未经证实:新生儿完全性肺静脉异位引流(TAPVD)修复后的死亡率仍然很高。分析危险因素可能有助于确定治疗靶点以提高生存率。
UNASSIGNED:对所有接受简单TAPVD修复的新生儿进行回顾性分析。
UNASSIGNED:在1973年至2021年之间,175例新生儿接受了TAPVD修复,中位年龄为6天(四分位数间距,2-15天),平均体重为3.2±0.6kg。在42.3%的患者中,TAPVD是心上的(175个中的74个),心脏占14.3%(175个中的25个),40%(175个中的70个),混合类型占3.4%(175个中的6个),梗阻占65.7%(175个中的115个)。肺动脉高压(PHT)危象发生在12%(175个中的21个)。早期死亡率为9.7%(175个中的17个),晚期死亡率为5.1%(158个中的8个),大多数死亡发生在1年内(75%;8个中的6个)。1年生存率为86.5%(95%CI,80.3%-90.8%),5、10、15和20年生存率为85.8%(95%CI,79.6%-90.3%)。TAPVD梗阻患者的生存率较低,紧急手术的患者,还有那些有PHT危机的人。PHT危机(危险比[HR],4.93;95%CI,1.95-12.51;P=.001),手术的紧迫性(HR,2.51;95%CI,1.11-5.68;P=0.027),和较高的肺动脉压与全身血压的百分比(HR,1.06;95%CI,1.01-1.11;P=0.026)被确定为死亡的危险因素。对17例患者(9.7%;175例中的17例)的组织病理学分析显示,肺动脉高压的征象为58.8%(17例中的10例)。
UNASSIGNED:TAPVD修复后的死亡率主要发生在生命的第一年内。手术的紧迫性和持续的PHT似乎是死亡的危险因素。肺活检可能有助于识别有风险的患者并指导更新的治疗方式。
UNASSIGNED: Mortality after repair of total anomalous pulmonary venous drainage (TAPVD) in neonates has remained high. Analysis of risk factors may help identify therapeutic targets to improve survival.
UNASSIGNED: Retrospective analysis of all neonates who underwent simple TAPVD repair.
UNASSIGNED: Between 1973 and 2021, 175 neonates underwent TAPVD repair, at a median age of 6 days (interquartile range, 2-15 days) and a mean weight of 3.2 ± 0.6 kg. TAPVD was supracardiac in 42.3% of the patients (74 of 175), cardiac in 14.3% (25 of 175), infracardiac in 40% (70 of 175), and mixed type in 3.4% (6 of 175), with obstruction in 65.7% (115 of 175). Pulmonary hypertension (PHT) crisis occurred in 12% (21 of 175). Early mortality was 9.7% (17 of 175) and late mortality was 5.1% (8 of 158), with most deaths occurring within 1 year (75%; 6 of 8). Survival was 86.5% (95% CI, 80.3%-90.8%) at 1 year and 85.8% (95% CI, 79.6%-90.3%) at 5, 10, 15, and 20 years. Survival was lower in patients with obstructed TAPVD, patients with emergent surgery, and those with PHT crisis. PHT crisis (hazard ratio [HR], 4.93; 95% CI, 1.95-12.51; P = .001), urgency of surgery (HR, 2.51; 95% CI, 1.11-5.68; P = .027), and higher pulmonary artery pressure-to-systemic blood pressure percentage ratio (HR, 1.06; 95% CI, 1.01-1.11; P = .026) were identified as risk factors for mortality. Histopathological analysis of 17 patients (9.7%; 17 of 175) showed signs of pulmonary arterial hypertension with media hypertrophy in 58.8% (10 of 17).
UNASSIGNED: Mortality after TAPVD repair occurred mainly within the first year of life. Urgency of surgery and persistent PHT appears to be risk factors for mortality. Lung biopsy might be useful for identifying patients at risk and guiding newer treatment modalities.