Ramachandran Preeti, Woo Jessica G, Ryan Thomas D, Bryant Roosevelt, Heydarian Haleh C, Jefferies John L, Towbin Jeffrey A, Lorts Angela
The Heart Institute, Cincinnati Children's Hospital, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH, 45229, USA.
Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital, Cincinnati, OH, USA.
Pediatr Cardiol. 2016 Oct;37(7):1307-12. doi: 10.1007/s00246-016-1435-2. Epub 2016 Jun 29.
Left ventricular non-compaction (LVNC) is a heterogeneous myocardial disorder characterized by prominent trabeculations and inter-trabecular recesses which may occur in association with congenital heart disease (CHD). To date, few studies have been performed to assess whether the concomitant diagnosis of LVNC affects the outcomes of CHD surgery. A retrospective review of patients with LVNC with CHD (LVNC-CHD), 0-5 years of age, was conducted. Patients with CHD without LVNC (CHD-only) and 0-5 years of age with similar diagnosis distribution were selected for comparison. Perioperative data, including CHD diagnosis, operative course, and postoperative complications were collected and compared between groups. LVNC-CHD was diagnosed in 26 children. Of the 26 with LVNC-CHD, 20 underwent surgery and these patients were compared with 276 CHD-only controls. Median total length of stay in the hospital was 12.5 days (IQR 5.5-63 days) in LVNC-CHD compared to 5 days (IQR 3-10 days) in CHD-only (p < 0.005). Postoperative death, cardiac arrest, or need for ECMO or transplantation occurred in 6/20 (30 %) of the LVNC-CHD patients compared to 3/276 (1 %) of the CHD-only group (p < 0.0001). LVNC-CHD patients had significantly longer hospital length of stay and higher perioperative complications compared to CHD-only patients without myocardial abnormalities. Pediatric cardiac care teams should be cognizant of the possibility of the increased perioperative risk associated with concomitant LVNC. Future prospective studies are warranted.
左心室心肌致密化不全(LVNC)是一种异质性心肌疾病,其特征为显著的肌小梁和小梁间隐窝,可能与先天性心脏病(CHD)相关。迄今为止,很少有研究评估LVNC的合并诊断是否会影响CHD手术的结果。对0至5岁的CHD合并LVNC(LVNC-CHD)患者进行了回顾性研究。选择年龄在0至5岁、诊断分布相似的无LVNC的CHD患者(单纯CHD)进行比较。收集两组患者的围手术期数据,包括CHD诊断、手术过程和术后并发症,并进行比较。26名儿童被诊断为LVNC-CHD。在这26名LVNC-CHD患者中,20名接受了手术,并将这些患者与276名单纯CHD对照患者进行比较。LVNC-CHD患者的中位总住院时间为12.5天(四分位间距5.5 - 63天),而单纯CHD患者为5天(四分位间距3 - 10天)(p < 0.005)。LVNC-CHD患者中有6/20(30%)发生术后死亡、心脏骤停或需要体外膜肺氧合(ECMO)或移植,而单纯CHD组为3/276(1%)(p < 0.0001)。与无心肌异常的单纯CHD患者相比,LVNC-CHD患者的住院时间明显更长,围手术期并发症更高。儿科心脏护理团队应认识到合并LVNC会增加围手术期风险的可能性。未来有必要进行前瞻性研究。