Cullen M, Splittgerber F, Sweezer W, Hakimi M, Arciniegas E, Klein M
J Pediatr Surg. 1986 Aug;21(8):675-7. doi: 10.1016/s0022-3468(86)80384-0.
Severe pulmonary hypertension complicating the correction of congenital cardiac defects is an unusual cause of early postoperative mortality. We present a case of a nine-month-old infant who developed paroxysmal pulmonary hypertension associated with severe hypoxemia after the successful repair of a large perimembranous ventricular septal defect (VSD). The pulmonary hypertension was refractory to all medical and pharmacologic therapy but was successfully treated with extracorporeal membrane oxygenation (ECMO). On ECMO, pharmacologic support was removed, pulmonary artery pressure reduced, and ECMO support withdrawn. To date, ECMO has been applied to pulmonary hypertension of the newborn, neonatal respiratory insufficiency, and for primary cardiac pump failure. Our experience with this case leads us to believe it is an effective therapy for acute pulmonary hypertension occurring after the repair of congenital cardiac anomalies.
严重肺动脉高压并发先天性心脏缺陷矫治是术后早期死亡的罕见原因。我们报告一例9个月大婴儿,在成功修复大型膜周部室间隔缺损(VSD)后出现阵发性肺动脉高压并伴有严重低氧血症。该肺动脉高压对所有药物和药理治疗均无效,但通过体外膜肺氧合(ECMO)成功治疗。在ECMO支持下,撤除了药物支持,肺动脉压力降低,随后撤除了ECMO支持。迄今为止,ECMO已应用于新生儿肺动脉高压、新生儿呼吸功能不全及原发性心泵衰竭。我们对该病例的经验使我们相信,它是治疗先天性心脏畸形修复术后发生的急性肺动脉高压的有效疗法。