Jonas R A, Hansen D D, Cook N, Wessel D
Department of Cardiac Surgery, Children's Hospital, Boston, MA 02115.
J Thorac Cardiovasc Surg. 1994 Apr;107(4):1121-7; discussion 1127-8.
We conducted a retrospective study of 78 patients who underwent palliative reconstructive operation for hypoplastic left heart syndrome representing an entire consecutive experience between 1983 and 1991 to identify predictors of mortality that might enable more appropriate triage of patients to either reconstruction or transplantation. Twenty-nine patients had aortic atresia, mitral atresia; 18 had aortic stenosis, mitral stenosis; 20 had aortic atresia and mitral stenosis; and 11 had miscellaneous forms of hypoplastic left heart syndrome. There were 29 hospital deaths (37%). A worst preoperative pH (p = 0.01) and immediate preoperative pH (p = 0.03) less than the median were predictors of hospital mortality. The anatomic subgroup aortic atresia, mitral stenosis (p = 0.06) had a possible increased hospital mortality. One patient was lost to follow-up. The Kaplan-Meier survival estimate among hospital survivors was 34% at 3 years and 25% at 5 years. The anatomic subgroup aortic atresia, mitral atresia (p = 0.02) had a worse late outcome (11% 3-year survival) whereas the subgroup aortic stenosis, mitral stenosis (p = 0.04; 76% 3-year survival) had a better late outcome. There were no other significant predictors of late survival other than immediate prerepair pH (p = 0.05). Interpretation of this experience is complicated by the large number of different surgical techniques used for both first-stage neonatal reconstruction and the Fontan procedure plus introduction of the bidirectional Glenn shunt as an intermediate step midway through the experience. Nevertheless in this time frame and with the variety of techniques used, this experience demonstrates that patients with aortic atresia, mitral atresia, particularly those who have been very acidotic in the neonatal period, are least likely to do well with the reconstructive approach to hypoplastic left heart syndrome and are the most appropriate subgroup to be directed to transplantation. Patients with aortic stenosis, mitral stenosis have an excellent late outcome with the reconstructive approach.
我们对78例接受姑息性重建手术治疗左心发育不全综合征的患者进行了一项回顾性研究，这些患者代表了1983年至1991年期间的连续完整病例，目的是确定死亡率的预测因素，以便能够更恰当地将患者分为重建组或移植组。29例患者为主动脉闭锁、二尖瓣闭锁；18例为主动脉狭窄、二尖瓣狭窄；20例为主动脉闭锁和二尖瓣狭窄；11例为其他形式的左心发育不全综合征。有29例患者死于医院（37%）。术前最差pH值（p = 0.01）和术前即刻pH值（p = 0.03）低于中位数是医院死亡率的预测因素。解剖学亚组主动脉闭锁、二尖瓣狭窄（p = 0.06）的医院死亡率可能增加。1例患者失访。医院幸存者的Kaplan-Meier生存估计在3年时为34%，在5年时为25%。解剖学亚组主动脉闭锁、二尖瓣闭锁（p = 0.02）的晚期结局较差（3年生存率为11%），而亚组主动脉狭窄、二尖瓣狭窄（p = 0.04；3年生存率为76%）的晚期结局较好。除了术前即刻pH值（p = 0.05）外，没有其他晚期生存的显著预测因素。由于用于一期新生儿重建和Fontan手术的手术技术种类繁多，以及在研究过程中引入双向Glenn分流作为中间步骤，对这一经验的解读变得复杂。然而，在这个时间段内，以及使用的各种技术中，这一经验表明，患有主动脉闭锁、二尖瓣闭锁的患者，尤其是那些在新生儿期严重酸中毒的患者，采用重建方法治疗左心发育不全综合征效果最差，是最适合进行移植的亚组。患有主动脉狭窄、二尖瓣狭窄的患者采用重建方法的晚期结局良好。
