Pizarro Christian, Norwood William I
Nemours Cardiac Center, Alfred I,. duPont Hospital for Children, 1600 Rockland Road, Wilmington, DE 19899, USA.
Eur J Cardiothorac Surg. 2003 Jun;23(6):991-5. doi: 10.1016/s1010-7940(03)00158-1.
Although significant progress has been made in the perioperative management of neonates with hypoplastic left heart syndrome (HLHS), early survival has plateaued. Moreover, low but important interstage mortality remains unsolved. With a systemic to pulmonary artery shunt, the combination of significant diastolic runoff into the pulmonary circulation, a large volume load on the single ventricle and precarious coronary perfusion result in a delicate physiologic state. In order to minimize these detrimental features, a right ventricle to pulmonary artery (RV to PA) conduit was used as the source of pulmonary blood flow in patients undergoing Stage I Norwood for HLHS.
Prospective data collection in 15 consecutive patients who underwent Stage I Norwood with an RV to PA conduit.
Mean age at surgery was 2.5+/-2 days (range 1-8), mean weight was 2.9+/-0.3 kg (range 2.2-3.6) and mean gestational age was 37 weeks (range 35-40). Anatomic diagnosis was HLHS in all patients, aortic atresia was present in ten. Mean ascending aortic size was 2.9+/-0.9 mm (range 1.5-5). Two patients had moderate atrioventricular valve regurgitation and a genetic syndrome and/or congenital anomaly was present in five patients. Thirteen patients received a 5-mm polytetrafluoroethylene RV to PA conduit, and a 4-mm conduit was used in two. Mean circulatory arrest time was 55+/-6 min. Postoperatively, mean diastolic blood pressure at 1, 8 and 24 h were 47+/-7, 46+/-3 and 43+/-6 mmHg, respectively. Median time to extubation was 23 h (range 9-96) and was less than 24 h in ten patients. Median intensive care unit and hospital stay were 5 days (range 2-19) and 10 days (6-22), respectively. Early mortality was 1/15 (6%). At a mean follow-up of 10.8+/-3.4 months, 12 patients underwent stage II, and three patient have completed the Fontan.
RV to PA conduit eliminated diastolic runoff into the pulmonary vascular bed resulting in a higher diastolic blood pressure. This physiology appears to be associated with a more stable postoperative course and improved hospital survival.
尽管在左心发育不全综合征（HLHS）新生儿的围手术期管理方面已取得显著进展，但早期生存率已趋于平稳。此外，较低但很重要的过渡期死亡率仍未得到解决。通过体肺分流，大量舒张期血流进入肺循环、单心室承受大容量负荷以及冠状动脉灌注不稳定相结合，导致生理状态脆弱。为了将这些有害特征降至最低，在接受HLHS一期诺伍德手术的患者中，采用右心室至肺动脉（RV至PA）导管作为肺血流来源。
对15例连续接受带RV至PA导管的一期诺伍德手术的患者进行前瞻性数据收集。
手术时的平均年龄为2.5±2天（范围1 - 8天），平均体重为2.9±0.3千克（范围2.2 - 3.6千克），平均胎龄为37周（范围35 - 40周）。所有患者的解剖诊断均为HLHS，10例存在主动脉闭锁。升主动脉平均直径为2.9±0.9毫米（范围1.5 - 5毫米）。2例患者有中度房室瓣反流，5例患者存在遗传综合征和/或先天性异常。13例患者接受了5毫米的聚四氟乙烯RV至PA导管，2例使用了4毫米的导管。平均循环阻断时间为55±6分钟。术后，1小时、8小时和24小时的平均舒张压分别为47±7毫米汞柱、46±3毫米汞柱和43±6毫米汞柱。拔管的中位时间为23小时（范围9 - 96小时），10例患者的拔管时间小于24小时。重症监护病房和住院的中位时间分别为5天（范围2 - 19天）和10天（6 - 22天）。早期死亡率为1/15（6%）。平均随访10.8±3.4个月时，12例患者接受了二期手术，3例患者完成了Fontan手术。
RV至PA导管消除了舒张期血流进入肺血管床的情况，导致舒张压升高。这种生理状态似乎与术后病程更稳定及住院生存率提高相关。
