산전진단된 지속 좌측 상대정맥 (persistent left superior vena cava)의 임상적 의의
Clinical significance of persistent left superior vena cava diagnosed in fetal life
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목적: 최근 산전심초음파검사 시 상부 종격에서 관찰되는 삼혈관 영상에 대한 중요성이 대두되면서 지속 좌측 상대정맥의 진단율이 증가되고 있다. 이에 본 연구에서는 선천성 심질환과의 연관성뿐만 아니라 구조적 심기형을 동반하지 않은 단독 지속 좌측 상대정맥을 가진 환아들의 예후를 조사하여 지속 좌측 상대정맥의 임상적 의의에 대해 밝혀보고자 하였다. 연구 방법: 2001년 5월부터 2008년 5월까지 서울아산병원에서 시행한 총 17,100명의 임신 중기 정밀초음파검사에서 지속 좌측 상대정맥으로 진단된 85명을 대상으로 의무기록과 산전 초음파 영상들을 후향적으로 분석하였다. 결과: 총 85예의 지속 좌측 상대정맥이 산전 진단되었으며 단독 지속 좌측 상대정맥은 15예에서 진단되었다. 85예 중 동반된 심기형으로는 심실 중격 결손, 양대혈관 우심실 기시, 방실 중격 결손이 각각 54.1%, 17.6%, 17.6%로 높게 나타났고 15예의 단독 지속 좌측 상대정맥 중 산후에 전 폐정맥 환류이상으로 진단된 1예와 경과 소실된 1예를 제외한 13예는 특별한 합병증 없이 양호한 상태로 확인되었다. 결론: 지속 좌측 상대정맥이 태아 선천 심기형을 예측하는 강력한 인자로 사용될 수 있으며, 심기형을 동반하지 않은 단독 지속 좌측 상대정맥은 출생 후 특별한 문제가 없는 정맥계 기형 중 하나로 산전상담 시 참고할 수 있겠다.
Abstract
Objective: To determine the clinical significance of persistent left superior vena cava (PLSVC) in a fetus with and without cardiac and extra-cardiac anomalies. Methods: This was a retrospective review of all cases of PLSVC detected prenatally at our institution between May 2001 and May 2008. This retrospective study included 85 fetuses with PLSVC who were diagnosed based on the presence of an additional vessel identified to the left of the pulmonary artery in the three-vessel view of the heart. Patient charts and recorded images were reviewed in order to identify associated conditions and outcomes. Telephone interviews were conducted to check patients’ conditions in cases of isolated PLSVC. Results: Eighty-five cases of PLSVC were detected prenatally during this study period. Of these 85 fetuses, 11 were aborted due to associated, prenatally proven, severe congenital heart anomalies or chromosomal anomalies, and 52 fetuses were delivered. The cases for other 22 fetuses were lost to follow-up. Postnatal echocardiography was performed in the 33, surviving patients, and PLSVC was confirmed in 32 of these patients. The most common associated congenital cardiac anomalies were seen included VSD, AVSD, and DORV (54.1%, 17.6% and 17.6%, respectively) (Table 3). PLSVC was also seen in seven cases (8.2%) of right isomerism and in four cases (4.7%) of left isomerism. In only two cases was the coexistence of PLSVC and extra-cardiac anomalies noted in this study. Fifteen cases were prenatally diagnosed as isolated PLSVC and all of them had live births. The follow-up period in our isolated PLSVC patients ranged from 0.5 to 84 months (Mean 24.5 months). Thirteen of these infants were doing well at the time of preparing this document and one case was diagnosed as TAPVR on postnatal echocardiography and one case was lost to follow-up. Conclusion: We strongly suggest that PLSVC is a benign vascular malformation and does not affect to the patient after birth. However, PLSVC is frequently associated with heterotaxy syndromes as well as other cardiac malformations and can be misdiagnosed as TAPVR. So if we find PLSVC in prenatal ultrasonography, meticulous inspection of the fetal anatomy must be performed.
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