Journal of Chest Surgery

The Korean Society for Thoracic and Cardiovascular Surgery (대한심장혈관흉부외과학회)
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Early and Midterm Results of Arterial Switch Operation for Double-Outlet Right Ventricle with Subpulmonary VSD

폐동맥하 심실증격결손을 동반한 양대혈관 우심실기시중에서 동맥전환술의 중단기 결과

  • 양승인 (부산대학교 의과대학 흉부외과학교실) ;
  • 이형두 (부산대학교 의과대학 소아과학교실) ;
  • 김시호 (동아대학교 의과대학 흉부외과학교실) ;
  • 조광조 (동아대학교 의과대학 흉부외과학교실) ;
  • 우종수 (동아대학교 의과대학 흉부외과학교실) ;
  • 이영석 (동아대학교 의과대학 소아과학교실) ;
  • 성시찬 (부산대학교 의과대학 흉부외과학교실)
  • Published : 2004.04.01
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Abstract

Excellent clinical results of the arterial switch operation and the limited availablity of the intraventricular rerouting has recently made an arterial switch operation to become the therapeutic method of choice for the repair of double-outlet right ventricle (DORV) with subpulmonary ventricular septal defect (VSD). The early and midterm outcomes of arterial switch operation for this anomaly were evaluated. Material and Method: Between August 1994 and July 2002, 13 patients underwent an arterial switch operation for the correction of double-outlet right ventricle with subpulmonary VSD at Dong-A university hospital.. The 50% rule was used to define DORV. Median age and mean body weight were 27 days (range, 3-120 days) and 3.8$\pm$0.7kg (range, 2.92-5.3kg) respectively. Aortic arch anomalies were associated in 6 cases (46.2%), which were all repaired through one-stage operation. The relationship of the great arteries were side-by-side in 8 cases (61.5%) and anteroposterior in 5 (38.5%). Coronary artery patterns were 1 LCx-2R in 6 cases, retropulmonary left coronary artery (LCA) in 6, and intramural LCA in 1 respectively. The enlargement of VSD was required in 1 patient and the patch enlargement of right ventricular outflow tract was performed in another one patient. The Lecompte maneuver was used in all but 3 patients with a side by side relationship of the great arteries. Result: Overall postoperative hospital mortality was 23.1 % (3/13). All operative deaths were occurred in the patients with aortic arch anomalies. There was one late death related to the postoperative complication of the central nerve system during the mean follow-up of 41.3$\pm$30.7 months. Pulmonary valvar stenosis (＞30mmHg of pressure gradient) developed in 1 patient (10%) and left pulmonary artery stenosis in 2 (20%), among them, one required reoperation 52 months after repair. There was an asymptomatic patient with moderate aortic regurgitation. 5-year survival rate including operative deaths was 68.3%. Conclusion: Although the operative mortality is high in the patients with aortic arch anomaly, the arterial switch operation for DORV with supbpulmonary VSD can be performed with low operative mortality and low reoperation rate in the patients Without arch anomaly. The arterial switch operation can be considered a good option for this complex anomaly.

폐동맥하 심실중격결손을 동반한 양대혈관 우심실기시증에서 동맥전환술은 심실내 교정의 제한된 적응증과 동맥전환술의 우수한 임상성적으로 인해 최근 이 기형의 선택적 치료법으로 자리잡고 있다. 이 기형에 대한 동맥전환술의 단기 및 중기 성적을 검토하였다. 대상 및 방법 1994년 8월부터 2002년 7월까지 8년 동안 동아대학교 병원에서 심실중격결손증 교정과 동맥전환술을 시행한 폐동맥하 심실중격결손을 동반한 양대혈관 우심실기시증 환자 13명을 대상으로 후향적 조사를 하였다. 양대혈관 우심실기시의 진단은 50% rule을 적용하였다. 중심나이와 평균체증은 각각 27일(범위, 3-120일)과 3.8$\pm$0.7kg (범위, 2.92-5.3kg)이었다. 대동맥궁기형은 6명(46.2%)에서 동반되었고 모두 일차 완전교정(one-stage repair)으로 교정되었다. 양대혈관의 위치관계는 좌우로 위치한 것이 8예(61.5%), 전후로 위치한 것이 5예(38.5%)였다. 관상동맥의 형태는 1LCx-2R과 좌관상동맥이 폐동맥의 뒤로 돌아가는 형태가 각각 6예씩(46.2%)이었으며 1명(7.7%)에서 벽속 좌관상동맥기형이 관찰되었다. 심실중격결손의 확장 및 패치를 이용한 우심실유출로 성형술이 각각 1명(7.7%)에서 시행되었다 좌우 대혈관 위치관계를 갖고 있는 3명(23.1%)을 제외한 모든 환자에서 Lecompte 술식을 시행하였다. 결과: 3예(23.1%)의 수술사망이 발생하였다. 3예 모두 대동맥궁기형을 갖고 있었던 경우였다. 수술 생존자의 평균추적기간 41.3$\pm$30.7개월(범위 1.7-79.1개월) 동안 만기사망은 1예(10%)로 술 후 5개월 뒤 중추신경계 합병증으로 사망하였다. 1예(10.0%)에서 압력차 30mmHg 이상의 폐동맥판 협착이 발견되었고 2예에서 좌폐동맥 협착으로 풍선확장술이 필요하였으며 이 중 1예(10%)에서 술 후 52개월만에 재수술이 필요하였다. 무증상의 중등도 대동맥판 폐쇄부전증이 1예(10%)에서 발견되었다. 수술사망을 포함한 5년 생존율은 68.3%였다. 결론: 대동맥궁기형을 동반한 폐동맥하 심실중격결손의 양대혈관 우심실기시증 환자에서는 높은 수술사망률을 보였으나 동반하지 않은 경우는 낮은 수술사망률과 재수술률을 보여 동맥전환술이 이 심기형에서 유용한 수술방법으로 고려될 수 있을 것이라고 생각한다.

Keywords

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