014年至2022年在我们机构诊断为HLHS的所有胎儿进行了回顾性研究。回顾性测量了右心室心肌性能指数（MPI）、面积变化分数（FAC）和整体纵向应变（GLS）的初始和随访胎儿超声心动图测量值。从胎儿超声心动图报告中记录TR的存在和严重程度。回顾了产后结局，包括无移植生存率、初次姑息治疗后住院时间>30天以及在<4个月时需要进行双向格林分流术。43名受试者符合纳入标准。就诊时的平均孕周为26.1±5.9周。9名受试者死亡，3名需要心脏移植。在无移植生存者中，初始胎儿超声心动图MPI显著更低（更好）（0.36±0.06对0.44±0.11；p= <0.001），FAC显著更高（更好）（45±6%对40±8%；p=0.035）。胎儿右心室GLS和轻度TR的存在与产后结局无关。在HLHS胎儿中，异常的MPI和右心室FAC与无移植生存率降低相关。未观察到GLS与产后结局之间的关联。据我们所知，这是第一项研究HLHS患者胎儿右心室功能和GLS及其与产后结局关系的研究。
