초록접수 현황
| 15F-051 | 구연 발표 |
One-stage Repair of Interrupted Aortic Arch in Combination with Aortopulmonary Window: Mid-term Outcome in Single Institution.
박지영¹, 김웅한¹, 현관용¹, 조성규¹, 이정렬¹, 김용진²
서울대학교 의과대학 서울대학교병원 흉부외과학교실¹, 서울대학교 분당서울대학교병원 흉부외과학교실²
Background : The combination of interrupted aortic arch (IAA) and aortopulmonary window (APW) is a rare presentation of congenital heart disease, which requires early diagnosis and surgical treatment. We assessed the mid-term outcome of the one-stage repair for this group of patients.
Methods : Between June 1993 and January 2015, 7 neonates and 4 infants with APW and IAA underwent one-stage repair. 7 patients achieved the one-stage repair with continuous cerebral perfusion using the dual-perfusion technique. Extended end-to-side anastomosis was performed for IAA in all patients
Results : The IAA was type A in 6 patients and type B in 5 patients. The APW morphology was type I in one patient, type II in 9 patients, and type III in 1 patient. There were 7 patients with Berry syndrome and 3 of 7 patients required right pulmonary artery (RPA) re-implantation. The median age at the operation was 15 days (range: 6~219), the mean body weight at the operation was 3.4 ± 1.1 kg. There was no early mortality. During a mean of 7.9 ± 7.1 years of follow-up, one late death occurred 2 years after the operation due to non-cardiac problem. Actuarial survival rate was 88.9 ± 0.1 % at 10 years. Among 6 patients who underwent 7 reoperations, 3 patients underwent right pulmonary artery angioplasty, 2 underwent aortopexy for airway obstruction and one patient got the operation for recurrent arch obstruction. The freedom from any reoperation were 81.8, 57.3% and 43.0% at 1, 3, and 5 years. Berry syndrome (p=0.65) and Low body weight less than 3 kg (p=0.23) are not risk factors for reoperation.
Conclusion : One-stage repair of interrupted aortic arch with aortopulmonary window can be safely performed with low mortality and good mid-term results. Careful follow-up is mandatory because stenosis at the site of the RPA and the aortic reconstruction is a potential problem.
Methods : Between June 1993 and January 2015, 7 neonates and 4 infants with APW and IAA underwent one-stage repair. 7 patients achieved the one-stage repair with continuous cerebral perfusion using the dual-perfusion technique. Extended end-to-side anastomosis was performed for IAA in all patients
Results : The IAA was type A in 6 patients and type B in 5 patients. The APW morphology was type I in one patient, type II in 9 patients, and type III in 1 patient. There were 7 patients with Berry syndrome and 3 of 7 patients required right pulmonary artery (RPA) re-implantation. The median age at the operation was 15 days (range: 6~219), the mean body weight at the operation was 3.4 ± 1.1 kg. There was no early mortality. During a mean of 7.9 ± 7.1 years of follow-up, one late death occurred 2 years after the operation due to non-cardiac problem. Actuarial survival rate was 88.9 ± 0.1 % at 10 years. Among 6 patients who underwent 7 reoperations, 3 patients underwent right pulmonary artery angioplasty, 2 underwent aortopexy for airway obstruction and one patient got the operation for recurrent arch obstruction. The freedom from any reoperation were 81.8, 57.3% and 43.0% at 1, 3, and 5 years. Berry syndrome (p=0.65) and Low body weight less than 3 kg (p=0.23) are not risk factors for reoperation.
Conclusion : One-stage repair of interrupted aortic arch with aortopulmonary window can be safely performed with low mortality and good mid-term results. Careful follow-up is mandatory because stenosis at the site of the RPA and the aortic reconstruction is a potential problem.
책임저자: 김웅한
서울대학교 의과대학 서울대학교병원 흉부외과학교실
연락처 : 박지영, Tel: 010-7765-8731 , E-mail : chunhaa@naver.com
