Fermont L, Wertheimer J, Batisse A, Bical O, Sidi D, Piéchaud J F, Kachaner J
Arch Fr Pediatr. 1982 Jun-Jul;39(6):359-66.
Interruption of the aortic arch, associated with curable cardiovascular anomalies (8 times out of 10) was observed over a period of 8 years in 50 children aged less than 10 days in 80% of cases. They presented with congestive heart failure, with variable degree of shock in most cases, suggesting the diagnoses of left ventricular hypoplasia or coarctation syndrome. Diagnosis was established with ultrasound and angiocardiography. Evolution was lethal in 21 children who were not operated on. 20 children were operated on: before treatment with prostaglandin E1, 9 attempts ended up in 78% immediate mortality and 89% global mortality; after treatment with prostaglandin. E1, these figures were respectively reduced to 45% and 55% (20 cases). This malformation should therefore be recognized as an emergency and then treated with prostaglandin up to surgery.
在8年的时间里,对50名10日龄以下的儿童进行观察,发现80%的病例中存在与可治愈的心血管异常相关的主动脉弓中断(十分之八)。他们表现为充血性心力衰竭,大多数病例伴有不同程度的休克,提示诊断为左心室发育不全或缩窄综合征。通过超声心动图和心血管造影术确诊。21名未接受手术的儿童病情进展致命。20名儿童接受了手术:在使用前列腺素E1治疗前,9次手术尝试的即时死亡率为78%,总体死亡率为89%;使用前列腺素E1治疗后,这些数字分别降至45%和55%(20例)。因此,这种畸形应被视为紧急情况,然后在手术前使用前列腺素进行治疗。