杂交技术治疗室间隔完整型肺动脉闭锁

目的 总结杂交技术经胸肺动脉瓣球囊扩张成形术治疗室间隔完整型肺动脉闭锁的即刻疗效及近、中期随访结果.方法 2005年3月至2010年3月,采用超声引导经胸肺动脉瓣球囊扩张成形术治疗室间隔完整型肺动脉膜性闭锁30例,年龄1天～48个月,平均(4.59±3.21)个月.胸骨正中切口,于右室流出道距离肺动脉瓣环下约2 cm缝荷包线,然后置入导丝.在超声引导下置入穿刺鞘管.确认穿刺针对准膜性闭锁的瓣膜后,在钢丝引导下放入球囊扩张管进行扩张,超声提示肺动脉瓣开放满意.＜3个月病婴行改良Blalock-Taussig(B-T)体肺分流术,并同期行动脉导管结扎术.＞3个月病婴行球囊扩张术后,如血氧饱和度改善明显,不常规行改良B-T分流术,并保留动脉导管开放,如血氧饱和度改善不明显,则考虑行改良B-T分流术,结扎或保留动脉导管.＞5个月病儿行球囊扩张后血氧饱和度改善不满意,且重度右心发育不良,则选择双向Glenn术.结果 30例行球囊扩张均取得成功,同期行动脉导管结扎术25例,改良B-T分流术8例,双向Glenn术2例.均未出现严重并发症.1例术后因低氧血症,术后第3天行动脉导管结扎术和改良B-T分流术;余者术后血流动力学稳定,顺利出院.术后随访1.5～62.0个月,平均(18.7±17.2)个月.血氧饱和度由术前0.73±0.08上升至0.94±0.04,心功能Ⅰ级.院外死亡5例,25例生长发育良好.结论 杂交技术经胸肺动脉瓣球囊扩张成形术是一种治疗新生儿及婴幼儿室间隔完整型肺动脉膜性闭锁的安全、有效的方法.

Abstract：

Objective In patients with pulmonary atresia and intact ventricular septum ( PAIVS) without right ventricular-dependent coronaries, catheter techniques including the use of a sniff wire, lasers, and radiofrequency have been the most widely used initial therapy. However, percutaneous perforation and balloon valvuloplasty were associated with higher rate of procedural failure and serious complications. Methods We report our experience with a hybrid approach for pulmonary atresia with intact ventricular septum, combining surgery and interventional catheterization techniques. Between March 2005 and March 2010, hybrid procedure was carried out successfully in 30 newboms and infants with favorable anatomy. The age ranged from 1 day to 48 months with a mean of (4.59 ±3.21) months. The heart was exposed through median sternotomy. A pursestring suture was placed in the right ventricular outflow tract 2 cm away from the pulmonary trunk. Then a 16-gauge intravenous catheter was punctured through the right ventrical and perforated the atretic PV with the guidance of echocardiography. A guide wire was then inserted into the sheath and used to guide the balloon across the PV. Sequential dilations were performed until a full opening of the PV with the guidance of epicardial echocardiography. In patients ＜ 3 months PDA ligation was performed followed by modified Blalock-Taussig (B-T) shunt. In patients ＞ 3 months PDA ligation was not performed. A modified B-T shunt was inserted if severe systemic oxygen desaturation occurred after PDA ligation. Bidirectional Glenn shunt was performed for severe hypoplasia. Hybrid procedure was achieved in all patients. The simultaneous procedures included 25 cases of PDA ligation. 6 newborns underwent modified B-T shunt placement (3.5 to 5 mm) after pulmonary valvuloplasty and PDA ligation, and 2 patients ＞ 1 month underwent modified B-T shunt. Another 2 patients were selected for univentricular palliative surgery because of a diminutive monopartite right ventricle and bidirectional Glenn procedure was performed. No pericardial effusion or cardiac tamponade was observed in all patients. Another case without PDA ligation underwent a modified B-T shunt because of hypoxemia three days after hybrid procedure, and the rest patients were discharged without any further surgical intervention.During the follow-up period of 1.5 to 62.0 months, 5 patients died. 25 (83.3%) survived and were all in New York Heart Association functional class 1. Peripheral oxygen saturation increased from 0.73 ± 0.08 to 0.94 ± 0.04 (P ＜ 0.05). One patient remains in a single-ventricle pathway, whereas 24 patients achieved a two-ventricle circulation. Results Conclusion Perventricular balloon pulmonary valvuloplasty using a hybrid approach is a safe and feasible procedure for patients with PAIVS.

Hybrid procedure for pulmonary atresia with intact ventricular septum

Objective In patients with pulmonary atresia and intact ventricular septum ( PAIVS) without right ventricular-dependent coronaries, catheter techniques including the use of a sniff wire, lasers, and radiofrequency have been the most widely used initial therapy. However, percutaneous perforation and balloon valvuloplasty were associated with higher rate of procedural failure and serious complications. Methods We report our experience with a hybrid approach for pulmonary atresia with intact ventricular septum, combining surgery and interventional catheterization techniques. Between March 2005 and March 2010, hybrid procedure was carried out successfully in 30 newboms and infants with favorable anatomy. The age ranged from 1 day to 48 months with a mean of (4.59 ±3.21) months. The heart was exposed through median sternotomy. A pursestring suture was placed in the right ventricular outflow tract 2 cm away from the pulmonary trunk. Then a 16-gauge intravenous catheter was punctured through the right ventrical and perforated the atretic PV with the guidance of echocardiography. A guide wire was then inserted into the sheath and used to guide the balloon across the PV. Sequential dilations were performed until a full opening of the PV with the guidance of epicardial echocardiography. In patients ＜ 3 months PDA ligation was performed followed by modified Blalock-Taussig (B-T) shunt. In patients ＞ 3 months PDA ligation was not performed. A modified B-T shunt was inserted if severe systemic oxygen desaturation occurred after PDA ligation. Bidirectional Glenn shunt was performed for severe hypoplasia. Hybrid procedure was achieved in all patients. The simultaneous procedures included 25 cases of PDA ligation. 6 newborns underwent modified B-T shunt placement (3.5 to 5 mm) after pulmonary valvuloplasty and PDA ligation, and 2 patients ＞ 1 month underwent modified B-T shunt. Another 2 patients were selected for univentricular palliative surgery because of a diminutive monopartite right ventricle and bidirectional Glenn procedure was performed. No pericardial effusion or cardiac tamponade was observed in all patients. Another case without PDA ligation underwent a modified B-T shunt because of hypoxemia three days after hybrid procedure, and the rest patients were discharged without any further surgical intervention.During the follow-up period of 1.5 to 62.0 months, 5 patients died. 25 (83.3%) survived and were all in New York Heart Association functional class 1. Peripheral oxygen saturation increased from 0.73 ± 0.08 to 0.94 ± 0.04 (P ＜ 0.05). One patient remains in a single-ventricle pathway, whereas 24 patients achieved a two-ventricle circulation. Results Conclusion Perventricular balloon pulmonary valvuloplasty using a hybrid approach is a safe and feasible procedure for patients with PAIVS.