Yi Tong, Fan Guohui, Xing Yunchao, Zhao Wei, Zhang Liang, Fan Fan, Jiang Xianchao, Ma Zhiling, Yan Jun, Li Shoujun, Wang Qiang
Department of Congenital Heart Disease, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 167 North Lishi Road, Xicheng District, Beijing, China.
Institute of Clinical Medical Sciences, China-Japan Friendship Hospital, Beijing, China.
Pediatr Cardiol. 2019 Apr;40(4):705-712. doi: 10.1007/s00246-018-2049-7. Epub 2019 Jan 16.
The bidirectional cavopulmonary shunt (BCPS) is an effective palliative procedure which has been widely used to boost outcome of the Fontan procedure. However, there is no standard duration time between these two procedures. Therefore, we investigated whether different time intervals between BCPS and Fontan procedure affects morbidity and mortality of Fontan patients.
Between 2004 and 2016, 210 post-BCPS patients underwent Fontan operation at Fuwai Hospital. The median interval between BCPS and Fontan procedure was 3.7 years (range 0.55-11.86 years) and this was used to divide study cohort into Group 1 (< 3.7 years; n = 124) and Group 2 (> 3.7 years; n = 86). We analyzed these patients retrospectively in terms of their preoperative characteristics and post-operative and follow-up results.
Weight z-scores for age at BCPS (- 0.73 ± 1.39 vs - 1.17 ± 1.60, p < 0.05) was significantly higher in Group 2. However, saturation at room air before Fontan (76.42 ± 20.01 vs 82.85 ± 9.69, p < 0.001) was significantly higher in Group 1. The morbidity and mortality were similar between two groups. There were twelve hospital deaths (5.7%): eight (8/124, 6.5%) presented in Group 1 and four (4/86, 4.7%) in Group 2. On multi-variable analysis, risk factors for death were prolonged mechanical ventilation [hazard ratio (HR) 1.02, p = 0.004] and single right ventricle (HR 7.17, p = 0.03). After a mean follow-up of 4.95 years (range 0.74-13.62 years), one patient in Group 1 died of heart failure 13 months after Fontan procedure. The overall Fontan failure in Group 1 was similar to that in Group 2 (2.7% vs 2.6%, p = 0.985). The incidence of arrhythmias and re-intervention were not different between the two groups.
Fontan procedure could be performed safely in patient who stayed in long duration between Fontan procedure and BCPS without affecting the operative and long-term follow-up results. However, for post-BCPS patients with severe hypoxemia, earlier age at Fontan might be a good choice.
双向腔肺分流术（BCPS）是一种有效的姑息性手术，已被广泛用于改善Fontan手术的效果。然而，这两种手术之间没有标准的间隔时间。因此，我们研究了BCPS与Fontan手术之间不同的时间间隔是否会影响Fontan患者的发病率和死亡率。
2004年至2016年期间，210例接受BCPS手术后的患者在阜外医院接受了Fontan手术。BCPS与Fontan手术之间的中位间隔时间为3.7年（范围0.55 - 11.86年），以此将研究队列分为第1组（<3.7年；n = 124）和第2组（>3.7年；n = 86）。我们对这些患者的术前特征、术后及随访结果进行了回顾性分析。
第2组患者在BCPS时的年龄体重Z评分（-0.73±1.39 vs -1.17±1.60，p < 0.05）显著更高。然而，第1组患者在Fontan手术前室内空气饱和度（76.42±20.01 vs 82.85±9.69，p < 0.001）显著更高。两组的发病率和死亡率相似。有12例住院死亡（5.7%）：第1组有8例（8/124，6.5%），第2组有4例（4/86，4.7%）。多变量分析显示，死亡的危险因素为机械通气时间延长[风险比（HR）1.02，p = 0.004]和单右心室（HR 7.17，p = 0.03）。平均随访4.95年（范围0.74 - 13.62年）后，第1组有1例患者在Fontan手术后13个月死于心力衰竭。第1组的总体Fontan失败率与第2组相似（2.7% vs 2.6%，p = 0.985）。两组心律失常和再次干预的发生率无差异。
对于BCPS与Fontan手术间隔时间较长的患者，Fontan手术可以安全进行，且不影响手术及长期随访结果。然而，对于BCPS术后严重低氧血症的患者，Fontan手术时年龄较小可能是一个不错的选择。
