Carney Molly, Gupta Aditi, Christopher Adam, Olivieri Laura, Da Silva Jose, Diaz Castrillon Carlos, Feingold Brian, Dobson Craig P, De Fonseca Da Silva Luciana, Alsaied Tarek
Department of Pediatrics, UPMC Children's Hospital, 4401 Penn Avenue, Pittsburgh, PA, 15224, USA.
The Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
Pediatr Cardiol. 2025 Aug;46(6):1702-1709. doi: 10.1007/s00246-024-03588-5. Epub 2024 Aug 1.
The cone operation has revolutionized care for patients with Ebstein anomaly; however, acute post-operative right ventricular dysfunction (RVD) is common in this patient population. A single-center, retrospective review of 28 patients with Ebstein anomaly who underwent cardiac MRI (CMR) prior to cone reconstruction of the tricuspid valve was conducted. Measurements of atrial and ventricular size/function were assessed. Post-operative RVD was defined as the presence of moderate or severe systolic dysfunction on discharge echo. A two-tail t test was employed to compare the two groups. The average age at operation was 21.4 years (range 1.6-57.8) and 14 (50%) had RVD at discharge. Patients with post-operative RVD had significantly larger pre-operative right atrial (RA) maximum volume (p = 0.016) and RA minimum volume (p = 0.030). Patients with RVD had smaller pre-operative left atrial (LA) minimum volume (p = 0.012). Larger pre-operative right ventricular (RV) end-systolic volume (p = 0.046), lower RV ejection fraction (0.029), and smaller left ventricular (LV) end-diastolic volume (p = 0.049) were significantly associated with post-operative RVD. Post-operative RVD was associated with longer milrinone duration (p = 0.009) and higher maximum milrinone dose (p = 0.005) but was not associated with intensive care or hospital length of stay (p = 0.19 and 0.67, respectively). Increased RA and RV dilation and decreased LA and LV volumes are associated with the development of post-operative RVD following cone operation for Ebstein anomaly. Post-operative RVD affects milrinone dose and duration but is not associated with increased length of stay.
圆锥手术彻底改变了对埃布斯坦畸形患者的治疗方式；然而，急性术后右心室功能障碍（RVD）在该患者群体中很常见。对28例在三尖瓣圆锥重建术前接受心脏磁共振成像（CMR）的埃布斯坦畸形患者进行了单中心回顾性研究。评估了心房和心室的大小/功能。术后RVD定义为出院时超声心动图显示存在中度或重度收缩功能障碍。采用双尾t检验比较两组。手术时的平均年龄为21.4岁（范围1.6 - 57.8岁），14例（50%）出院时患有RVD。术后RVD患者术前右心房（RA）最大容积（p = 0.016）和RA最小容积（p = 0.030）显著更大。RVD患者术前左心房（LA）最小容积更小（p = 0.012）。术前右心室（RV）收缩末期容积更大（p = 0.046）、RV射血分数更低（0.029）以及左心室（LV）舒张末期容积更小（p = 0.049）与术后RVD显著相关。术后RVD与米力农使用时间更长（p = 0.009）和最大米力农剂量更高（p = 0.005）相关，但与重症监护或住院时间无关（分别为p = 0.19和0.67）。埃布斯坦畸形圆锥手术后，RA和RV扩张增加以及LA和LV容积减小与术后RVD的发生相关。术后RVD影响米力农剂量和使用时间，但与住院时间延长无关。
