Behrend Lea, Schaeffer Thibault, Matsubara Muneaki, Palm Jonas, Lemmen Teresa, Piber Nicole, Heinisch Paul Philipp, Georgiev Stanimir, Hager Alfred, Ewert Peter, Hörer Jürgen, Ono Masamichi
Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, University Hospital of Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Europäisches Kinderherzzentrum München, Munich, Germany.
Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, University Hospital of Technische Universität München, Munich, Germany.
Int J Cardiol Congenit Heart Dis. 2025 Jul 18;21:100611. doi: 10.1016/j.ijcchd.2025.100611. eCollection 2025 Sep.
This study evaluated the current incidence of pulmonary arteriovenous malformations (PAVMs) following stage 2 palliation (S2P).
Patients who underwent S2P, either through a bidirectional cavopulmonary shunt (BCPS) or the Kawashima procedure (KP) between 1992 and 2022, were reviewed. The cumulative incidence of PAVMs was compared between BCPS and KP. Risk factors for the development of PAVMs were identified.
Among 682 patients who underwent S2P, 661 (96.9 %) underwent BCPS and 21 (3.1 %) KP. Median age at S2P was 5.1 (interquartile ranges (IQR): 3.6-9.6) months. During the median interstage follow-up of 1.6 (IQR: 1.6-2.2) years, PAVMs developed in 11 (1.6 %) patients (1.1 % (n = 7) after BCPS and 19.0 % (n = 4) after KP). Cumulative incidence of PAVMs was higher in patients after KP than those after BCPS (p < 0.001). PAVMs were observed in the right lung in 9 patients and both lungs in two. One patient with biliary atresia died of progressive PAVMs and liver cirrhosis after KP, and the remaining 10 patients underwent Fontan completion with a median interval of 1.9 (IQR: 1.5-2.4) years. PAVMs improved in all patients (9 resolutions and 1 improved). Independent risk factors for the development of PAVMs were KP (hazard ratio (HR): 16.364, p < 0.001) in all patients, anomalous pulmonary venous connection (HR: 6.772, p = 0.023) in BCPS patients, and hypoplastic left heart syndrome (HR: 18.819, p = 0.018) in KP patients.
The incidence of PAVMs after S2P is very low after BCPS but still relevant after KP. Resolution or improvement of PAVMs is probable after Fontan completion.
本研究评估了二期姑息治疗（S2P）后肺动静脉畸形（PAVM）的当前发病率。
回顾了1992年至2022年间接受S2P治疗的患者，这些患者通过双向腔肺分流术（BCPS）或川岛手术（KP）进行治疗。比较了BCPS和KP术后PAVM的累积发病率。确定了PAVM发生的危险因素。
在682例接受S2P治疗的患者中，661例（96.9%）接受了BCPS，21例（3.1%）接受了KP。S2P时的中位年龄为5.1（四分位间距（IQR）：3.6 - 9.6）个月。在中位1.6（IQR：1.6 - 2.2）年的分期随访期间，11例（1.6%）患者发生了PAVM（BCPS术后为1.1%（n = 7），KP术后为19.0%（n = 4））。KP术后患者PAVM的累积发病率高于BCPS术后患者（p < 0.001）。9例患者的PAVM出现在右肺，2例出现在双肺。1例胆道闭锁患者在KP术后死于进行性PAVM和肝硬化，其余10例患者进行了Fontan手术，中位间隔时间为1.9（IQR：1.5 - 2.4）年。所有患者的PAVM均有改善（9例消退，1例改善）。PAVM发生的独立危险因素在所有患者中为KP（风险比（HR）：16.364，p < 0.001），在BCPS患者中为异常肺静脉连接（HR：6.772，p = 0.023），在KP患者中为左心发育不全综合征（HR：18.819，p = 0.018）。
BCPS术后S2P后PAVM的发病率非常低，但KP术后仍然相关。Fontan手术完成后，PAVM有可能消退或改善。
