초록접수 현황
| 15F-089 | 포럼 발표 |
Long-term Results after Surgical Treatment of Ebstein’s Anomaly; 30-year Experience
김민석, 임홍국, 김웅한, 이정렬, 김용진
서울대학교 의과대학 서울대학교병원 흉부외과학교실
Background : Ebstein’s anomaly is a rare disorder accounting for about 1% of all congenital heart diseases. Due to its wide spectrum and complexity of anomaly, a variety of surgical methods have been introduced in the treatment of Ebstein’s anomaly. The aim of the study was to evaluate the long-term results after surgical repair of Ebstein’s anomaly at Seoul National University Children’s hospital, during the past 30 years.
Methods : The medical records of 48 pediatric patients who underwent surgical treatment of Ebstein’s anomaly between 1982 and 2014 were studied. Functional, demographic, and echocardiographic parameters were studied both preoperatively and postoperatively. Of 48 patients, 28 patients were female, and median age at operation was 5.6 years (1 day ~ 42.1 years). Major preoperative symptoms were exertional dyspnea (n=24, 50%) and cyanosis (n=24, 50%). Most common associated cardiac anomalies was atrial septal defect (n=40, 83.3%). Thirty-four patients (70.8%) demonstrated tricuspid regurgitation of greater than moderate degree. When the patients were divided according to Carpentier’s classification, types A, B, C and D were 11 (22.9%), 21 (43.8%), 12 (25.0%), and 4 (8.3%), respectively. Biventricular repair (n=38), one-and-a-half ventricular repair (n=5), and single ventricle palliation (n=5) were performed for surgical treatment of Ebstein’s anomaly. Tricuspid valve repair using Danielson’s technique was performed in 24 patients, Carpentier’s technique was performed in 12 patients, and Cone repair was performed in 4 patients. Median follow-up was 9.6 years.
Results : Surgical treatment strategies were different according to Carpentier’s types (p < 0.001) and patient’s age (p = 0.022); single ventricle repair was more frequently performed in patients with more progressed Carpentier’s type, and it was only performed in infants. The severity of tricuspid valve regurgitation decreased significantly after surgery in majority of patients, although it tended to progress at latest follow-up. There were 2 in-hospital mortalities and 2 late mortalities. Thirteen patients underwent reoperations during the follow-up, including 11 reoperations for progressed tricuspid valve regurgitation. Survival rates for 5, 10, and 20 years were 95.8%, 95.8%, and 85.6%, respectively. Freedom from reoperation rates for 5, 10, and 15 years were 85.9%, 68.0%, and 55.8%, respectively.
Conclusion : Various surgical treatment strategies were used according to Carpentier’s type and patient’s age. Survival and freedom from reoperation rates at 10 years were 95.8% and 68.0%, respectively. Despite anatomic and clinical complexities, Ebstein’s anomaly can be repaired with reasonable clinical results in children. However, surgical strategy should be individualized for the better surgical outcome.
Methods : The medical records of 48 pediatric patients who underwent surgical treatment of Ebstein’s anomaly between 1982 and 2014 were studied. Functional, demographic, and echocardiographic parameters were studied both preoperatively and postoperatively. Of 48 patients, 28 patients were female, and median age at operation was 5.6 years (1 day ~ 42.1 years). Major preoperative symptoms were exertional dyspnea (n=24, 50%) and cyanosis (n=24, 50%). Most common associated cardiac anomalies was atrial septal defect (n=40, 83.3%). Thirty-four patients (70.8%) demonstrated tricuspid regurgitation of greater than moderate degree. When the patients were divided according to Carpentier’s classification, types A, B, C and D were 11 (22.9%), 21 (43.8%), 12 (25.0%), and 4 (8.3%), respectively. Biventricular repair (n=38), one-and-a-half ventricular repair (n=5), and single ventricle palliation (n=5) were performed for surgical treatment of Ebstein’s anomaly. Tricuspid valve repair using Danielson’s technique was performed in 24 patients, Carpentier’s technique was performed in 12 patients, and Cone repair was performed in 4 patients. Median follow-up was 9.6 years.
Results : Surgical treatment strategies were different according to Carpentier’s types (p < 0.001) and patient’s age (p = 0.022); single ventricle repair was more frequently performed in patients with more progressed Carpentier’s type, and it was only performed in infants. The severity of tricuspid valve regurgitation decreased significantly after surgery in majority of patients, although it tended to progress at latest follow-up. There were 2 in-hospital mortalities and 2 late mortalities. Thirteen patients underwent reoperations during the follow-up, including 11 reoperations for progressed tricuspid valve regurgitation. Survival rates for 5, 10, and 20 years were 95.8%, 95.8%, and 85.6%, respectively. Freedom from reoperation rates for 5, 10, and 15 years were 85.9%, 68.0%, and 55.8%, respectively.
Conclusion : Various surgical treatment strategies were used according to Carpentier’s type and patient’s age. Survival and freedom from reoperation rates at 10 years were 95.8% and 68.0%, respectively. Despite anatomic and clinical complexities, Ebstein’s anomaly can be repaired with reasonable clinical results in children. However, surgical strategy should be individualized for the better surgical outcome.
책임저자: 김용진
서울대학교 의과대학 서울대학교병원 흉부외과학교실
연락처 : 김민석, Tel: 02-732-3285 , E-mail : mesmerist84@gmail.com
