Friedman Kevin G, Freud Lindsay, Escobar-Diaz Maria, Banka Puja, Emani Sitaram, Tworetzky Wayne
Department of Cardiology, Children's Hospital Boston, Boston, MA, 02115, USA.
Department of Pediatrics, Harvard Medical School, Boston, MA, USA.
Pediatr Cardiol. 2015 Oct;36(7):1502-9. doi: 10.1007/s00246-015-1193-6. Epub 2015 May 15.
Fetal aortic valvuloplasty (FAV) has shown promise in averting the progression of fetal aortic stenosis to hypoplastic left-heart syndrome. Altered loading conditions due to valvar disease, intrinsic endomyocardial abnormalities, and procedures that alter endomyocardial mechanics may place patients with biventricular circulation (BiV) after FAV at risk of abnormal LV remodeling and function. Using the most recent echo data on BiV patients after technically successful FAV (n = 34), we evaluated LV remodeling pattern, risk factors for pathologic LV remodeling, and the association between LV remodeling pattern and LV function. Median age at follow-up was 4.7 years (range 1.0-12.5). Cardiac interventions were common. At latest follow-up, no patient had hypoplastic LV. Nineteen patients (55 %) had dilated LV, and five (16 %) patients had severely dilated LV. LV remodeling patterns were as follows: 12 (35 %) normal ventricle, 11 (32 %) mixed hypertrophy, 8 (24 %) eccentric hypertrophy or remodeling, and 3 (9 %) concentric hypertrophy. Univariate factors associated with pathologic LV remodeling were long-standing AR, ≥2 cardiac interventions, EFE resection, and aortic or mitral regurgitation ≥ moderate at most recent follow-up. In multivariate analysis, only long-standing AR fraction remained associated with pathologic remodeling. Pathologic LV remodeling was associated with depressed ejection fraction, lower septal E´, and higher E/E´. Pathologic LV remodeling, primarily eccentric or mixed hypertrophy, is common in BiV patients after FAV and is related to LV loading conditions imposed by valvar disease. Pathologic remodeling is associated with both systolic and diastolic dysfunction in this population.
胎儿主动脉瓣成形术（FAV）已显示出有望避免胎儿主动脉狭窄进展为左心发育不全综合征。瓣膜疾病导致的负荷条件改变、内在的心内膜异常以及改变心内膜力学的手术，可能使接受FAV后具有双心室循环（BiV）的患者面临左心室（LV）异常重塑和功能异常的风险。利用技术上成功实施FAV后BiV患者的最新超声心动图数据（n = 34），我们评估了LV重塑模式、病理性LV重塑的危险因素以及LV重塑模式与LV功能之间的关联。随访时的中位年龄为4.7岁（范围1.0 - 12.5岁）。心脏干预很常见。在最近一次随访时，没有患者出现左心室发育不全。19例患者（55%）出现左心室扩张，5例患者（16%）出现严重左心室扩张。LV重塑模式如下：12例（35%）为正常心室，11例（32%）为混合性肥厚，8例（24%）为离心性肥厚或重塑，3例（9%）为向心性肥厚。与病理性LV重塑相关的单因素包括长期存在的主动脉反流（AR）、≥2次心脏干预、心内膜弹力纤维增生症（EFE）切除术以及在最近一次随访时主动脉或二尖瓣反流≥中度。在多因素分析中，只有长期存在的AR比例仍与病理性重塑相关。病理性LV重塑与射血分数降低、室间隔E´降低以及E/E´升高有关。病理性LV重塑，主要是离心性或混合性肥厚，在FAV后的BiV患者中很常见，并且与瓣膜疾病导致的LV负荷条件有关。在这一人群中，病理性重塑与收缩和舒张功能障碍均相关。
