Villain E, Vetter V L, Garcia J M, Herre J, Cifarelli A, Garson A
Department of Pediatrics, Baylor College of Medicine, Houston, Texas.
Circulation. 1990 May;81(5):1544-9. doi: 10.1161/01.cir.81.5.1544.
We reviewed the records of 26 infants with congenital junctional ectopic tachycardia (JET) from seven institutions to examine the evolution in the management of this tachycardia that is difficult to treat. JET was defined electrocardiographically as an incessant tachycardia with normal QRS morphology and atrioventricular (AV) dissociation. The ventricular rate ranged from 140 to 370 beats/min (mean, 230 beats/min); 16 of 26 patients had cardiac failure. Treatment success was defined as a stable decrease in the rate of JET, below 150 beats/min; partial success was a significant decrease of JET rate with alleviation of symptoms. All patients received digoxin with no significant effect. Propranolol was given to 16 patients, with two successes and one partial success. Combinations of other conventional agents were used in 11 patients with two successes; 14 patients were treated with amiodarone, which resulted in eight successes and three partial successes; three patients died suddenly on medical treatment (amiodarone, one patient; propranolol, one patient; or amiodarone plus propranolol, one patient); sudden AV block was a possible cause and consequently, two later patients had pacemaker implantation as well as medical treatment. His catheter ablation was successfully performed twice but contributed to death in a newborn; three surgical His ablations were performed for intractable JET with two successes and one death. The overall mortality was 35%. Among survivors, treatment has been stopped without any complications in five patients ranging in age from 10 months to 8 years (mean, 3.5 years). It seems that amiodarone alone is the best drug for treatment of congenital JET; necessity for permanent pacing remains unsettled. His ablation should be reserved only for intractable JET.
我们回顾了来自7家机构的26例先天性交界性异位性心动过速（JET）婴儿的记录，以研究这种难以治疗的心动过速的治疗演变情况。JET在心电图上被定义为一种持续的心动过速，QRS形态正常且存在房室（AV）分离。心室率为140至370次/分钟（平均230次/分钟）；26例患者中有16例出现心力衰竭。治疗成功定义为JET心率稳定下降至150次/分钟以下；部分成功为JET心率显著下降且症状缓解。所有患者均接受地高辛治疗，但无显著效果。16例患者使用了普萘洛尔，2例成功，1例部分成功。11例患者使用了其他传统药物联合治疗，2例成功；14例患者接受了胺碘酮治疗，8例成功，3例部分成功；3例患者在接受药物治疗时突然死亡（胺碘酮治疗1例；普萘洛尔治疗1例；胺碘酮加普萘洛尔治疗1例）；突然出现的房室传导阻滞可能是原因，因此，后来有2例患者在接受药物治疗的同时植入了起搏器。希氏束导管消融成功进行了2次，但导致1例新生儿死亡；3例因顽固性JET进行了外科希氏束消融，2例成功，1例死亡。总死亡率为35%。在幸存者中，5例年龄在10个月至8岁（平均3.5岁）的患者停止治疗后无任何并发症。似乎单独使用胺碘酮是治疗先天性JET的最佳药物；永久性起搏的必要性仍未确定。希氏束消融仅应保留用于顽固性JET。
