Barry Oliver M, Friedman Kevin G, Bergersen Lisa, Emani Sitaram, Moeyersoms Acadia, Tworetzky Wayne, Marshall Audrey C, Lock James E
Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts; Department of Pediatrics, Harvard Medical School, Boston, Massachusetts.
Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts; Department of Pediatrics, Harvard Medical School, Boston, Massachusetts.
Am J Cardiol. 2018 Aug 1;122(3):511-516. doi: 10.1016/j.amjcard.2018.04.035. Epub 2018 May 1.
At our institution a multidisciplinary team has performed fetal aortic valvuloplasty (FAV) for severe aortic stenosis with evolving hypoplastic left heart syndrome with high technical success rates. Measurement of postnatal success has been biventricular circulation (BC). Postnatal survival for patients after FAV who achieved a BC appears encouraging. However, there are limited late clinical and hemodynamic outcomes in this cohort and there is concern for diastolic dysfunction. We reviewed all patients with FAV at our institution who initially underwent single ventricle palliation and subsequently BC, as this is likely the subset at high-risk for poor outcomes. Clinical, imaging, and surgical data were collected. Two of 7 patients (29%) died within 16 months of BC, and 1 patient has been listed for transplant. Diastolic dysfunction was common and progressive with median left ventricular end diastolic pressure of 12 mm Hg before BC, and increasing to 22 mm Hg for survivors at last follow-up. Left ventricular size was adequate with all patients reaching a left ventricular end diastolic volume (LVEDV) z score in the normal or elevated range. Presence and severity of residual valve lesions decreased over time secondary to a median of 6 interventions (range 3 to 10), either surgical or cath-based, performed for these 7 patients during the study period. In conclusion, clinical outcomes are concerning for this high-risk group. Diastolic dysfunction is persistent and progressive despite anatomic interventions and adequate left ventricular growth. The main contributing factor to poor outcomes may be intrinsic myocardial dysfunction and primordial pathology. Achievement of a BC after FAV may not be an appropriate measure of success.
在我们机构，一个多学科团队对患有逐渐发展的左心发育不全综合征的严重主动脉瓣狭窄患者进行了胎儿主动脉瓣成形术（FAV），技术成功率很高。出生后成功的衡量标准是双心室循环（BC）。对于实现了BC的FAV术后患者，其出生后的生存率似乎令人鼓舞。然而，该队列中晚期临床和血流动力学结果有限，且存在舒张功能障碍的问题。我们回顾了我们机构所有最初接受单心室姑息治疗并随后实现BC的FAV患者，因为这可能是预后不良的高风险亚组。收集了临床、影像学和手术数据。7名患者中有2名（29%）在BC后16个月内死亡，1名患者已被列入移植名单。舒张功能障碍很常见且呈进行性发展，BC前左心室舒张末期压力中位数为12 mmHg，在最后一次随访时，存活患者的该数值增加到了22 mmHg。所有患者的左心室大小足够，左心室舒张末期容积（LVEDV）z评分均在正常或升高范围内。在研究期间，这7名患者因残余瓣膜病变接受了中位数为6次（范围3至10次）的手术或基于导管的干预，残余瓣膜病变随时间推移而减轻。总之，该高风险组的临床结果令人担忧。尽管进行了解剖学干预且左心室生长充分，但舒张功能障碍仍然持续且呈进行性发展。预后不良的主要因素可能是心肌内在功能障碍和原始病理状态。FAV后实现BC可能不是衡量成功的合适指标。
