Pulmonary Artery Development Over Time in Severe Ebstein Anomaly.
Pulmonary Artery Development Over Time in Severe Ebstein Anomaly.
复制标题
严重 Ebstein 异常中肺动脉随时间的发展。
DOI：
10.1007/s00246-022-02898-w
复制
发表时间：
2022
影响因子：
1.6
通讯作者：
Sagawa K.
中科院分区：
文献类型：
--
作者：
Shirozu H;Kodama Y;Kuraoka A;Ishikawa Y;Nakano T;Kado H;Sagawa K.
最近，严重Ebstein畸形（EA）的结局随着Starnes手术的流行而得到改善。然而，中央肺动脉（PA）大小的时间依赖性变化尚未完全了解。在福冈儿童医院对新生儿期接受Starnes手术的EA患者和室间隔完整的肺动脉闭锁（PAIVS）患者进行了回顾性病历审查。重度EA组有14例患者，PAIVS组有36例患者，平均观察期分别为3.8年和4.2年。两组之间的生存率无显著差异。然而，在每个手术阶段（体肺分流后、双向Glenn手术后和Fontan手术后），重度EA组的中央PA平均尺寸较小。Fontan手术后，重度EA组的心室容积明显增大。重度EA患者的中央PA生长较差。重度EA患者在接受Fontan手术之前和之后都应仔细监测这方面的情况。关于长期预后的进一步研究是值得期待的。
Recently, the outcome of severe Ebstein anomaly (EA) has improved with the prevalence of the Starnes procedure. However, time-dependent changes in the size of the central pulmonary artery (PA) have not been fully understood. A retrospective chart review of patients with EA who underwent the Starnes procedure during the neonatal period and those with pulmonary atresia with intact ventricular septum (PAIVS) was performed at Fukuoka Children’s Hospital. There were 14 patients in the severe EA group and 36 in the PAIVS group, with mean observational periods of 3.8 and 4.2 years, respectively. No significant difference in survival was observed between the groups. However, the mean size of the central PA was smaller in the severe EA group at each surgical stage (after systemic-to-pulmonary shunt, after the bidirectional Glenn procedure, and after the Fontan procedure). A significantly larger ventricular volume was observed in the severe EA group after the Fontan procedure. The growth of the central PA was poor in patients with severe EA. Patients with severe EA should be carefully monitored in this regard both before and after undergoing the Fontan procedure. Further studies regarding long-term prognosis are expected.