Pôle des cardiopathies congénitales de l'enfant et de l'adulte, centre de référence malformations cardiaques congénitales complexes (M3C), hôpital Marie-Lannelongue, université Paris-Sud, université Paris-Saclay, 133, avenue de la Résistance, 92350 Le Plessis-Robinson, France.
Pôle des cardiopathies congénitales de l'enfant et de l'adulte, centre de référence malformations cardiaques congénitales complexes (M3C), hôpital Marie-Lannelongue, université Paris-Sud, université Paris-Saclay, 133, avenue de la Résistance, 92350 Le Plessis-Robinson, France.
Arch Cardiovasc Dis. 2019 May;112(5):323-333. doi: 10.1016/j.acvd.2018.11.015. Epub 2019 Feb 20.
Transcatheter pulmonary valvuloplasty in neonates with pulmonary atresia and intact ventricular septum (PA-IVS) or duct-dependent pulmonary valve stenosis (DD-PVS) has become a reasonable alternative to surgical right ventricle decompression.
To investigate mid-term outcomes following pulmonary valvuloplasty.
Sixty-five neonates with PA-IVS (n=29) or DD-PVS (n=36) (median age 4 days; mean weight 3.0kg) undergoing pulmonary valvuloplasty were reviewed retrospectively. Procedural data and clinical outcomes were assessed.
Pulmonary valvuloplasty was successful in 59 patients (90.8%). Preterm birth, larger tricuspid valve annulus diameter and PA-IVS correlated with procedural failure. Eleven patients (18.6%) required a Blalock-Taussig shunt during early follow-up, despite valvuloplasty. These neonates had smaller tricuspid and pulmonary valve annulus Z-scores (-1.9 vs. -0.8 [p=0.04] and -2.5 vs. -0.9 [P=0.005], respectively) and a higher incidence of "bipartite" right ventricle (P=0.02). Mean follow-up was 5.4±3.3 years. Mortality after successful valvuloplasty was 8.5% (n=5). Among the 54 survivors, biventricular repair was achieved in 52 patients (96.3%), including nine with a previous Blalock-Taussig shunt. The cumulative rate of subsequent surgery (excluding Blalock-Taussig shunt) was 13.7% (95% confidence interval 6.8-26.7%) and 16.4% (95% confidence interval 8.5-30.4%) at 2 and 4 years, respectively. Secondary surgery was significantly more frequent in PA-IVS compared with DD-PVS, and in neonates with a Blalock-Taussig shunt (P=0.003 and 0.01, respectively).
Selected neonates with DD-PVS or PA-IVS managed by transcatheter pulmonary valvuloplasty had a good mid-term outcome. In neonates with a borderline small right ventricle, a hybrid strategy with a supplementary source of pulmonary blood flow can be efficient to achieve biventricular repair.
经导管肺动脉瓣成形术已成为治疗伴有完整室间隔的肺动脉瓣闭锁（PA-IVS）或依赖导管的肺动脉瓣狭窄（DD-PVS）新生儿的合理选择，可替代外科右心室减压术。
探讨肺动脉瓣成形术后的中期结果。
回顾性分析 65 例接受肺动脉瓣成形术的 PA-IVS（n=29）或 DD-PVS（n=36）新生儿（中位年龄 4 天，平均体重 3.0kg）。评估手术资料和临床结果。
59 例（90.8%）患儿肺动脉瓣成形术成功。早产、较大的三尖瓣瓣环直径和 PA-IVS 与手术失败相关。11 例（18.6%）患儿尽管行肺动脉瓣成形术，仍需在早期行 Blalock-Taussig 分流术。这些新生儿的三尖瓣和肺动脉瓣环 Z 评分更小（-1.9 与-0.8，P=0.04；-2.5 与-0.9，P=0.005），“二分叶”右心室发生率更高（P=0.02）。平均随访 5.4±3.3 年。肺动脉瓣成形术成功患儿的死亡率为 8.5%（n=5）。54 例存活者中，52 例（96.3%）行双心室修复术，其中 9 例行 Blalock-Taussig 分流术。随后的手术（不包括 Blalock-Taussig 分流术）累计率分别为 2 年时 13.7%（95%可信区间 6.8-26.7%）和 4 年时 16.4%（95%可信区间 8.5-30.4%），且双心室修复术在 PA-IVS 患儿中明显比 DD-PVS 患儿更频繁（P=0.003 和 0.01），也在 Blalock-Taussig 分流术患儿中更频繁（P=0.003 和 0.01）。
经导管肺动脉瓣成形术治疗的选择性 DD-PVS 或 PA-IVS 新生儿有良好的中期结果。对于右心室较小的新生儿，可采用带辅助肺血来源的杂交策略，有效实现双心室修复。
