Out of 883 fetuses with CHD, fetal echocardiographic results in 110(12%) with HLHS were correlated with postnatal outcome. Fetal echocardiography was performed in one fetal cardiology reference center. Neonates were operated on in two different pediatric cardiac surgery centers. There were just 8 fetuses who had severe AS leading to HLHS at the end of pregnancy. 21 fetuses had severe Fo restriction(no survivors) and 8 moderate restriction(survived). Since 2002 more precise prenatal diagnosis was made, with special interest in Fo communication. In 3 progression of Fo restriction was observed. The study was divided into 2periods: (1)1994-2001(22fetuses) and (2)2002-2008(88fetuses). Follow up in the(1) period: TOP-2(9%), IUD-1, live born-19. Neonatal death – 10: ECM-3, compassionate care due to parents’ request - 7. 1 newborn died after unsuccesfull aortic valvuloplasty, 7 after classic Norwood operation, it was 1 survivor after modified Norwood. Follow up in the (2)period: TOP-13(15%); IUD–3; neonatal death-15: prematurity–2, severe restrictionFo-11, 2 after unsucesfull BAS, 3–compassionate care due to severe RV dysfunction, 3-ECM. 2babies died after oesophageal atresia operation, 9 after Norwood procedure, in whom 4 had restrictive Fo. There were 29 survivors: 33% of all, but 40% of live-borns, all after modified Norwood procedure.
Conclusions. Results of HLHS treatment improved significantly, but still prenatal diagnosis is the risk factor. Fetal echocardiography must be repeated monthly due to precise counseling and organizing optimal perinatal care. As a restrictive Fo is a real risk factor for adverse outcome, prenatal or very quick postnatal intervention must be taken into consideration.