Luciani G B, Chang A C, Starnes V A
Division of Cardiothoracic Surgery, Children's Hospital Los Angeles, California, USA.
Ann Thorac Surg. 1996 Mar;61(3):800-5. doi: 10.1016/0003-4975(95)01089-0.
Pulmonary hypertension due to persistent fetal circulation is rarely associated with transposition of the great arteries and intact ventricular septum. Previous attempts at management of affected neonates using prostaglandin E1 and balloon atrial septotomy followed by surgical repair have been largely unsuccessful.
Between September 1992 and April 1995, 45 neonates underwent repair of transposition of the great arteries with the arterial switch operation. Two patients (4%) with transposition of the great arteries and intact ventricular septum presented with profound reversed differential desaturation and right-to-left shunting at the level of the ductus arteriosus after balloon atrial septotomy. A diagnosis of persistent pulmonary hypertension was established and both neonates entered an experimental management protocol using inhaled nitric oxide and rapid arterial switch operation.
Preoperative hemodynamic stabilization was achieved in 1 patient using 40 parts per million of inhaled nitric oxide, whereas the other required in addition extracorporeal membrane oxygenation for severe biventricular dysfunction. Both underwent successful surgical repair 4 to 5 days after admission, but received postoperatively 1 week of inhaled nitric oxide therapy for persistent pulmonary hypertension. Follow-up echocardiography at 3 months showed good biventricular function and normal geometry of the ventricular septum, suggesting low pulmonary artery pressure, in both.
A management protocol using inhaled nitric oxide and extracorporeal membrane oxygenation followed by the arterial switch operation was successfully used in neonates with transposition of the great arteries, intact ventricular septum, and persistent pulmonary hypertension. Wider use of preoperative and postoperative inhaled nitric oxide may improve the surgical outcome of this difficult subset of patients.
持续性胎儿循环所致的肺动脉高压很少与大动脉转位及室间隔完整相关。以往尝试使用前列腺素E1和球囊房间隔造口术治疗此类新生儿，随后进行手术修复，但大多未成功。
1992年9月至1995年4月期间，45例新生儿接受了大动脉转位的动脉调转手术修复。2例（4%）大动脉转位且室间隔完整的患儿在球囊房间隔造口术后出现严重的反向差异血氧饱和度降低及动脉导管水平的右向左分流。确诊为持续性肺动脉高压，两名新生儿均进入了使用吸入一氧化氮和快速动脉调转手术的实验性治疗方案。
1例患儿使用百万分之40的吸入一氧化氮实现了术前血流动力学稳定，而另1例因严重的双心室功能障碍还需要体外膜肺氧合。两人均在入院后4至5天成功接受了手术修复，但术后因持续性肺动脉高压接受了1周的吸入一氧化氮治疗。3个月时的随访超声心动图显示两人双心室功能良好，室间隔形态正常，提示肺动脉压力较低。
使用吸入一氧化氮和体外膜肺氧合，随后进行动脉调转手术的治疗方案成功应用于大动脉转位、室间隔完整且伴有持续性肺动脉高压的新生儿。术前和术后更广泛地使用吸入一氧化氮可能会改善这类难治性患者的手术效果。
