Objective

This study aimed to evaluate and discuss the outcomes of creating a single systemic-pulmonary shunt and reconstruction of the pulmonary artery continuity in patients with a single functional ventricle, absent central pulmonary artery, and bilateral patent ductus arteriosus.

Methods

Six infants diagnosed with a functional single ventricle, absent central pulmonary artery, and bilateral patent ductus arteriosus were treated by creating a single systemic-pulmonary shunt and reconstructing the pulmonary artery continuity (primary operation) between January 2010 and September 2020. Pulmonary artery continuity was ensured using the remnant pulmonary artery and an autologous pericardial patch in five patients and a rolled autologous pericardium in one patient.

Results

All patients eventually underwent total cavopulmonary connection. Two patients underwent intrapulmonary artery septation before Glenn or total cavopulmonary connection procedure. The median follow-up period was 9.02 years (interquartile range, 3.90–9.53). No late deaths were observed.

Conclusions

Our strategy of establishing a single systemic-pulmonary shunt with reconstruction of the pulmonary artery continuity was useful for treating patients with a functional single ventricle with absent central pulmonary artery and bilateral patent ductus arteriosus. This procedure helped accomplish pulmonary artery growth and ensured an appropriate volume load after total cavopulmonary connection.

中文翻译：

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中央肺动脉缺如双侧动脉导管未闭的功能性单心室原发性肺动脉重建

客观的

本研究旨在评估和讨论在单功能心室、肺中央动脉缺失和双侧动脉导管未闭的患者中创建单一体肺分流术和重建肺动脉连续性的结果。

方法

在 2010 年 1 月至 2020 年 9 月期间，6 名被诊断为功能性单心室、肺中央动脉缺失和双侧动脉导管未闭的婴儿通过建立单一的体肺分流术和重建肺动脉连续性（初步手术）进行治疗。肺动脉连续性是确保在 5 名患者中使用残肺动脉和自体心包贴片，在 1 名患者中使用滚动自体心包。

结果

所有患者最终都接受了全腔静脉肺连接。两名患者在 Glenn 或全腔静脉肺连接手术前接受了肺内动脉分隔。中位随访时间为 9.02 年（四分位距，3.90-9.53）。没有观察到晚期死亡。

结论

我们建立单个体肺分流并重建肺动脉连续性的策略对于治疗功能性单心室、中央肺动脉缺失和双侧动脉导管未闭的患者很有用。该程序有助于完成肺动脉生长，并确保在全腔肺连接后适当的容量负荷。