The Heart Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
Department of Cardiology, Boston Children's Hospital, Boston, MA, USA; Department of Pediatrics, Harvard Medical School, Boston, MA, USA.
J Cardiovasc Magn Reson. 2024 Summer;26(1):101029. doi: 10.1016/j.jocmr.2024.101029. Epub 2024 Feb 23.
Identification of risk factors for biventricular (BiV) repair in children with hypoplastic left ventricles (HLV) has been challenging. We sought to identify preoperative cardiovascular magnetic resonance (CMR) predictors of outcome in patients with HLVs who underwent BiV repair, with a focus on the mitral valve (MV).
Single-center retrospective analysis of preoperative CMRs on patients with HLV (≤50 mL/m) and no endocardial fibroelastosis who underwent BiV repair from 2005-2022. CMR measurements included MV orifice area in diastole. The primary composite outcome included time to death, transplant, BiV takedown, heart failure admission, left atrial decompression, or unexpected reoperation; and the secondary outcome included more than or equal to moderate mitral stenosis and/or regurgitation.
Median follow-up was 0.7 (interquartile range 0.1, 2.2) years. Of 122 patients [59 atrioventricular canal (AVC) and 63 non-AVC] age 3 ± 2.8 years at the time of BiV repair, freedom from the primary outcome at 2 years was 53% for AVC and 69% for non-AVC (log rank p = 0.12), and freedom from the secondary outcome at 2 years was 49% for AVC and 79% for non-AVC (log rank p < 0.01). Independent predictors of primary outcome for AVC patients included MV orifice area z-score <-2 and transitional AVC; for non-AVC patients, predictors included MV orifice area z-score <-2, abnormal MV anatomy, and conal-septal ventricular septal defect. Independent predictors of secondary outcome for AVC patients included older age at surgery, transitional AVC, and transposition of the great arteries.
In children with HLV, low MV orifice area and pre-existing MV pathology are risk factors for adverse outcome after BiV repair.
识别左心室发育不全（HLV）患儿双心室（BiV）修复的风险因素一直具有挑战性。我们试图确定接受 BiV 修复的 HLV 患者术前心血管磁共振（CMR）的预测因素，重点关注二尖瓣（MV）。
对 2005 年至 2022 年期间接受 BiV 修复且无心内膜纤维弹性组织增生的 HLV（≤50 mL/m）且无心内膜纤维弹性组织增生的患者进行单中心回顾性分析。CMR 测量包括 MV 瓣口在舒张期的面积。主要复合结局包括死亡、移植、BiV 切除、心力衰竭入院、左心房减压或意外再次手术的时间；次要结局包括中重度二尖瓣狭窄和/或反流。
中位随访时间为 0.7（四分位距 0.1，2.2）年。在 122 例患者中[59 例房室管（AVC）和 63 例非 AVC]，BiV 修复时年龄为 3±2.8 岁，2 年时 AVC 的主要结局无事件生存率为 53%，而非 AVC 为 69%（对数秩检验 p=0.12），2 年时次要结局无事件生存率为 AVC 为 49%，而非 AVC 为 79%（对数秩检验 p<0.01）。AVC 患者主要结局的独立预测因素包括 MV 瓣口面积 z 评分<-2 和过渡型 AVC；非 AVC 患者的预测因素包括 MV 瓣口面积 z 评分<-2、MV 解剖异常和圆锥隔室间隔缺损。AVC 患者次要结局的独立预测因素包括手术时年龄较大、过渡型 AVC 和大动脉转位。
在左心室发育不全的患儿中，MV 瓣口面积低和预先存在的 MV 病变是 BiV 修复后不良结局的危险因素。
