Children's Hospital of Philadelphia, Philadelphia, PA, USA.
J Thorac Cardiovasc Surg. 2010 Sep;140(3):537-44. doi: 10.1016/j.jtcvs.2010.03.045.
We previously reported no difference in morbidity or mortality in a cohort of infants undergoing stage 1 and 2 reconstructions for hypoplastic left heart syndrome with either a modified Blalock-Taussig shunt or a right ventricular to pulmonary artery conduit. This article compares the hemodynamics and perioperative course at the time of the Fontan completion and reports longer-term survival for this cohort.
We retrospectively reviewed the hospital records of all patients who underwent stage 1 reconstruction between January 2002 and May 2005 and subsequent surgical procedures, as well as cross-sectional analysis of hospital survivors.
A total of 176 patients with hypoplastic left heart syndrome or a variant underwent stage 1 reconstruction with either modified Blalock-Taussig shunt (n = 114) or right ventricular to pulmonary artery conduit (n = 62). Shunt selection was at the discretion of the surgeon. The median duration of follow-up was 58 months (range 1-87 months). By Kaplan-Meier analysis, shunt type did not influence survival or freedom from transplant at 5 years (right ventricular to pulmonary artery conduit 61%; 95% confidence limit, 47-72 vs modified Blalock-Taussig shunt 70%; 95% confidence limit, 60-77; P = .55). A total of 107 patients underwent Fontan (69 modified Blalock-Taussig shunts and 38 right ventricular to pulmonary artery conduits) with 98% (105/107) early survival. Patients with a right ventricular to pulmonary artery conduit shunt pre-Fontan had higher pulmonary artery (13 +/- 8 mm Hg vs 11 +/- 3 mm Hg, P = .026) and common atrial (8 +/- 2.3 mm Hg vs 6.8 +/- 2.7 mm Hg, P = .039) pressures. By echocardiography evaluation, there was more qualitative moderate to severe ventricular dysfunction (right ventricular to pulmonary artery conduit 31% [12/36] vs modified Blalock-Taussig shunt 17% [11/67], P = .05) and moderate to severe atrioventricular valve regurgitation (right ventricular to pulmonary artery conduit 40% [14/35] vs modified Blalock-Taussig shunt 16% [11/67], P = .01) in the right ventricular to pulmonary artery conduit group. Use of diuretic therapy, angiotensin-converting enzyme inhibition, reflux medications, and tube feedings were not different between groups. Overall, 5 patients underwent heart transplantation (right ventricular to pulmonary artery conduit 4 vs modified Blalock-Taussig shunt 1, P = .1) before Fontan. There was no difference in age or weight at Fontan, bypass time, intensive care unit or hospital length of stay, postoperative pleural effusions, or need for reoperation between groups.
Interim analyses continue to suggest there is no survival advantage of one shunt type compared with the other. Longer-term follow-up of a randomized patient population remains of utmost importance.
我们之前报道过，在接受 1 期和 2 期左心发育不全综合征修复术的患儿中，使用改良 Blalock-Taussig 分流术或右心室至肺动脉导管的患儿，在发病率和死亡率方面没有差异。本文比较了 Fontan 完成时的血液动力学和围手术期情况，并报告了该队列的长期生存情况。
我们回顾性分析了 2002 年 1 月至 2005 年 5 月间接受 1 期重建术且随后接受手术治疗的所有患者的医院记录，并对医院幸存者进行了横断面分析。
共有 176 例左心发育不全综合征或变异患儿接受了 1 期改良 Blalock-Taussig 分流术（n=114）或右心室至肺动脉导管（n=62）重建术。分流术的选择由外科医生决定。中位随访时间为 58 个月（1-87 个月）。Kaplan-Meier 分析显示，分流术类型对 5 年生存率或无移植生存率无影响（右心室至肺动脉导管 61%；95%置信区间，47-72 与改良 Blalock-Taussig 分流术 70%；95%置信区间，60-77；P=0.55）。107 例患者接受了 Fontan 手术（69 例改良 Blalock-Taussig 分流术和 38 例右心室至肺动脉导管），早期存活率为 98%（105/107）。Fontan 术前接受右心室至肺动脉导管分流术的患者肺动脉压（13±8mmHg 与 11±3mmHg，P=0.026）和共同心房压（8±2.3mmHg 与 6.8±2.7mmHg，P=0.039）更高。通过超声心动图评估，右心室至肺动脉导管组有更多的中度至重度心室功能障碍（右心室至肺动脉导管 31%[12/36]与改良 Blalock-Taussig 分流术 17%[11/67]，P=0.05）和中度至重度房室瓣反流（右心室至肺动脉导管 40%[14/35]与改良 Blalock-Taussig 分流术 16%[11/67]，P=0.01）。两组在利尿剂治疗、血管紧张素转换酶抑制、反流药物和管饲方面没有差异。总体而言，Fontan 术前有 5 例患者（右心室至肺动脉导管 4 例与改良 Blalock-Taussig 分流术 1 例，P=0.1）接受了心脏移植。两组在 Fontan 时的年龄、体重、旁路时间、重症监护病房或住院时间、术后胸腔积液或再次手术的需要方面没有差异。
中期分析继续表明，两种分流术类型之间没有生存优势。随机患者人群的长期随访仍然至关重要。
