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How can we counsel the parents of fetuses diagnosed classical HLHS based on recent data?
울산대학교 서울아산병원 산부인과¹, 소아심장과², 소아심장외과³
원혜성¹, 백수진¹,박인숙²,장원경³, 박정준³,윤태진 ³,서동만³
Introduction The past two decades have brought significantly improved survival for infants with HLHS associated with modified surgical techniques, alterations in preoperative and postoperative intensive care management and monitoring. Despite improved outcomes, early termination of pregnancy remains popular choice for these affected fetuses. To better explore this issue, we examined the treatment choices and demographic characteristics of all fetuses with HLHS diagnosed prenatally. Material and Method: Between December 1997 and December 2008, 60 fetuses were identified with classical HLHS on fetal echocardiography in our institution. we retrospectively reviewed perinatal and postnatal medical records. Prenatal karyotyping was offered to all parents. In cases of termination of pregnancy, postmorterm examination of the fetus was advised. Results: The mean maternal age and median gestational age at diagnosis were 30.42±3.98 years and 24.6±6.48 weeks respectively. Karyotyping was performed in 43/60(72%) cases. The overall prevalence of aneuploidy was 7/43(16.2%) cases. After counseling, 43 couples chose to terminate the pregnancies (including seven with chromosomal anomalies). 21 of 43 patients delivered at our hospital. Of the 18 couples who continued with the pregnancy, one had stillbirths. There were 17 live births of whom five babies had an alternative major congenital heart disease diagnosed postnatally and appropriated surgery was carried out. A total of 10 babies died in the postnatal period. The overall survival was 17% (2/12). Conclusion: Despite the advances in pediatric cardiothoracic techniques, the outlook for antenatally diagnosed babies with HLHS still remain poor. Antenatal diagnosis allows time for parents to accept the diagnosis, like a chromosomal study or targeted ultrasonography at tertiary center, and explore the options available to them, but these data again highlight the poor outcome for babies with classical HLHS diagnosed prenatally. This is valuable information when counseling parents with an affected fetus.
  


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