Barnet Isabel R, Schulz Noah E, Ghelani Sunil J, Hoganson David M, Feins Eric N, Hammer Peter E, Emani Sitaram M, Sleeper Lynn A, Beroukhim Rebecca S
Harvard Medical School, Boston, Massachusetts, USA.
Harvard Medical School, Boston, Massachusetts, USA; Department of Cardiovascular Surgery, Boston Children's Hospital, Boston, Massachusetts, USA.
J Cardiovasc Magn Reson. 2024 Dec 6;27(1):101131. doi: 10.1016/j.jocmr.2024.101131.
Patients with hypoplastic left ventricles (LV) who undergo volume-loading procedures (recruitment, biventricular [BIV] repair) are at risk for adverse outcomes, including heart failure and death. We investigated pre-BIV LV shape as a predictor of outcome after BIV repair in patients with hypoplastic LVs.
Baseline and post-recruitment cardiac magnetic resonance imaging and computed tomography data were analyzed in patients with hypoplastic LV (<50 mL/m). Statistical shape modeling (SSM) was utilized to generate a model of the shape and variability of LVs. Traditional measures of LV sphericity and eccentricity were also measured. Major adverse cardiovascular events (MACE) included heart failure, transplant, and death.
Of 95 patients with baseline mean LV volume 29 ± 13 mL/m, 45/95 (47%) had a right dominant atrioventricular canal defect, 31/95 (33%) had a variant of hypoplastic left heart syndrome, and 18/95 (19%) had endocardial fibroelastosis (EFE). A wide variation in LV shape was found by SSM, and shape modes were associated with right ventricle (RV) and LV size, and diagnosis. BIV repair was achieved in 74/95 (78%) patients; 13/74 (18%) of BIV patients had MACE. Predictors of MACE following BIV repair included EFE, higher RV mass index, and higher RV end-diastolic volume index. No baseline or post-recruitment LV shape parameter was associated with the outcome after BIV repair.
The shape model of hypoplastic LVs demonstrated a wide array of LV shapes. LVs gained sphericity and size and lost eccentricity with recruitment. Though the ventricles changed shape with recruitment, no specific LV shape characteristic at the baseline or post-recruitment stage was predictive of decision to proceed with BIV repair or outcome. Higher RV mass and volume may represent new biomarkers that predict outcomes following BIV repair in patients with hypoplastic LV. Further investigation could determine the reproducibility of these findings.
接受容量负荷手术（扩容、双心室修复）的左心室发育不全（LV）患者面临不良结局的风险，包括心力衰竭和死亡。我们研究了左心室发育不全患者在双心室修复术前的左心室形状作为双心室修复术后结局的预测指标。
对左心室发育不全（<50 mL/m）患者的基线和扩容后的心脏磁共振成像及计算机断层扫描数据进行分析。采用统计形状建模（SSM）生成左心室形状和变异性模型。还测量了左心室球形度和偏心率的传统指标。主要不良心血管事件（MACE）包括心力衰竭、移植和死亡。
95例基线平均左心室容积为29±13 mL/m的患者中，45/95（47%）患有右优势型房室通道缺损，31/95（33%）患有左心发育不全综合征变异型，18/95（19%）患有心内膜弹力纤维增生症（EFE）。通过SSM发现左心室形状存在广泛差异，形状模式与右心室（RV）和左心室大小以及诊断相关。74/95（78%）的患者完成了双心室修复；双心室修复患者中有13/74（18%）发生了MACE。双心室修复后MACE的预测因素包括EFE、较高的右心室质量指数和较高的右心室舒张末期容积指数。双心室修复术后的结局与基线或扩容后的左心室形状参数无关。
左心室发育不全的形状模型显示出左心室形状的广泛差异。随着扩容，左心室获得球形度和大小并降低偏心率。尽管心室随着扩容而改变形状，但在基线或扩容后阶段，没有特定的左心室形状特征可预测进行双心室修复的决策或结局。较高的右心室质量和容积可能代表预测左心室发育不全患者双心室修复术后结局的新生物标志物。进一步研究可确定这些发现的可重复性。
