Serraf A, Conte S, Lacour-Gayet F, Bruniaux J, Sousa-Uva M, Roussin R, Planché C
Department of Pediatric Cardiac Surgery, Marie Lannelongue Hospital, Le Plessis-Robinson, France.
Ann Thorac Surg. 1995 Oct;60(4):970-6; discussion 976-7. doi: 10.1016/0003-4975(95)00520-u.
The surgical management for bridging patients with univentricular heart and systemic obstruction to a Fontan procedure remains controversial.
Twenty-seven of 96 patients with univentricular heart and unobstructed pulmonary blood flow referred for surgical palliation were seen with systemic obstruction. Twenty-six were neonates with coarctation of the aorta in 21 and subaortic stenosis in 5. In 8 other patients, subaortic stenosis developed after initial pulmonary artery banding. Four different palliative procedures were performed: coarctation repair with pulmonary artery banding (group I, n = 15); Norwood or Damus-Kaye-Stansel or arterial switch operation (group II, n = 9); coarctation repair with pulmonary artery banding and bulboventricular foramen enlargement (group III, n = 2); and orthotopic heart transplantation with coarctation repair (group IV, n = 1).
The mortality rate was 34.3% (n = 12) for all patients, 53.3% in group I, 33.3% in group II (p = 0.003 versus group I), and 50% in group III. Nine patients (8 in group I and 1 in group II) had development of subaortic stenosis and underwent a subsequent procedure: Damus-Kaye-Stansel operation in 5, arterial switch operation in 3, and bulboventricular foramen enlargement in 1. Three had a concomitant or subsequent Fontan procedure and 2, a bidirectional Glenn procedure. In group II, 1 patient underwent a subsequent Fontan procedure and another, a bidirectional Glenn anastomosis. Six of the 8 patients with subaortic stenosis after initial pulmonary artery banding underwent a second stage consisting of a Damus-Kaye-Stansel procedure (n = 3), bulboventricular foramen enlargement (n = 2), or creation of an aortopulmonary window (n = 1). Three had a concomitant Fontan procedure and 2, a bidirectional Glenn procedure. Actuarial 4-year survival was 65.5% +/- 8.4% (70% confidence limits) for all patients; it was 40% +/- 13.3% in group I and 66.6% +/- 16.3% in group II (p < 0.05).
Initial management of patients with univentricular heart and systemic obstruction by Norwood-like procedures provides a better outcome. Success of the Fontan operation relies on the ability to provide timely relief of subaortic stenosis.
对于单心室心脏合并体循环梗阻的患者，在进行Fontan手术前的外科治疗方案仍存在争议。
96名单心室心脏且肺血流无梗阻的患者被转诊接受外科姑息治疗，其中27例存在体循环梗阻。26例为新生儿，21例患有主动脉缩窄，5例患有主动脉瓣下狭窄。另外8例患者在初次肺动脉环扎术后出现主动脉瓣下狭窄。实施了四种不同的姑息手术：肺动脉环扎联合主动脉缩窄修复术（I组，n = 15）；Norwood手术、Damus-Kaye-Stansel手术或动脉调转术（II组，n = 9）；肺动脉环扎联合主动脉缩窄修复及球室孔扩大术（III组，n = 2）；原位心脏移植联合主动脉缩窄修复术（IV组，n = 1）。
所有患者的死亡率为34.3%（n = 12），I组为53.3%，II组为33.3%（与I组相比，p = 0.003），III组为50%。9例患者（I组8例，II组1例）出现主动脉瓣下狭窄并接受了后续手术：5例行Damus-Kaye-Stansel手术，3例行动脉调转术，1例行球室孔扩大术。3例同时或随后接受了Fontan手术，2例接受了双向Glenn手术。II组中，1例患者随后接受了Fontan手术，另1例接受了双向Glenn吻合术。最初肺动脉环扎术后发生主动脉瓣下狭窄的8例患者中，6例接受了第二阶段手术，包括Damus-Kaye-Stansel手术（n = 3）、球室孔扩大术（n = 2）或建立主肺动脉窗（n = 1）。3例同时接受了Fontan手术，2例接受了双向Glenn手术。所有患者的4年精算生存率为65.5%±8.4%（70%置信区间）；I组为40%±13.3%，II组为66.6%±16.3%（p < 0.05）。
采用类似Norwood手术的方法对单心室心脏合并体循环梗阻的患者进行初始治疗可获得更好的结果。Fontan手术的成功依赖于及时解除主动脉瓣下狭窄的能力。
