Ohuchi Hideo, Mori Aki, Nakai Michikazu, Fujimoto Kazuto, Iwasa Toru, Sakaguchi Heima, Kurosaki Kenichi, Shiraishi Isao
Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Suita, Japan.
Adult Congenital Heart Disease, National Cerebral and Cardiovascular Center, Suita, Japan.
Front Pediatr. 2022 Jun 9;10:713219. doi: 10.3389/fped.2022.713219. eCollection 2022.
The Fontan operation is a surgical procedure used in children with univentricular hearts. Pulmonary arteriovenous fistulae (PAVF) is a major complication after a Fontan operation. However, the incidence and related clinical pathophysiology of PAVF remain unclear.
This study aimed to clarify the incidence of PAVF, its clinical characteristics, and its influence on all-cause mortality.
We serially assessed the presence of PAVF using pulmonary artery angiography and/or contrast echocardiography during catheterization in 391 consecutive patients who underwent the Fontan procedure and compared the results with the Fontan pathophysiology and all-cause mortality. PAVF developed in 36 patients (9.2%), including 30 diffuse- and six discrete-PAVF types. The PAVF-free rates at 1, 5, 10, 15, 20, and ≥25 years after Fontan operation were 97, 96, 93, 88, 87, and 83%, respectively. The mean arterial blood oxygen saturation (SaO) in patients with diffuse PAVF at each corresponding postoperative stage were 90, 91, 91, 91, 89, and 88%, respectively, indicating lower SaO levels than those in patients without PAVF (all < 0.01). However, there was no difference in the SaO levels between patients with discrete PAVF and those without PAVF. During a median follow-up period of 2.9 years after the last catheterization, 31 patients, including 12 patients with PAVF, died. Patients with PAVF, especially those with diffuse PAVF, had a higher mortality rate ( = 0.01) than those without PAVF (hazard ratio: 3.6, 95% confidence interval: 1.6-7.8, = 0.0026).
Patients who underwent Fontan surgery had an increased incidence of PAVF as they aged. Discrete PAVF did not influence SaO or mortality, whereas the presence of diffuse PAVF caused hypoxia and was associated with all-cause mortality.
Fontan手术是用于单心室患儿的一种外科手术。肺动静脉瘘（PAVF）是Fontan手术后的一种主要并发症。然而，PAVF的发生率及相关临床病理生理学仍不清楚。
本研究旨在明确PAVF的发生率、临床特征及其对全因死亡率的影响。
我们对391例连续接受Fontan手术的患者在导管插入术期间使用肺动脉造影和/或对比超声心动图对PAVF的存在情况进行了连续评估，并将结果与Fontan病理生理学及全因死亡率进行了比较。36例患者（9.2%）发生了PAVF，包括30例弥漫型和6例离散型PAVF。Fontan手术后1、5、10、15、20及≥25年无PAVF的发生率分别为97%、96%、93%、88%、87%和83%。各相应术后阶段弥漫型PAVF患者的平均动脉血氧饱和度（SaO）分别为90%、91%、91%、91%、89%和88%，表明其SaO水平低于无PAVF的患者（均P<0.01）。然而，离散型PAVF患者与无PAVF患者的SaO水平无差异。在最后一次导管插入术后的中位随访期2.9年期间，31例患者死亡，其中包括12例PAVF患者。PAVF患者，尤其是弥漫型PAVF患者，死亡率高于无PAVF患者（P=0.01，风险比：3.6，95%置信区间：1.6-7.8，P=0.0026）。
接受Fontan手术的患者随着年龄增长PAVF发生率增加。离散型PAVF不影响SaO或死亡率，而弥漫型PAVF的存在会导致缺氧并与全因死亡率相关。
