태아 심장종양의 산전 진단과 산후 결과
Fetal cardiac tumor: Prenatal diagnosis and postnatal outcome
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목적: 본 연구의 목적은 분만 전 및 후의 태아 심장종양의 초음파상 모습을 평가하고 결절상 경화증 등 연관 문제들을 기술하 고자 함이다. 방법: 1996년부터 2006년까지 단일 기관에서 산전에 태아 심장종양을 진단받고 출생한 환아들의 의무 기록을 후향적으로 분석 하였다. 결과: 총 10예의 태아 심장종양 사례가 발견되었다. 출산 당시의 산모의 평균 연령은 30.2±2.4세였고, 진단 당시의 임신주수는 30.6±5.4주였다. 심장종양은 5예에서 단일 종괴였고 나머지 5예에서는 다수였다. 종괴의 크기는 7 mm에서 34 mm로 다양했 다. 대부분의 종괴는 우심실에 위치하였으며 (n=9), 좌심실 (n=6), 심실 중벽 (n=4), 우심방 (n=1)에도 위치하였다. 1예에서 태아 부정맥이 있었으나 출생 후 2일째 정상화 되었고, 다른 1예에선 경도의 심혈류장애가 발견되었다. 생후 추적관찰기간은 2개월에서 36개월로 다양하였다 (평균 18.9±13.1개월). 대부분의 사례에서 종양의 크기는 생후 줄어들었다 (n=6). 하지만 태 내에선 변화없는 경우가 많았다 (n=5). 10예 중 3예에서 결절성 경화증이 진단되었고 수술적 치료를 요한 경우는 없었다. 결론: 태아 심장종양과 태아 심장종양의 심장 기능에 대한 영향은 이차원 Doppler 심초음파로 충분히 평가할 수 있었다. 태아 심장종양은 출산 전과 후 모두에서 혈역학적 면에서는 태아 안녕에 거의 영향을 미치지 않았다. 하지만 태아 심장종양은 결절성 경화증의 조기 진단의 단서가 될 수 있으므로 관련 산모 및 태아에 대한 철저한 산전 상담 및 관리가 필요하다 하겠다.
Abstract
Objective: The objective of this study was to evaluate the ultrasonographic appearance of suspected fetal cardiac tumor and their evolution until delivery and in the postnatal period, and to document the associated problems including tuberous sclerosis. Methods: We retrospectively reviewed the medical records of all cases born in Yonsei University Health System, Seoul, Korea, between September 1996 and August 2006, and diagnosed as fetal cardiac tumor prenatally. Results: 10 cases were found in all medical records. The mean age of the mothers on delivery was 30.2±2.4 and the mean gestational age on diagnosis was 30.6±5.4 weeks. The cardiac tumors were single in five cases and multiple in the other five cases. The size ranged from 7 to 34mm. Most of the tumors were located in right ventricle (RV, n=9), left ventricle (LV, n=6), but they also located in interventricular septum (IVS, n=4), right atrium (RA, n=1). In one case, fetal arrhythmia was found, which was normalized in two days after birth, and in another case, mild intracardiac flow obstruction was noted. The duration of postnatal follow-up ranged from 2 months to 36 months (mean, 18.9±13.1 months). In most cases the tumor masses decreased after birth (n=6), but had no change in utero (n=5). Three of them were diagnosed as tuberous sclerosis after birth, and none of them needed surgical intervention. Conclusion: Fetal cardiac tumors and their effect on the fetal cardiac function could be well evaluated by two-dimensional and Doppler echocardiography. The fetal cardiac tumors may have little effect on the fetal well being both prenatally and postnatally from the cardiovascular standpoint in most affected fetuses, but are important in the early diagnosis of tuberous sclerosis and in suggesting careful follow-up and management.
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