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대동맥 축착증 및 심한 대동맥 협착을 가진 생후 5일의 환자가 대동맥 협착을 해결하려는 중재적 시술 도중 유도 카테터에 의한 우측 경동맥 손상으로 인한 출혈 및 심낭 내 탐폰으로 인한 심정지가 발생하여 심폐소생술 후 체외막성산화기 보조를 받았다. 환자의 체중은 2.4 ㎏이었다. 1일 후 수술적 완전 교정술을 시행하였으나, 수술 직후 심한 심기능 저하로 인하여 양심실 보조 장치로 순환 보조를 하였고, 3일 후 양심실 보조 장치 이탈에 성공하였다. 이후 환자는 일반적인 치료 후 특별한 문제없이 퇴원하였다. A 5-day-old neonate (body weight=2.4 ㎏) with coarctation of the aorta and critical aortic stenosis underwent an interventional balloon valvuloplasty for aortic stenosis. During the intervention, cardiac arrest occurred due to injury of the right carotid artery by the guide wire. An extracorporeal membrane oxygenator (ECMO) was applied. After 1 day's support, total surgical correction was achieved; however, in the immediate postoperative period, cardiac function was severely depressed. We applied a bi-ventricular assist device (bi-VAD) instead of an ECMO and we were able to wean the patient off the bi-VAD device after 3 days' support. The patient was discharged without severe complications.
Recently, surgical outcomes of repair of tetralogy of Fallot (TOF) have improved. For patients with TOFolder than 3 months, primary repair has been advocated regardless of symptoms. However, a surgicalapproach to symptomatic TOF in neonates or very young infants remains elusive. Traditionally, therehave been two surgical options for these patients: primary repair versus an initial aortopulmonary shuntfollowed by repair. Early primary repair provides several advantages, including avoidance of shuntrelatedcomplications, early relief of hypoxia, promotion of normal lung development, avoidance ofventricular hypertrophy and fibrosis, and psychological comfort to the family. Because of advances incardiopulmonary bypass techniques and accumulated experience in neonatal cardiac surgery, primaryrepair in neonates with TOF has been performed with excellent early outcomes (early mortality<5%),which may be superior to the outcomes of aortopulmonary shunting. A remaining question regardingsurgical options is whether shunts can preserve the pulmonary valve annulus for TOF neonates withpulmonary stenosis. Symptomatic neonates and older infants have different anatomies of right ventricularoutflow tract (RVOT) obstructions, which in neonates are nearly always caused by a hypoplastic pulmonaryvalve annulus instead of infundibular obstruction. Therefore, a shunt is less likely to preserve thepulmonary valve annulus than is primary repair. Primary repair of TOF can be performed safely in mostsymptomatic neonates. Patients who have had primary repair should be closely followed up to evaluatethe RVOT pathology and right ventricular function.
Background and Objectives: Mechanical circulatory support with extracorporeal membrane oxygenation (ECMO) and ventricular assist device has always been the optimal choice for treating the majority of medically intractable low cardiac output case. We retrospectively investigated our institution's outcomes and variables associated with a high risk of mortality. Subjects and Methods: From 1999 to 2014, 86 patients who were of pediatric age or had grown-up congenital heart disease underwent mechanical circulatory support for medically intractable low cardiac output in our pediatric intensive care unit. Of these, 9 grown-up congenital heart disease patients were over 18 years of age, and the median age of the subject group was 5.82 years (range: 1 day to 41.6 years). A review of all demographic, clinical, and surgical data and survival analysis were performed. Results: A total of 45 (52.3%) patients were successfully weaned from the mechanical assist device, and 25 (29.1%) survivors were able to be discharged. There was no significant difference in results between patients over 18 years and under 18 years of age. Risk factors for mortality were younger age (<30 days), functional single ventricle anatomy, support after cardiac operations, longer support duration, and deteriorated pre-ECMO status (severe metabolic acidosis and increased levels of lactate, creatinine, bilirubin, or liver enzyme). The survival rate has improved since 2010 (from 25% before 2010 to 35% after 2010), when we introduced an upgraded oxygenator, activated heart transplantation, and also began to apply ECMO before the end-stage of cardiac dysfunction, even though we could not reveal significant correlations between survival rate and changed strategies associated with ECMO. Conclusion: Mechanical circulatory support has played a critical role and has had a dramatic effect on survival in patients with medically intractable heart failure, particularly in recent years. Meticulous monitoring of acid-base status, laboratory findings, and early and liberal applications are recommended to improve outcomes without critical complication rates, particularly in neonates with single ventricle physiology.