Hancock Hayley S, Romano Jennifer C, Armstrong Aimee, Yu Sunkyung, Lowery Ray, Gelehrter Sarah
1 Division of Pediatric Cardiology, Department of Pediatrics, Ward Family Heart Center, Children's Mercy Kansas City, Kansas City, MO, USA.
2 Division of Pediatric Cardiology, Department of Pediatrics, University of Michigan, Ann Arbor, MI, USA.
World J Pediatr Congenit Heart Surg. 2018 Jul;9(4):434-439. doi: 10.1177/2150135118771344.
Single ventricle (SV) patients with total anomalous pulmonary venous connection (TAPVC) are at high risk. Given the limited published data available, we examined outcomes and the implications of a prenatal diagnosis of SV/TAPVC.
A single-center, retrospective review was performed in neonates with SV/TAPVC from 1998 to 2014, identified through institutional databases. Patient demographic, perioperative, and follow-up data were collected.
Thirty-four eligible infants with SV/TAPVC were identified (mean birth weight: 3.0 kg). The TAPVC types were supracardiac (59%), infracardiac (21%), mixed (12%), and cardiac (9%). Heterotaxy syndrome was present in 25 (74%) infants. A prenatal diagnosis of SV was made in 26 (76%) infants, with TAPVC identified in 12 (35%). Seventeen (50%) had obstructed TAPVC within the first 48 hours of life; 7 of these patients had obstructed TAPVC identified prenatally. There were two preoperative deaths. Overall survival for the cohort was 65% at 1 year and 50% at 3 years. Survival in the obstructed group was significantly worse compared to the unobstructed group (47% vs 81% at 1 year; 27% vs 73% at 3 years, P = .01). Obstructed TAPVC and a prenatal prediction of obstructed TAPVC were significantly associated with postoperative mortality ( P = .01 and .03, respectively).
Patients with SV/TAPVC remain a high-risk group, with obstructed TAPVC a significant risk factor for mortality. Prenatal diagnosis of TAPVC in SV patients is challenging, but given those with obstructed TAPVC are especially at high risk, improved prenatal diagnostic techniques in this group may enhance counseling/delivery planning.
患有完全性肺静脉异位连接（TAPVC）的单心室（SV）患者风险很高。鉴于现有已发表的数据有限，我们研究了SV/TAPVC产前诊断的结果及其影响。
对1998年至2014年期间通过机构数据库识别出的患有SV/TAPVC的新生儿进行了单中心回顾性研究。收集了患者的人口统计学、围手术期和随访数据。
共识别出34例符合条件的SV/TAPVC婴儿（平均出生体重：3.0千克）。TAPVC类型为心上型（59%）、心下型（21%）、混合型（12%）和心脏型（9%）。25例（74%）婴儿存在内脏异位综合征。26例（76%）婴儿在产前被诊断为SV，其中12例（35%）被诊断为TAPVC。17例（50%）在出生后48小时内出现TAPVC梗阻；其中7例患者在产前被诊断为TAPVC梗阻。有2例术前死亡。该队列1年总体生存率为65%，3年为50%。梗阻组的生存率明显低于非梗阻组（1年时分别为47%和81%；3年时分别为27%和73%，P = 0.01）。TAPVC梗阻和产前预测的TAPVC梗阻与术后死亡率显著相关（分别为P = 0.01和0.03）。
SV/TAPVC患者仍然是高危人群，TAPVC梗阻是死亡的重要危险因素。SV患者TAPVC的产前诊断具有挑战性，但鉴于TAPVC梗阻患者尤其高危，改进该组患者的产前诊断技术可能会加强咨询/分娩计划。
